IAP Textbook of Pediatrics, 5E (2013) (PDF) (UnitedVRG) PDF [PDF]

Zitiervorschau

IAP Textbook of i i

iat

r cs

iat

Ped

V d

ti e

n U

G R

vip.persianss.ir

V d

ti e

n U

G R

vip.persianss.ir

IAP Textbook of i

i

r cs

iat

iat

Ped

E

Fifth dition Chief Academic Editor

A Parthasarathy MD(Ped) DCH FIAP

P N Menon MD MNAMS FIAP S

Editor-in-Chief President Indian Academy of Pediatrics 1997 Distinguished Professor of Pediatrics The Tamil Nadu Dr MGR Medical University Senior Clinical Professor of Pediatrics (Retd) Madras Medical College Deputy Superintendent, Institute of Child Health and Hospital for Children, Chennai, Tamil Nadu

President Indian Society for Pediatric and Adolescent Endocrinology 2011−12 Consultant and Head, Department of Pediatrics, Jaber Al-Ahmed Armed Forces Hospital, Kuwait Former Professor of Pediatrics, All India Institute of Medical Sciences, New Delhi

G R

Piyush Gupta MD FAMS FIAP

R

Editor-in-Chief Indian Academy of Pediatrics 2008−13 Professor Department of Pediatrics University College of Medical Sciences, Delhi

ti e

Editorial Advisers

n U

President Indian Academy of Pediatrics 2012 Consultant Pediatrician Kohinoor Hospital and Chandra-Jyoti Children Hospital Mumbai, Maharashtra

TU ukumaran MD DCH MNAMS MPhil FIAP President Indian Academy of Pediatrics 2011 Professor Department of Pediatrics Pushpagiri Institute of Medical Sciences Thiruvalla, Kerala

Forewords S

P Bansal, ohit Agrawal, TU ukumaran R

C

President Indian Academy of Pediatrics 2004 Professor of Pediatrics and Clinical Epidemiology Director, Child Development Centre Government Medical College Thiruvananthapuram, Kerala

S

ohit Agrawal MD FIAP

MK Nair PhD MD M Med Sc MBA, FIAP C

V d

Academic Editors

IAP, National Publication House, Gwalior

JAYPEE BROTHERS MEDICAL PUBLISHERS (P) LTD. New Delhi • London • Philadelphia • Panama

vip.persianss.ir

Jaypee Brothers Medical Publishers (P) Ltd.

Headquarters Jaypee Brothers Medical Publishers (P) Ltd. 4838/24, Ansari Road, Daryaganj New Delhi 110 002, India Phone: +91-11-43574357 Fax: +91-11-43574314 Email: [email protected]

G R

Overseas Offices

Jaypee Brothers Medical Publishers (P) Ltd. 17/1-B, Babar Road, Block-B, Shaymali Mohammadpur, Dhaka-1207 Bangladesh Mobile: +08801912003485 Email: [email protected]

Jaypee Brothers Medical Publishers (P) Ltd. Shorakhute, Kathmandu Nepal Phone: +00977-9841528578 Email: [email protected]

Website: www.jaypeebrothers.com Website: www.jaypeedigital.com

m

V d

ti e

n U

© 2013, Jaypee Brothers Medical Publishers

p

Jaypee-Highlights edical ublishers Inc. City of Knowledge, Bld. 237, Clayton Panama City, Panama Phone: +507-301-0496 Fax: +507-301-0499 Email: [email protected]

 

J.P. Medical Ltd. 83, Victoria Street, London SW1H 0HW (UK) Phone: +44-2031708910 Fax: +02-03-0086180 Email: [email protected]

Jaypee Brothers Medical Publishers Ltd. The Bourse 111, South Independence Mall East Suite 835, Philadelphia, PA 19106, USA Phone: + 267-519-9789 Email: [email protected]

All rights reserved. No part of this book may be reproduced in any form or by any means without the prior permission of the publisher. Inquiries for bulk sales may be solicited at: [email protected] This book has been published in good faith that the contents provided by the contributors contained herein are original, and is intended for educational purposes only. While every effort is made to ensure accuracy of information, the publisher and the editors specifically disclaim any damage, liability, or loss incurred, directly or indirectly, from the use or application of any of the contents of this work. If not specifically stated, all figures and tables are courtesy of the editors. Where appropriate, the readers should consult with a specialist or contact the manufacturer of the drug or device.

IAP Textbook of Pediatrics First Edition: 1999, 2000 Second Edition: 2002,2003 Third Edition: 2006 Revised Reprint: 2007 Fourth Edition: 2009 Fifth Edition: 2013  

ISBN 978-93-5025-945-0 Printed at

vip.persianss.ir

D

G R

edicated to

The future citizens of India whose Care and Nurture are our Goal, Concern and Commitment

n U

ti e

V d

vip.persianss.ir

V d

ti e

n U

G R

vip.persianss.ir

Contributors AP ubey Director-Professor and Head Department of Pediatrics Maulana Azad Medical College New Delhi [email protected]

Abraham K Paul Consultant Pediatrician Cochin Hospital Kochi, Kerala [email protected]

Anju Aggarwal Associate Professor Department of Pediatrics University College of Medical Sciences Delhi [email protected]/ [email protected]

A Parthasarathy Distinguished Professor of Pediatrics The Tamil Nadu Dr MGR Medical University Senior Clinical Professor of Pediatrics (Retd) Madras Medical College Deputy Superintendent Institute of Child Health and Hospital for Children Chennai, Tamil Nadu [email protected]

C

D

AK utta Director-Professor and Head Department of Pediatrics Lady Hardinge Medical College New Delhi [email protected]

ti e

n U

Amita Trehan Additional Professor Pediatric Hematology, Oncology and Advanced Pediatric Centre Postgraduate Institute of Medical Education and Research Chandigarh [email protected]

V d

Anoop Verma Consultant Pediatrician Swapnil Nursing Home Raipur, Chhattisgarh [email protected]

Anupam achdeva Director Pediatric Hematology and Oncology and Bone Marrow Transplantation Chairman Academics Convener Research Sir Ganga Ram Hospital New Delhi [email protected] Anupama Borker Associate Professor Department of Pediatrics In-Charge Pediatric Hematology and Oncology Kasturba Medical College, Manipal University, Manipal, Karnataka [email protected]

A iyaz Professor and Head Department of Pediatrics Government Medical College Kozhikode, Kerala [email protected] R

AJ hitkara Head Department of Pediatrics Max Hospital, Pitampura Max Super Speciality Hospital Shalimar Bagh, New Delhi [email protected]/ [email protected]

G R

Anju Virmani Senior Consultant Indraprastha Apollo, Max, Pentamed and Sunderlal Jain Hospitals, Delhi [email protected]

S

S

Aditi inha Senior Research Associate Division of Pediatric Nephrology All India Institute of Medical Sciences New Delhi [email protected]

D

Anita axena Professor Department of Cardiology All India Institute of Medical Sciences New Delhi [email protected] S

A Balachandran Pediatric Pulmonologist Mehta Children’s Hospital, Chetput Chennai, Tamil Nadu [email protected]/ [email protected]

Archana Kher Consultant Pediatrician Seven Hills Hospital Mumbai, Maharashtra Ex-Professor Pediatrics Seth Gordhandas Sunderdas Medical College and King Edward Memorial Hospital, Mumbai, Maharashtra [email protected]/ [email protected] Arun Bansal Assistant Professor Department of Pediatrics Postgraduate Institute of Medical Education and Research Chandigarh [email protected]

vip.persianss.ir

Arun Phatak Professor and Head Department of Pediatrics KG Patel Children Hospital Vadodara, Gujarat [email protected]

B Bhaskar aju Professor and Head Department of Pediatric Gastroenterology (Retd) Institute of Child Health and Hospital for Children Chennai, Tamil Nadu [email protected]

R

R

Ashok K Patwari Research Professor (International Health), Center for Global Health and Development (CGHD), Boston University School of Public Health Coordinator, CGHD India Country Program, New Delhi [email protected]/ [email protected]

Ashok Kumar Professor, Department of Pediatrics Institute of Medical Sciences Banaras Hindu University Varanasi, Uttar Pradesh [email protected]

Baldev Prajapati Professor Sheth LG General Hospital, AMC MET Medical College, Ahmedabad, Gujarat [email protected] Balvir Tomar Professor and Head Department of Pediatrics NIMS Medical College and Hospital Chancellor and Chairman NIMS University Jaipur, Rajasthan [email protected]/ [email protected] S

Bibek Talukdar Director-Professor and Head Department of Pediatrics Chacha Nehru Bal Chikitsalaya Delhi [email protected] B Nammalwar Professor of Pediatric Nephrolgy (Retd.) Institute of Child Health Hospital for Children Director of Medical Education Mehta Children Hospital Chennai, Tamil Nadu [email protected] R

V d

ti e

n U

Ashok Kapse Pediatrician, Kapse Children Hospital Surat, Gujarat [email protected]

G R

Bala amachandran Consultant and Head Department of Intensive Care and Emergency Medicine Honorary Assistant Medical Director Kanchi Kamakoti Childs Trust Hospital Chennai, Tamil Nadu [email protected]/ [email protected] R

Ashish Bavdekar Associate Professor Consultant Pediatric Gastroenterologist Department of Pediatrics King Edward Memorial Hospital Pune, Maharashtra [email protected]

B Vishnu Bhat Professor of Pediatrics Head of Neonatology Division Jawaharlal Institute of Medical Education and Research Puducherry [email protected]

Bharat Agarwal Head Department of Pediatrics Hematology and Oncology Bai Jerbai Wadia Hospital for Children Mumbai, Maharashtra [email protected]

Brijesh Arora Associate Professor Department of Pediatric Oncology Tata Memorial Hospital Mumbai, Maharashtra [email protected] Thangadorai Senior Clinical Professor of Pediatrics (Retd) Madras Medical College Chennai, Tamil Nadu [email protected] C

Arvind Bagga Professor Department of Pediatrics All India Institute of Medical Sciences New Delhi [email protected]

B Gupta Professor and Head Department of Pediatrics Umaid Hospital Additional Principal Dr SN Medical College Senior Consultant Pediatrics Rajdadisa Hospital, Jodhpur, Rajasthan [email protected] D

ATK au Pediatric Hematologist-Oncologist Professor and Head Department of Pediatrics MS Ramaiah Medical College Bengaluru, Karnataka [email protected]

S

i

r cs iat k

f

oo o Ped tb

Tex

Arun Kumar Gupta Professor and Head Department of Radiodiagnosis All India Institute of Medical Sciences New Delhi [email protected]/ [email protected]

viii

vip.persianss.ir

D

George F Moolayil Consultant Pediatrician Chazhikattu Hospitals Pvt Ltd Thodupuzha, Kerala [email protected]

C

C

Jagdish handra Director-Professor Department of Pediatrics Kalawati Saran Children’s Hospital Lady Hardinge Medical College New Delhi [email protected] Jayakar Thomas Professor and Head Department of Dermatology Sree Balaji Medical College and Hospital Chennai, Tamil Nadu [email protected] Jaydeep houdhury Assistant Professor Department of Pediatrics Institute of Child Health Kolkata, West Bengal [email protected] C

S

Geetha M Clinical Professor Pediatric Gastroenterologist Department of Gastroenterology and Hepatology Amrita Institute of Medical Sciences Kochi, Kerala [email protected]/ [email protected]/ [email protected]

S

D

ti e

Gauri Kapoor Director Pediatric Hematology and Oncology Rajiv Gandhi Cancer Institute and Research Center New Delhi [email protected]

Ishwar Verma Sr Consultant and Director Center of Medical Genetics Sir Ganga Ram Hospital New Delhi [email protected]

Jeeson Unni Editor-in-Chief IAP Drug Formulary Consultant Pediatrician Dr Kunhalu’s Nursing Home Kochi, Kerala [email protected]/ [email protected] C

S

D

igant hastri Pediatrician and CEO Killol Children Hospital, NICU and Vaccination Center Surat, Gujarat [email protected] D

Gadadhar arangi Professor, Department of Pediatrics Hi-Tech Medical College, Pandara Bhubaneswar, Odisha [email protected]

n U

heeraj hah Associate Professor Department of Pediatrics University College of Medical Sciences New Delhi [email protected]

C

D

D D D D

evendra Mishra Associate Professor Department of Pediatrics Maulana Azad Medical College New Delhi [email protected]

G R

V d

D

eepak Ugra Consultant Pediatrician Lilavati Hospital and Research Centre Consultant Pediatrician Hinduja Health Care Pvt. Ltd. Visiting Consultant Pediatrician Breach Candy Hospital Mumbai, Maharashtra [email protected]

nt

eepak Bansal Additional Professor Hematology and Oncology Unit, Advanced Pediatric Center, Postgraduate Institute of Medical Education and Research Chandigarh [email protected]

K Agarwal Ex-Professor and Head Department of Pediatrics Banaras Hindu University Varanasi, Uttar Pradesh Secretary Health Care and Research Association Noida, Uttar Pradesh [email protected]

Indira Agarwal Professor and Head Department of Child Health Christian Medical College Vellore, Tamil Nadu [email protected]/ [email protected]

ibut

ivya Prabhat Head Department of ENT Wadia Children Hospital Mumbai, Maharashtra [email protected]

D

Vijayasekaran Associate Professor in Pediatrics and Respiratory Diseases Madras Medical College, ICH and HC Pediatric Pulmonologist and Bronchoscopist [email protected]

ors

H Paramesh Pediatric Pulmonologist Environmentalist, Medical Director Lakeside Medical Center and Hospital Director Lakeside Institute of Child Bengaluru, Karnataka [email protected]

r

ilip Mukherjee Emeritus Professor of Pediatrics Ramakrishna Mission Seva Pratishthan Vivekananda Institute of Medical Sciences, Kolkata, West Bengal [email protected]/ [email protected]/ [email protected]

o

Nirmala Professor and Head Department of Pediatric Gastroenterology Institute of Child Health and Hospital for Children Chennai, Tamil Nadu

ix

vip.persianss.ir

Kanishka as Professor and Head Department of Neonatal and Pediatric Surgery St John’s Medical College St John’s National Academy of Health Sciences Bengaluru, Karnataka [email protected]

Lalitha Kailas Professor and Head Department of Pediatrics SAT Hospital Government Medical College Thiruvananthapuram, Kerala [email protected]

Jugesh hhatwal Professor Department of Pediatrics Vice Principal (Postgraduate Studies) Christian Medical College and Hospital, Ludhiana, Punjab [email protected]

Ketan Parikh Consultant Pediatric Surgeon and Pediatric Laparoscopist Tara Neo-Surg Hospital Jaslok Hospital, LH Hiranandani Memorial Hospital Mumbai, Maharashtra [email protected]/ [email protected]

S

K urendran Surveillance Medical Officer WHO – National Polio Surveillance Project, Chennai [email protected] R

S

Kamer ingh ana Consultant Pediatrics and Pediatric Neurology Command Hospital x Pune, Maharashtra [email protected]

R

KN Agarwal Professor of Pediatrics President Health Care and Research Association for Adolescents Noida, Uttar Pradesh [email protected]

M Vijayakumar Head Department of Pediatric Nephrology Mehta Children’s Hospital Chennai, Tamil Nadu [email protected]

Krishan hugh Director Institute of Health, Sir Ganga Ram Hospital, New Delhi Chairman Department of Pediatrics Director Pediatric Intensive Care Unit [email protected]

Madhulika Kabra Professor Division of Genetics, Department of Pediatrics All India Institute of Medical Sciences New Delhi [email protected]/ [email protected]

D

Kishore Phadke Professor and Head Department of Pediatrics St John’s Medical College Hospital Bengaluru, Karnataka [email protected]

M Jayashree Additional Professor Department of Pediatrics Advanced Pediatrics Center Postgraduate Institute of Medical Education and Research Chandigarh [email protected]

C

C

n U

K Nedunchelian Associate Professor of Pediatrics Government Dharmapuri Medical College Dharmapuri, Tamil Nadu [email protected]

V d

ti e

M Indrashekhar ao Former Medical Superintendent Professor and Head Department of Pediatrics Institute of Child Health Niloufer Hospital Hyderabad, Andhra Pradesh Director Medical Education Government of Andhra Pradesh Member Executive Council NT Rama Rao University of Health Sciences, Vijayawada, Andra Pradesh [email protected]

G R

E

K lizabeth Professor of Pediatrics Consultant in Nutrition Sree Avittom Thirunal Hospital Government Medical College Thiruvananthapuram, Kerala [email protected]

D

JP adhich National Coordinator Breastfeeding Promotion Network of India New Delhi [email protected]/ [email protected]

K Nagaraju Senior Consultant in Pediatric Allergy Kanchi Kamakoti Childs Trust Hospital Chennai, Tamil Nadu [email protected]

S

L ubramanyam Senior Pediatrician and Pulmonologist Mehta Children’s Hospital Chennai, Tamil Nadu [email protected]

D

S

Joseph L Mathew Assistant Professor (Pediatric Pulmonology) Advanced Pediatrics Center Postgraduate Institute of Medical Education and Research Chandigarh [email protected]/ [email protected]

Kamran Afzal Reader Department of Pediatrics Jawaharlal Nehru Medical College Aligarh Muslim University Aligarh, Uttar Pradesh [email protected]

E

i

r cs iat

Tex

tb

k

f

oo o Ped

JN harma Professor and Head Department of Pediatrics Gauhati Medical College Guwahati, Assam [email protected]

vip.persianss.ir

Meenakshi Mehta Head and Consultant Pediatrician Mahatma Gandhi Memorial Hospital Mumbai, Maharashtra [email protected]

R S

Munni ay Additional Professor of Pediatrics Postgraduate Institute of Medical Education and Research Chandigarh [email protected]

nt ibut

S

Naveen Thacker Director Deep Children Hospital and Research Centre Kutch, Gujarat [email protected]/ [email protected] Neelam Mohan Director Department of Pediatric Gastroenterology, Hepatology and Liver Transplantation Medanta – The Medicity Hospital Gurgaon, Haryana [email protected]/ [email protected] Niranjan hendurnikar Consultant Pediatrician KG Patel Children Hospital Vadodara, Gujarat [email protected] Nitin hah Honorary Pediatric HematologistOncologist Bai Jerbai Wadia Hospital for Children Mumbai, Maharashtra Consultant Pediatrician PD Hinduja Hospital Mumbai, Maharashtra [email protected] S

D

Meena P esai Honorary Consultant Endocrinology Division Bai Jerbai Wadia Hospital for Children and Institute of Child Health and Research Centre Honorary Pediatrician Sir HN Hospital and Research Centre Honorary Medical Advisor Sir HN Medical Research Society Mumbai, Maharashtra [email protected]

M Mahadeviah Honorary Professor Kempegowda Institute of Medical Sciences Honorary Consultant in Developmental Neurology Indira Gandhi Institute of Child Health Honorary Medical Director Spastics Society of Karnataka Bengaluru, Karnataka [email protected] R

Manorama Verma Former Professor of Pediatrics Christian Medical College Ludhiana, Punjab [email protected]

M Lokeshwar Ex-Professor, Department of Pediatrics LTMG Hospital and Medical College Mumbai, Maharashtra [email protected]

Naveen ankhyan Assistant Professor Department of Pediatrics, Advanced Pediatric Center Postgraduate Institute of Medical Education and Research Chandigarh [email protected]

S

S

Manju Mehta Professor (Clinical Psychology) Department of Psychiatry All India Institute of Medical Sciences New Delhi [email protected]

Monjori Mitra Consultant Pediatrician Institute of Child Health Kolkata, West Bengal [email protected]

ors

Malathi athiyasekaran Consultant Pediatric Gastroenterologist Kanchi Kamakoti Childs Trust Hospital Apollo Children’s Hospital Sundaram Medical Foundation Chennai, Tamil Nadu [email protected]

ML Kulkarni Professor and Head Department of Pediatrics JJM Medical College Davanagere, Karnataka [email protected]

r

Madhuri Kanitkar Consultant Pediatrics and Pediatric Nephrology Base Hospital Delhi Cantt, New Delhi Professor and Head Department of Pediatrics Army College of Medical Science New Delhi [email protected]

C

Naveen Jain Associate Professor Department of Neonatology Kerala Institute of Medical Sciences Thiruvananthapuram, Kerala [email protected]

R

MK Nair Professor of Pediatrics and Founder Director, Child Development Centre Thiruvananthapuram, Kerala [email protected]

S

Narendra athi Consultant Pediatrician Rathi Children’s Hospital and Maternity Home Akola, Maharashtra [email protected]

C

O

Milind Tullu Associate Professor Department of Pediatrics Seth Gordhandas Sunderdas Medical College and King Edward Memorial Hospital, Parel, Mumbai, Maharashtra [email protected]

o

Madhumati tiv Pediatric Intensivist Program Director for ISCCM-IAP Certificate Course in Pediatric Intensive Care Kind Edward Memorial Hospital Pune, Maharashtra [email protected]

Narendra Kumar Arora Executive Director The INCLEN Trust International and Child Health and Nutrition Research Initiative Adjunct Professor (Research) Public Health Foundation of India [email protected]

xi

vip.persianss.ir

R

P amachandran Professor of Pediatrics, Sri Ramchandra Medical College and Research Centre, Porur, Chennai Tamil Nadu [email protected] PA Mohammed Kunju Professor and Head Department of Pediatric Neurology Government Medical College Thiruvananthapuram, Kerala [email protected] Pankaj Hari Additional Professor of Pediatrics All India Institute of Medical Sciences New Delhi [email protected] C

Panna houdhury Consultant Pediatrician (Retd.) Lok Nayak Hospital New Delhi [email protected] Parang N Mehta Consultant Pediatrician Mehta Hospital Surat, Gujarat [email protected]

PP Maiya Former Professor and Head Department of Pediatrics MS Ramaiah Medical Teaching Hospital Bengaluru, Karnataka [email protected]

D

C

R

S

S

PK Baskar Director Sri Viveka Institute of Dental Sciences GN Chetty Road T Nagar, Chennai, Tamil Nadu [email protected]

Prisca olaco Professor Department of Pediatrics Mahatma Gandhi Mission’s Medical College Navi Mumbai Consultant Pediatrician and Pediatric Endocrinologist Holy Family Hospital Mumbai, Maharashtra [email protected] P N Menon Consultant and Head Department of Pediatrics Jaber Al-Ahmed Armed Forces Hospital Kuwait Ex-Professor Department of Pediatrics All India Institute of Medical Sciences New Delhi [email protected] S

R

P aghupathy Professor Department of Pediatrics (Pediatric Endocrinology) Indira Gandhi Institute of Child Health Senior Consultant Pediatric Endocrinology Sagar Hospitals Bengaluru, Karnataka [email protected]

Piyush Gupta Professor Department of Pediatrics University College of Medical Sciences Delhi [email protected]

Preeti abadghao Associate Professor Department of Endocrinology Sanjay Gandhi Postgraduate Institute of Medical Sciences Lucknow, Uttar Pradesh [email protected]

Prakash P Kotwal Professor and Head Department of Orthopedics All India Institute of Medical Sciences New Delhi [email protected]

Pukhraj Bafna Head Department of Pediatrics United Hospital and Research Centre Rajnandgaon, Chhattisgarh [email protected]

Pratibha inghi Chief Pediatric Neurology and Neurodevelopment Advanced Pediatrics Centre Postgraduate Institute of Medical Education and Research Chandigarh [email protected]

Purna A Kurkure Professor Emeritus In-Charge After Completion of Therapy (ACT) Clinic Department of Pediatric Oncology Tata Memorial Hospital Mumbai, Maharashtra [email protected]

Praveen Khilnani Head Pediatric Critical Care Services BLK Superspeciality Hospital New Delhi [email protected]

Krishna Kumar Clinical Professor and Head Department of Pediatric Cardiology Amrita Institute of Medical Sciences Kochi, Kerala [email protected]/ [email protected] R

Nupur Ganguly Associate Professor Institute of Child Health Kolkata, West Bengal [email protected]

Paul wamidhas udhakar ussell Professor (Psychiatry) Child and Adolescent Psychiatry Unit Christian Medical College Vellore, Tamil Nadu [email protected]

S

i

r cs iat

Tex

tb

k

f

oo o Ped

Noel Narayanan Formerly Professor and Head Department of Pediatrics Government Medical College Thiruvananthapuram, Kerala [email protected]

xii

vip.persianss.ir

R

anjan Kumar Pejaver Honorary Professor Department of Pediatrics Kalinga Institute of Medical Sciences Consultant Neonatologist Krishna Rajendra Hospital Head of Department St. Philomenas Hospital Bengaluru, Karnataka [email protected]

C

R

nt ibut

S S

R S

S

rinivasan Professor and Head Department of Pediatrics Jawaharlal Institute of Medical Education and Research [email protected]/ [email protected]

R

itabrata Kundu Professor Department of Pediatrics Institute of Child Health Kolkata, West Bengal [email protected]

amesh Consultant Pediatrician BRS Hospitals Chennai, Tamil Nadu [email protected]

K Agarwal Consultant Pediatrician RK Hospital Udaipur, Rajasthan [email protected]/ [email protected]

S

Yamuna Pediatrician and Adolescent Physician Child and Adolescent Clinic Director Centre for Excellence in Parenting Chennai, Tamil Nadu [email protected]

K Anand Head Department of Pediatrics and Neonatology Jaslok Hospital and Research Centre Mumbai, Maharashtra [email protected]

Kamath Consultant Pediatrician Welcare Hospital Ernakulam, Kerala [email protected] SS

RD

Potdar Trustee and Hon. General Secretary Centre for the Study of Social Change. Pediatrician, Jeevak Children Hospital Mumbai, Maharashtra [email protected]

Balasubramanian Senior Consultant Pediatrician Kanchi Kamkoti Childs Trust Hospital Nungambakkam, Chennai, Tamil Nadu [email protected]

R

R

aju P Khubchandani Consultant Pediatrician In-Charge Pediatric Rheumatology Clinic Jaslok Hospital and Research Center Mumbai, Maharashtra [email protected]

R

D

R R

hishikesh Thakre Professor Department of Pediatrics Division of Neonatology Mahatma Gandhi Medical College and Hospital Aurangabad, Maharashtra [email protected]/ [email protected]

R

S

C

R

aju hah Professor and Head GCS Medical College Ankur Institute of Child Health Ahmedabad, Gujarat [email protected]

uchi Nimish Nanavati Professor and Head Department of Neonatology Seth Gordhandas Sunderdas Medical College King Edward Memorial Hospital Mumbai, Maharashtra [email protected]

ailesh Gupta Arushee Childcare Hospital and Ashna Children’s Hospital Mumbai, Maharashtra [email protected]/ [email protected]/ [email protected] S

D

C

R

ekha Mittal Senior Consultant Department of Pediatric Neurology Pushpanjali Crosslay Hospital Ghaziabad, Uttar Pradesh [email protected] R

S

R R R R

ajniti Prasad Associate Professor Department of Pediatrics Institute of Medical Sciences BHU, Varanasi, Uttar Pradesh [email protected]/ [email protected]

ors

ajiv handra Mathur Consultant Pediatrician and Hepatologist Aditya Hospital Hyderabad, Andhra Pradesh [email protected]

ashmi Kumar Professor Department of Pediatrics Chhatrapati Shahuji Maharaj Medical University Lucknow, Uttar Pradesh [email protected]

ohit Agrawal Consultant Pediatrician Kohinoor Hospital and Chandra-Jyoti Children Hospital Mumbai, Maharashtra [email protected]/ [email protected]

r

ajeshwar ayal Professor and Head Department of Pediatrics SN Medical College Agra, Uttar Pradesh [email protected]

ashmi alvi Consultant Pediatric Hematologist-Oncologist Professor and Head Department of Pediatrics Bombay Hospital and Institute of Medical Sciences Mumbai, Maharashtra [email protected]

o

N rivastava Consultant Pediatric Nephrologist Apollo Indraprastha Hospitals New Delhi [email protected]/[email protected]

xiii

vip.persianss.ir

Banerjee Head, Department of Pediatrics Islamia Hospital Kolkata, West Bengal [email protected]

S

S

R

C

S

R

unanda K eddy Consultant Developmental Pediatrics and Community Health Chief NGO Functionary Centre for Applied Research and Education on Neurodevelopmental Impairments and Disability-related Health Initiatives New Delhi Adjunct Faculty, South Asia Centre for Disability Inclusive Development and Research Hyderabad, Andhra Pradesh [email protected]/ [email protected] S

R

S

S S

oonu Udani Head of Section of Pediatrics Hinduja Hospital Mumbai, Maharashtra [email protected]

S

hashidhararao Nagabhushana Clinical Associate, CSI Hospital, Bangaluru Visiting Consultant, Columbia Asia Hospital, Hebbal, Bangaluru, Karnataka [email protected]/ xiv [email protected]

omu ivabalan Consultant Pediatrician and Pulmonologist Sundaram Medical Foundation Chennai, Tamil Nadu [email protected]

ukanta hatterjee Professor and Head Department of Pediatrics In-Charge Adolescent Health Clinic Medical College Kolkata, West Bengal [email protected]/ [email protected]

SR S

rikanta Basu Associate Professor Department of Pediatrics Lady Hardinge Medical College and Kalawati Saran Children’s Hospital New Delhi [email protected]

uneeta Mittal Head Department of Obstetrics and Gynecology Director-in-Charge WHO-CCR in Human Reproduction Chief ART Centre and IVF Facility All India Institute of Medical Sciences New Delhi [email protected] S

S

hashi N Vani Emeritus Professor of Pediatrics and Neonatology Pramukh Swami Medical College Karamsad, Gujarat Professor (Retd.) and Head of Department Pediatrics and Neonatology BJ Medical College Ahmedabad, Gujarat [email protected]

uchitra anjit Senior Consultant and Head Pediatric Intensive Care and Emergency Services Apollo Children’s Hospital Chennai, Tamil Nadu [email protected]

S

S

eema Alam Department of Pediatric Hepatology Institute of Liver and Biliary Sciences New Delhi [email protected]

iddarth amji Director-Professor (Pediatrics) and Head Department of Neonatology Maulana Azad Medical College New Delhi [email protected] S

S

S

avitri hrivastava Director, Department of Pediatric and Congenital Heart Disease Fortis Escorts Heart Institute New Delhi [email protected]

hubha Phadke Professor Department of Medical Genetics Sanjay Gandhi Postgraduate Institute of Medical Sciences Lucknow, Uttar Pradesh [email protected]

rinivas ankaranarayanan Consultant Pediatrics Gastroenterologist Apollo Children’s Hospital Chennai and Kanchi Kamakoti Childs Trust Hospital Chennai, Tamil Nadu [email protected] S

S

heila Bhave Associate Professor Department of Pediatrics King Edward Memorial Hospital Research Centre Pune, Maharashtra [email protected]/[email protected]

S

atya Prakash Yadav Consultant (Pediatric Hematology and Oncology and Bone Marrow Transplant Unit) Department of Pediatrics Institute of Child Health Sir Ganga Ram Hospital, New Delhi [email protected]

heffali Gulati Additional Professor and Chief Child Neurology Division Department of Pediatrics All India Institute of Medical Sciences New Delhi [email protected]/ [email protected] S

S

atinder Aneja Director-Professor Department of Pediatrics Lady Hardinge Medical College New Delhi [email protected] S

i

r cs iat k

f

oo o Ped tb

Tex

andeep B Bavdekar Professor and Head Department of Pediatrics Topiwala National Medical College and BYL Nair Hospital Central Mumbai, Maharashtra [email protected]

vip.persianss.ir

Tanmay Amladi Consultant Pediatrician, Zulekha Hospital, Al Nahda, Dubai, UAE Tanu inghal Consultant Pediatrics and Infectious Disease, Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute Mumbai, Maharashtra [email protected]/ [email protected]

C

S

nt ibut

Uday B Nadkarni Consultant, Pediatric Intensive Care Seven Hills Hospital Mumbai, Maharashtra [email protected] Ujjal Poddar Associate Professor of Pediatrics Gastroenterology, Sanjay Gandhi Postgraduate, Institute of Medical Sciences, Lucknow, Uttar Pradesh [email protected] Uma Ali Head of Nephrology Division and PICU, BJ Wadia Hospital for Children, Mumbai Consultant Pediatrician, Lilavati Hospital Mumbai, Jupiter Hospital Thane, Maharashtra [email protected] Upreet haliwal Professor of Ophthalmology University College of Medical Sciences and GTB Hospital, Delhi [email protected] D

Utpal Kant ingh Professor and Head Department of Pediatrics Nalanda Medical College Patna, Bihar [email protected] S

R

T avikumar Consultant Pediatrician Kanchi Kamakoti Childs Trust Hospital Chennai, Tamil Nadu [email protected]

S

R

S S S

T Jacob John Emeritus Professor, Department of Virology, Christian Medical College and Hospital, Vellore, NA District Tamil Nadu [email protected]

S

S

ushil K Kabra Professor, Pediatric Pulmonology Division Department of Pediatrics, All India Institute of Medical Sciences New Delhi [email protected]

S

S

S

S

usan Uthup Additional Professor Department of Pediatric Nephrology Government Medical College Thiruvananthapuram, Kerala Honorary Consultant Pediatric Nephrologist Sree Chitra Thirunal Institute of Medical Science and Technology Thiruvananthapuram, Kerala [email protected]/ [email protected]

wati Y Bhave Senior Visiting Consultant, Advanced Center of Pediatrics Indraprastha Apollo Hospital, New Delhi Advisor Apollo Hospital Research and Education Foundation (AHERF) Former Professor of Pediatrics BJ Medical College, Pune, Maharashtra [email protected]

R

S S S S

urjit ingh Professor Department of Pediatrics Advanced Pediatrics Centre Postgraduate Institute of Medical Education and Research Chandigarh [email protected]

warna ekha Bhat Professor, Pediatrics and Neonatology Head of the Department of Neonatology St Johns Medical College and Hospital Bengaluru, Karnataka [email protected]

Tulika eth Assistant Professor of Hematology All India Institute of Medical Sciences New Delhi [email protected]

ors

unit inghi Professor and Head Department of Pediatric Emergency and Intensive Care, Advanced Pediatrics Centre Postgraduate Institute of Medical Education and Research Chandigarh [email protected]

ushmita Banerjee Consultant, Calcutta Medical Research Institute and Institute of Child Health Kolkata, West Bengal [email protected]

TU ukumaran Professor of Pediatrics Pushpagiri Institute of Medical Sciences, Thiruvalla, Kerala [email protected]

r

unil Karande Professor and In-Charge Learning Disability Clinic Department of Pediatrics Seth Gordhandas Sunderdas Medical College and King Edward Memorial Hospital Mumbai, Maharashtra [email protected]

ushila ussell Lecturer in Clinical Psychology Department of Psychiatry Child and Adolescent Psychiatry Unit Christian Medical College Vellore, Tamil Nadu [email protected]

o

unil Gomber Professor Department of Pediatrics UCMS and GTB Hospital Delhi [email protected]

Vaman Khadilkar Consultant Pediatric Endocrinologist Jehangir Hospital, Pune and Bombay Hospital Mumbai, Maharashtra Assistant Professor Pediatric Endocrinology Bharati Vidyapeeth Medical College Pune, Maharashtra [email protected]

xv

vip.persianss.ir

Vijay Yewale Consultant Pediatrician and Neonatologist, Dr. Yewale’s Multispecialty Hospital for Children Mumbai, Maharashtra Honarary Pediatrician Mathadi Hospital Trust Consultant Pediatrician, Fortis Hospital [email protected]

S

V avikumar Formerly Professor of Pediatric Surgery, Coimbatore Medical College Consultant Pediatric Surgeon G Kuppuswami Naidu Memorial Hospital, Coimbatore, Tamil Nadu [email protected] R

D

S

Vibhawari ani Ex-Professor of Pediatrics, Ex-Dean Government Medical College Nagpur, Maharashtra [email protected]

Vimlesh eth Retired Senior Professor and Head Department of Pediatrics All India Institute of Medical Sciences New Delhi [email protected]/ [email protected] R

S

VH ankar Consultant Geneticist, Genetic Clinic Associate Professor of Pediatrics Department of Pediatrics, SAT Hospital, Medical College Thiruvananthapuram, Kerala [email protected]

Vrajesh Udani Consultant, Child Neurology and Epilepsy, PD Hinduja National Hospital Mumbai, Maharashtra [email protected]

S

YK Amdekar Medical Director BJ Wadia Hospital for Children Mumbai and Consultant Pediatrician Jaslok Hospital and Research Center Mumbai, Maharashtra [email protected] Yogesh Govil (Late) Professor, Department of Pediatrics Chhatrapati Shahuji Maharaj Medical University (Upgraded King George’s Medical College) Lucknow, Uttar Pradesh [email protected]/ [email protected] C

Vikram atta Professor of Neonatology Department of Pediatrics Lady Hardinge Medical College New Delhi Secretary, National Neonatology Forum, India [email protected]

V ankaranarayanan Honorary Director of Pediatric Medical Education and HOD and Senior Consultant Department of Pediatric Gastroenterology and Hepatology Kanchi Kamakoti Childs Trust Hospital Chennai, Tamil Nadu [email protected] S

Vijayalakshmi Bhatia Professor of Endocrinology Sanjay Gandhi Postgraduate Institute of Medical Sciences Lucknow, Uttar Pradesh [email protected] D

S

Ved Prakash houdhry Director, Sunflag Pahuja Centre for Blood Disorders Sunflag Hospital [email protected] C

i

r cs iat k

f

oo o Ped tb

Tex

Varinder ingh Professor of Pediatrics Lady Hardinge Medical College and Associated Kalawati Saran Children’s Hospital New Delhi [email protected]

Zulfiqar A Bhutta Professor and Founding Chair Division of Women and Child Health The Aga Khan University Karachi, Pakistan [email protected]

xvi

vip.persianss.ir

Foreword









It gives me great pleasure to write the foreword for IAP Textbook of Pediatrics (Fifth Edition), a prestigious publication of Indian Academy of Pediatrics. We, as Pediatricians, are dealing with the health of more than 50 percent of the population. Infant and childhood morbidity continue to be very high and adolescent health problems are still not adequately addressed by medical profession. Thus, it is imperative that all of us need to have sound training to look after the children from birth to 18 years. The book has fulfilled this void very well. All previous editions of this book have been very popular amongst undergraduate and postgraduate students. The book has been well received in many developing countries having similar health scenario. Contributors of this book are renowned experts in their respective fields. An invariable problem with multiauthored texts is the diversity of presentations adopted by a multitude of contributors. Since inception, Dr A Parthasarathy, Editorin-Chief, an accomplished academician with tremendous zeal, managed this issue by carefully crafting a common editorial style. In this venture, he has been aptly assisted by other very well-known academicians, Dr PSN Menon, Dr Piyush Gupta and Dr MKC Nair, along with many highly reputed chapter editors. Keeping with time, the text of the book has been aptly modified and all the chapters have been thoroughly revised. The book is designed to be relevant to the need of the developing countries. Emphasis has been on common prevalent conditions though allied subjects seen in children have also received due attention. Section on Community Pediatrics deserves special mention as most textbooks with Western approach hardly provide any information in this respect. The book certainly will continue to remain as a very prestigious publication of Indian Academy of Pediatrics.

C

P Bansal President Indian Academy of Pediatrics 2013

vip.persianss.ir

vip.persianss.ir

Foreword







It indeed is a proud privilege and great honor for me to write the foreword for IAP Textbook of Pediatrics, an ultimate academic publication of the Indian Academy of Pediatrics, which is nothing less of an international class. If “Nelson” is considered as the “Bible of Pediatrics”, this creation should surely be taken as “Gita/Quran of Pediatrics”, at least for the pediatric health professionals of South Asia. The Indian Academy of Pediatrics is always been a front runner in dissemination of knowledge and academics being its citadel. The revolutionary concept, envisaged way back in the late 1990s, has undergone many revisions, and, today; the Fifth Edition is getting released on the 50th Golden Jubilee Year of Indian Academy of Pediatrics; will imprint itself in the golden letters in the golden history of Indian Academy of Pediatrics at the golden hands of His Excellency Dr APJ Abdul Kalam. This mission of Indian Academy of Pediatrics is a vision towards the cause of every pediatrician, a student, a practitioner or an academician alike, which may translate into better child health care services and reduction in child mortality… a humble attempt to achieve MDG-4. In its Fifth Edition, this textbook slowly but surely inching towards the “Nelson’s” textbook. Almost the entire gambit of pediatrics is being brilliantly and aesthetically crafted in this reader-friendly textbook by the editors. Latest and updated information is encompassed and compiled in their respective subspecialty sections by a team of learned section editors. The authors who contributed are Key Academic Opinion Leaders in their respective fields. A dedicated team of astute academicians, such as Dr A Parthasarathy, Editor-in-Chief, Dr PSN Menon, Chief Academic Editor, Dr Piyush Gupta and Dr MKC Nair, Academic Editors, have probably put in the best of their academic life for the last 2 years to bring out this “immortal” Fifth Edition of IAP Textbook of Pediatrics. My heartfelt gratitude, tributes and innumerable salutes to this “Immortal quartet”. R

ohit Agrawal President Indian Academy of Pediatrics 2012

vip.persianss.ir

vip.persianss.ir

Foreword



It is my pleasure and privilege to write the foreword for the Fifth Edition of the IAP Textbook of Pediatrics. This textbook was published as an action plan of Dr A Parthasarathy who continues to excel as the Editor-in-Chief for the Fifth Edition of this remarkable book. The book is not only an accepted reference book of Pediatrics in India but is also widely accepted by all the academicians, postgraduates and practitioners around the country. There are a lot of unique features included in the Fifth Edition. Every chapter has been updated and rewritten by experts in their respective fields with a great acumen. I congratulate the dynamic editors Dr PSN Menon, Dr Piyush Gupta and Dr MKC Nair for their hard work to release the book during the Golden Jubilee Year of Indian Academy of Pediatrics in Pedicon (Kolkata, West Bengal, India). I also congratulate Dr Rohit Agrawal, President, IAP 2012, and Dr CP Bansal, President, IAP 2013, and all the contributors who have made this wonderful book possible.

S

TU ukumaran President Indian Academy of Pediatrics 2011

vip.persianss.ir

vip.persianss.ir

Preface to the Fifth Edition











The Editorial Board dedicates this new revised and updated Fifth Edition of the IAP Textbook of Pediatrics to children and adolescents—the future citizens of India whose Care and Nurture are our goal, concern and commitment. In a way, it is also our humble homage to the Indian Academy of Pediatrics, which has been in the forefront for the care of children of all hues and colors, at all ages and without barriers; and had trained and nurtured us as responsible professionals to care them with love and compassion. It is our honor and privilege that this dedication coincides with the time when the Indian Academy of Pediatrics is celebrating 50 years of its existence and active service. The last decade has witnessed rapid strides in medicine and technological advances in biological sciences. The specialty of Pediatrics has made substantial advances in preventive and therapeutic care. New vistas for care of children have been opened and many fresh thrust areas requiring our immediate or continued attention to decrease both morbidity and mortality have been delineated. These have provided fresh impetus to revise knowledge, harvest new information and thus continue the process of learning. The opportunities are limitless and it is for us to take advantage of these new frontiers. There was a felt need for publication of an updated Fifth Edition of this book after a gap of 4 years. This was also prompted by the enthusiastic response to the previous editions from practicing pediatricians, postgraduates, undergraduates as well as faculty of Departments of Pediatrics throughout India and other SAARC countries. It has been our endeavor to present this subject in a simplified and practical manner to provide adequate clinical guidance to pediatricians so that children derive the benefits of early diagnosis and optimal treatment. The basic simple and practical outline of the book is retained. We have tried our best to oversee that the “art and science” of Clinical Pediatrics maintains its central position without being overshadowed by newer technical advances. The main focus of the new edition has now shifted to practicing pediatricians as against the original concept of undergraduates. The Indian Academy of Pediatrics now has a membership of nearly 20,000 pediatricians and there is a felt need to continuously update our members—young and old—with the rapid advances in the field and the new national initiatives in the care of children. The previous editions were very popular amongst practitioners of pediatrics as well as family medicine. This is not a watered down version; efforts have also been made to keep a balance with incorporation of the curricular needs of the undergraduates and postgraduates and teachers in Pediatrics. The book represents a substantial revision and reorganization of the text based on a complete review of the field of Pediatrics. A major change in this edition is the concerted effort to condense the contents of the Fifth Edition in a single volume instead of the 1,565 pages of the Fourth Edition in two volumes. The number of chapters has been brought down to 21 from the original 36 in the previous edition. These changes are in accordance with the wishes expressed by several readers and also suggestions received from the publisher. The entire contents of this textbook were formulated to provide relevant clinical information and national priorities at one site. The text of the new edition was written afresh or revised and edited accordingly by selected reputed experts in respective fields. A judicious balance of old and young authors was made by retaining most authors as far as possible and at the same time inducting new experts in chosen fields. Almost all the chapters have been thoroughly revised and updated in a lucid and readable style. The Editorial Board and the Indian Academy of Pediatrics are indebted to these experts who made their valuable contributions without any remuneration or honorarium for their services to the Indian Academy of Pediatrics. Some of the thrust areas in this edition refer to felt needs of our country. It is our constant endeavor to inform, educate and update the reader about the current status of national community-oriented initiatives for children. We are justifiably proud of our achievements in preventive care with attainment of the status of polio-free nation among others, early this year. Strategies for incorporating newer technology and better coverage of immunization to contain communicable diseases and further reduction in disease burden, especially in the underprivileged areas of our society hence find a better thrust in this book. While we are able to combat infections at a better footing than before, the continued onslaught of newer infections and pandemics, especially from resistant microorganisms is our national priority. As pediatricians, our major commitment is to ensure a decrease in mortality and morbidity, especially among the under-fives. The use of simple strategies to improve health status (e.g. uniform growth charts and low cost food supplements); more complex issues such as the increasing disease burden of new epidemics of non-infectious chronic

vip.persianss.ir



i

r cs iat k

f

oo o Ped tb

Tex

diseases like obesity, especially in the urban India; and screening for congenital disorders in the newborns thus find adequate mention in this book. Several new chapters have been added keeping in mind the changing concepts of Pediatric care in the global scenario. The Indian Academy of Pediatrics would like to place on record its appreciation to all the authors who have taken great pains to contribute and/or edit reader-friendly sections with practical guidelines amidst their busy professional and academic schedules. We are proud to inform you that we have about 75 new authors in this edition of the textbook with valuable contributions. We welcome the new section editors of this edition and gratefully acknowledge the efforts and time spent by Senior Editors and Chapter Editors who have devoted great deal of their time reviewing and editing the manuscripts. We do sincerely hope their efforts have made the book look more concise and precise. It is our earnest hope that this book will help in early diagnosis and efficient management leading to optimal outcome and improving the quality of patient care. Your valuable suggestions and comments are most welcome for improving the contents and quality of future editions.

C

S

A Parthasarathy P N Menon Piyush Gupta MK Nair

xxiv

vip.persianss.ir

Preface to the First Edition









Pediatrics has grown and developed with significant milestones in preventive and therapeutic care over the past few decades. The WHO and UNICEF in their Primary Health Care (PHC) approach have given due importance for effective child survival programs. So the medical students need to be well oriented towards these approaches in Pediatrics as the future middle level managers in Primary Health Care. Several luminaries in the Indian pediatric scenario have contributed their might in bringing out books for the undergraduate medical students. However, the rapid advances made in the various pediatric subspecialties have necessitated the updating of these books from time to time. Nevertheless, the need for a full-fledged textbook was felt for long. The Indian Academy of Pediatrics thought it fit to shoulder the responsibility of bringing out such a need-based Textbook in Pediatrics for medical students. Our erudite and enthusiastic editors and contributors made it possible at a record time. The Indian Academy of Pediatrics owes its gratitude to all these experts for their worthy contribution. The book has been divided into several sections. A few chapters included in this book are entirely newer concepts, which are not usually found in the conventional pediatric textbooks. It has also worthy annexures to the main contents. However, editing the text to suit the needs of medical students was a Himalayan task. The idea is to equip the medical students with adequate knowledge in Pediatrics in order to make them confident to shoulder the responsibilities concerned with preventive and curative Pediatrics. Thus, it is hoped that the practitioners of Pediatric Medicine will benefit from this book. We are confident that this book will serve the needs of medical students, especially at a time when the Medical Council of India has made Pediatrics as a major examination subject. Thus, the publication of the book is not only timely but also out of necessity. The Indian Academy of Pediatrics would like to place on record its appreciation to the senior editors, chapter editors, contributors, and staff members of Indian Academy of Pediatrics Central Secretariat for help rendered in the creation of this book and M/s Jaypee Brothers Medical Publishers (P) Ltd., New Delhi, India, for their excellent cooperation in bringing out the First Edition at record time.

C

S

A Parthasarathy P N Menon MK Nair

vip.persianss.ir

Acknowledgments















We are indebted to the President and Members of the Executive Board 1997 of the Indian Academy of Pediatrics for the initiation and completion of the project of bringing out the first ever IAP Textbook of Pediatrics for the medical students and practitioners. We are also thankful to the successive Presidents and Members of the Executive Boards 1998–2012 for their encouragement in sustaining the project toward the publication of the Fifth Edition. Our grateful thanks go to the various contributors and chapter editors, past and present teachers of Pediatrics who have contributed to the Fifth Edition and made the “Himalayan task” of production of the current edition a reality by their precise and updated text. We are indebted to the faculty and residents of All India Institute of Medical Sciences, New Delhi, for shaping the contents of the First Edition which has made the production of the subsequent editions an easy task and to Ms Manju, Ms Chitra and Ms Suman for their assistance in typing and drafting the text of the book for the First Edition. The secretarial and organizational skills of Mr Joseph A Gonzalves and his supportive staff of Indian Academy of Pediatrics Central Office, Mumbai, the meticulous guidance and cooperation and coordination provided by Dr Rohit Agrawal, IAP President 2012, Dr TU Sukumaran, IAP President 2011, Dr CP Bansal, IAP President 2013, Dr Sailesh Gupta, IAP Honorary Secretary General 2012−13, Dr Pravin J Mehta, IAP Honorary Treasurer 2012−13, are gratefully acknowledged. Our grateful thanks to Mrs Nirmala, Mr R Janardhanan, Dr Pratibha, Ms Shruthi Pavana, Ms Swathi Pavana, Mr P Balaji, Ms Kavya and Ms Mahiya for secretarial assistance and to Mr D Prakash, Mr Ajay Kumar, Mr Shukla, Mr Sathiyathasan and Mrs Umadevi Sathish for help rendered in scrutiny and formatting of manuscripts, typesetting the editorial corrections, correspondence assistance, etc. to the Editor-in-Chief at Chennai need special mention for their untiring assistance in correspondence and formatting of the book with updated contents. Our grateful thanks are due to Mr Shaji Jacob Ninan and Dr Nazeer Ahmed at Kuwait for the assistance rendered to the Chief Academic Editor in editing, scrutinizing and proofreading of the individual chapter files. Our special thanks to Ms Anju Kumari and Mr RG Bhardwaj for assisting Dr Piyush Gupta, Academic Editor, at New Delhi and Mr N Asokan and Ms S Suja for assisting Dr MKC Nair, Academic Editor, at Thiruvananthapuram. Our sincere and grateful thanks go to M/s Jaypee Brothers Medical Publishers (P) Ltd., New Delhi, especially Shri Jitendar P Vij (Group Chairman); Mr Ankit Vij (Managing Director); Mr Tarun Duneja (Director-Publishing); Mr Tarun Vij (DirectorPharma); Mr KK Raman (Production Manager); Mrs Samina Khan (PA to the Director-Publishing); Dr Richa Saxena (Editorin-Chief); Mr Syed Amir Haider and Mr Amit Rai (Medical Editors); Ms Kamlesh Rawat (Proofreader); Mr Hemant Kumar (Typesetter); Mr Santosh Kumar Pandit (Graphic Designer) and other members of Jaypee Head Office, New Delhi, for their untiring coordination efforts in the production of the Fifth Edition. We also place on record our sincere appreciation of the help rendered by the local branch managers of the Jaypee Brothers—Mr Mukherjee (Branch Manager) and Mr Jayanandan (Senior Commissioning Editor), Chennai Branch Office, for the help rendered to the Editor-in-Chief and Academic Editors. All attempts have been made to acknowledge the sources of information and illustrations. Inadvertent omission, if any, is regretted.

vip.persianss.ir



C

s

1

are

E







2

E





1.2 History licitation

4





YK Amdekar

E



16



C

ommunication and ounseling

C

1.4

Parang N Mehta E





23



rc ountries

26



b

N

ew orn

R



2.1 Neonatal Nomenclature

27



S

ection ditor : iddarth amji E

C

are of the

20

C

Zulfiqar A Bhutta

S

Saa

hild Health in

C

1.7



O

R

esearch in ffice Practice

Narendra Kumar Arora





1.6



R

C

are of the Normal Newborn

32





B Vishnu Bhat

S





2.4 Identification and Approach to a ick Newborn



2.5 Low Birth Weight

2.7 Neonatal Hyperbilirubinemia

44



42

C

2.6 Parent ounseling





Vikram Datta









Ranjan Kumar Pejaver





2.8 Neonatal Infections

48





Rhishikesh Thakre

S



55



D

espiratory istress

R

2.10

Ashok Kumar



2.11 Bleeding Neonate

59







51

Ruchi Nimish Nanavati





2.9 Neonatal eizures





Naveen Jain



35 39





Swarna Rekha Bhat







Siddarth Ramji

2.3



28





Siddarth Ramji

2.2 Neonatal esuscitation





63

evelopment

D



JN Sharma

E

ection ditor : KN Agarwal



C

D









Vaman Khadilkar

D

S



D



DK Agarwal

evelopmental elay



Madhulika Kabra





MKC Nair

3.7 Failure to Thrive



D

3.6

D





3.5 Normal evelopment



KN Agarwal





3.4 Growth and exual evelopment in Puberty



86



S

h



3.3 W o Under-5 Growth tandards 2006



68

KN Agarwal





3.2 Growth—Birth to Puberty



64

Dilip Mukherjee





3.1 Growth and evelopment: Basic oncepts



S

18

George F Moolayil





1.5 Legal and thical Issues to Pediatric Practice







7

C Thangadorai





1.3 Physical xamination





T Ravikumar

Section 3: Growth and



hild

1.1 Pediatrics—Yesterday, Today and Tomorrow

Section 2:



oncept of

C

s



ic

ection ditor : Piyush Gupta

S



Section 1: Ba

C

Contents

95 101 104 113

vip.persianss.ir

S



C



127



RK Anand

4.4 Water oluble Vitamins

150



134



4.3 Malnutrition



S







Meenakshi Mehta

S





4.5 Fat oluble Vitamins

153





Dheeraj Shah

E





4.6 Trace lements

158





Panna Choudhury



s

162

R

s

s

D

I

I

163



S



5.1 Basics of Immune ystem



i ea e

ection ditor : itabrata Kundu E

I

S

mmunity, mmunization and nfectiou

s



KE Elizabeth

Section 5:

D





5.2 Primary Immunodeficiency isorders

170





Naveen Thacker



ationale of election of Vaccines in National Immunization Program and IAP Immunization Time Table



R

5.4



174

T Jacob John





5.3 The Principles and Practice of Immunization





Tanu Singhal

S



Vijay Yewale

E





5.4.1 Polio nd Game: Global and National Perspective













S



5.7 Vaccine torage and Handling

198







194

Anju Aggarwal



Digant D Shastri E



AJ Chitkara

221

5.13 Kala-azar (Visceral Leishmaniasis)

226





C







240



hikungunya Fever

Rajniti Prasad

243

AK Dutta

hickenpox (Varicella)

247

Lalitha Kailas

5.19 Mumps

252

5.20

254



250



5.18 Measles R



ubella



Jaydeep Choudhury Nupur Ganguly

abies

256



R



5.21



AP Dubey





228



engue Illnesses

Ashok Kapse



C



Utpal Kant Singh



D





5.17



R







Ritabrata Kundu





S

C







Jayakar Thomas

5.16 Poliomyelitis





5.12 Malaria

O

218



5.11 An Approach to a hild with Fever and kin ash

5.15



209 213

PP Maiya

5.14

Ritabrata Kundu



Milind S Tullu



DS

S



5.22 Pediatric Human Immunodeficiency Virus (HIV) Infection or Acquired Immunodeficiency yndrome (AI )



203

5.10 Fever and Fever of Unknown rigin







5.9 Future Vaccines, Adjuvants and Immunization Techniques



M Indrashekhar Rao





5.8 Adverse vents Following Immunization and Their Management







Tanmay Amladi

5.6 Non UIP Vaccines in India



188 189 192





K Surendran

5.4.1.1 Global Perspective 5.4.1.2 National Perspective 5.5 The Universal Immunization Program (UIP) in India

177 188











4.2 Infant and Young hild Feeding



f k

118

JP Dadhich



oo o Ped

D

E

S

iat

ection ditor : heeraj hah 4.1 Breastfeeding

tb

Tex xxviii

117

utrition



N



i

r cs

Section 4:

259

vip.persianss.ir





275





5.24.2 Tuberculous Lymphadenitis

280





Vimlesh Seth





282





Vimlesh Seth

5.24.3 Neurotuberculosis



5.24.4 Abdominal Tuberculosis

287





Vimlesh Seth

C





5.24.5 National Tuberculosis ontrol Program D







297



304





5.29 Leprosy



309





Rajeshwar Dayal

5.30 Pandemic Influenza

314

tem



Sy

S

C



6.1 Neuroanatomic Localization in hildren

315



E

ection ditor : Pratibha inghi

s

ervou

s

entral

N

s

s

s

i ea e of

C



Nitin Shah



Vrajesh Udani

S

C

D



PA Mohammed Kunju

D





D



C



D



367



E

ncephalitis and ncephalopathies E



Rashmi Kumar

S



384



hildhood Brain Tumors

Kamer Singh Rana

388



aised Intracranial Pressure

Sunit Singhi

hildhood troke

392

Satinder Aneja

C



395

Bibek Talukdar

C

D

407



erebral Palsy, Mental etardation and Autism

MS Mahadeviah



Sunanda K Reddy



E

D

D



6.18 Global evelopmental elay and arly Intervention



D

D

D



Sunil Karande





6.17 Learning isabilities and Attention eficit Hyperactivity isorder





C

6.16

398

Sheffali Gulati

R





6.15 Neuromuscular isorders in hildren







6.14 Floppy Infant- linical Approach



C





6.13

379



R





6.12

oma in hildren

Arun Bansal

S

C





6.11

371

Pratibha Singhi C

C





6.10

C







6.9 Infections of the entral Nervous ystem







Anoop Verma

6.8



362







Munni Ray

6.7 Movement isorders



358





Rekha Mittal

6.6 Headaches and Migraines in hildren



339



E

eizures, pilepsy and Nonepileptic vents E

S

6.5



336

Devendra Mishra





6.4 Neurocutaneous isorders

320 330

Naveen Sankhyan





6.3 Neurodegenerative isorders







6.2 Prenatal evelopment and entral Nervous ystem Malformations



D

S

301



D

R

ickettsial iseases in India

Narendra Rathi





5.28



299

Raju C Shah





5.27 Tetanus





Sailesh Gupta

Section 6:



294

Monjori Mitra





iphtheria





Vimlesh Seth

5.26 Pertussis



290





Vimlesh Seth

5.25

E

5.24.1 Pulmonary Tuberculosis





Vimlesh Seth

S

C



275





5.24 Tuberculosis in hildren

O



C





273

S Ramesh

c NT NT

5.23 Leptospirosis in hildren

412 415

xxix

vip.persianss.ir



472



s

s

R

e piratory Sy tem S

S

R

477

espiratory istress

481

D

R



R









D

S

uppurative Lung isease



L Subramanyam





8.10 Bronchial Asthma

H Paramesh

D



9.1 Acute iarrhea Ashok K Patwari



s

s

ankaranarayanan

S

E

S

s

s

s

s

C

S







Neelam Mohan

540



onstipation in hildren C

C



C



D









BD Gupta

VR Ravikumar

546



9.9 Abdominal Pain in hildren

D

543



9.8 Hirschsprung isease



C





Kanishka Das



Srinivas Sankaranarayanan

D



550 D

R

C

linical elevance of Liver Function Tests and Imaging Modalities in Hepatobiliary isorders



Geetha M



Sheila Bhave

555

9.13

562



558



9.12 Viral Hepatitis



VS Sankaranarayanan



S

C



A Riyaz



C

C

hronic Liver isease and irrhosis of Liver in hildren D

C



Malathi Sathiyasekaran

9.14 Neonatal holestasis yndromes



535

D

9.6 Gastrointestinal Bleeding in hildren

R

531

C

9.5 Parasitic Bowel isease



527







522

Shashidhararao Nagabhushana





518

9.4 Vomiting in hildren and Gastroesophageal eflux isease

9.11



511 512

9.10 Hepatomegaly: A Practical iagnostic Approach



505

Ujjal Poddar

9.7



501



C

D

C







9.3 Malabsorption yndrome in hildren

498

iver

9.2 Persistent and hronic iarrhea in hildren Gadadhar Sarangi

494



D

S

n U

i ea e of Ga trointe tinal Sy tem and

ection ditor : V

L

ti e



E





mpyema

8.9









A Balachandran

489





Rohit Agrawal

Varinder Singh

487

V d

Deepak Ugra

8.8

484



C

roup







Sushil K Kabra

8.7 Bronchiolitis



D





Madhumati Otiv

8.6 Pneumonia



G R

R

D

iagnostic Procedures and Investigations in espiratory iseases

D Vijayasekaran

8.5



473



E

E

xamination of the espiratory ystem

8.4 Upper espiratory Tract Infections







467

TU Sukumaran

8.3





D

C

C

s

s

s

i ea e of







C

D

459

ection ditor : TU ukumaran

8.2





iseases of ndocardium, Myocardium and Pericardium E

D



D

450

ardiac Arrhythmias in hildren

S

ongestive Heart Failure: iagnosis and Management

S Srinivasan

8.1



s

s

C

S

C R C





443

Srikanta Basu

7.6



heumatic Fever

Anita Saxena

7.5



427







f k

ongenital Heart isease

Savitri Shrivastava

7.4

Section 9:

420

R Krishna Kumar





oo o Ped

s

s

E

S

C

linical Approach to Pediatric ardiology

7.3

Section 8:

419

S Srinivasan

7.2

tb

Tex xxx

ardiova cular Sy tem

ection ditor : rikanta Basu 7.1



iat

i ea e of s

D



i

r cs

Section 7:

570

vip.persianss.ir









604



9.20

C

598



9.19 Acute Pancreatitis in hildren

D Nirmala



C

C

hronic Pancreatitis in hildren

Seema Alam

O

609

E



ract

T

rinary

U

s

s

s

i ea e of Kidney and

ection ditor : Arvind Bagga



E

613





10.4 Glomerulonephritis k

amran Afzal

enal Vasculitis and Lupus Nephritis

R

10.5

Sushmita Banerjee







10.6 Acute Kidney Injury S





10.7 Nephrotic yndrome

V d





Arvind Bagga



Arvind Bagga



D



Indira Agarwal





Uma Ali



10.14 Hypertension s

s

s



641 645 648 653 655





C

D

678



11.3 Nutritional Anemias in Infancy and hildhood

S



684

S



690





Nitin Shah

ed ell Membrane isorders

704



700

D

S

ickle ell isease C



MR Lokeshwar



D

R

C



Vibhawari S Dani

708



E

R

ed ell nzymopathies C



Rashmi Dalvi

11.8

D



11.11 Platelet and Vascular isorders Sunil Gomber



oagulation isorders and Hemophilia

Sunil Gomber



C



716

Bharat Agarwal D





11.9 Autoimmune Hemolytic Anemia





Jagdish Chandra

11.10



C





Niranjan Shendurnikar

11.7



637

R







631

671

Ved Prakash Choudhry

11.6



627

11.2 An Approach to iagnosis of Anemia in hildren

11.5 Thalassemia yndromes



624

666

Ved Prakash Choudhry

11.4 Bone Marrow Failure yndrome



620

665

11.1 An Approach to Anemia in the Newborn



618

658

ection ditor : M Lokeshwar E

D

i ea e of Blood



Pankaj Hari

S





10.13 Nephrolithiasis



n U





10.12 Asymptomatic Hematuria



R

R

D

C R

enal Tubular iseases

Aditi Sinha





10.11



ti e

hronic Kidney isease, enal eplacement Therapies

Kishore D Phadke





10.10



O

D

isorders of Micturition and bstructive Uropathy

Madhuri Kanitkar





10.9

Section 11:

R

R



M Vijayakumar





10.8 Urinary Tract Infection, Vesicoureteric eflux and eflux Nephropathy



G R



D

evelopmental Anomalies

Susan Uthup





10.3





S



D

iagnostic valuation of Kidney and Urinary Tract

RN Srivastava





10.2



610

BR Nammalwar





10.1 The Kidney: tructure and Function



S

596

Ashish Bavdekar



D

Section 10:

C

D

C

ommon Metabolic Liver isorders in hildren

E



Balvir S Tomar

S



C

582



9.17 Ascites



O

D

C

579

Rajiv Chandra Mathur

9.18





574

9.16 Fulminant Hepatic Failure







holestatic isorders in lder hildren

B Bhaskar Raju

c NT NT

9.15

721 724

xxxi

vip.persianss.ir



745



s

g

M

ali nancie

747



12.2 Leukemia

C







C





12.3 Lymphomas in hildren

753





Anupama Borker





12.4 Wilms Tumor

761





Brijesh Arora





S

oft Tissue arcoma

S

12.7





Satya Prakash Yadav

ndocrinolo y





isorders of Pituitary

D

13.2



PSN Menon

D

isorders of Thyroid Gland

Meena P Desai



P Raghupathy

D

S

isorders of exual evelopment

D

13.9

D

iabetes Mellitus

s



R



S

E



D



14.5 Genetic Metabolic isorders

Manorama Verma





C

C

E



820 824

C





MKC Nair

15.2 Family Life ducation

809

880



s

ealth

ection ditor : MK Nair 15.1 Why Adolescent are ounseling? E

A

S

H



Shubha Phadke

dole cent

804

870





Ishwar C Verma

Section 15:

799

866





14.4 Advances in Genetics

794

855



D

C

ommon Genetic isorders

Madhulika Kabra





14.3

788

844



D

C

linical ysmorphology

782

835

ML Kulkarni





14.2

781

834

VH Sankar





S

ection ditor : hubha Phadke 14.1 Basic Genetics

777

 



Vijayalakshmi Bhatia

Section 14: Genetic

773

828





P Raghupathy

13.10





isorders of Adrenocortical Biosynthesis

D

13.8

770

814



Preeti Dabadghao



D

isorders of Adrenal Glands

D

13.7



n U

isorders of Bone and Mineral Homeostasis

Vijayalakshmi Bhatia





13.6





13.5





D

isorders of Puberty

Prisca Colaco





13.4



ti e



O

besity

Anju Virmani





13.3







V d

S

E

isorders of Growth

PSN Menon





13.1

D

S

ection ditor : P N Menon



g

E

Section 13:







Gauri Kapoor

12.8 Pediatric Bone Marrow Transplantation



G R



R

etinoblastoma

Amita Trehan





12.6



765

ATK Rau





12.5 Neuroblastoma





Purna A Kurkure



S

E

746



12.1 Introduction: Malignancies in hildren Purna A Kurkure





C

C





P

ediatric

ection ditor : PA Kurkure





C

E



Section 12:



739

Deepak Bansal



k

734

Anupam Sachdeva

11.14 Transfusion Medicine and omponent Therapy

tb

Tex xxxii

727

isseminated Intravascular oagulation

D

11.13



iat f

oo o Ped

valuation of a hild with Thrombosis

Tulika Seth



i



r cs

11.12

881 882

vip.persianss.ir



S



15.9 Adolescent Friendly chool Initiatives

900



897



15.8 Adolescent Gynecology



S





Suneeta Mittal



Abraham K Paul

Section 16:



g

R

S

C



903



S

E

S

16.1 Approach to a hild with Arthritis





E

S

C









ystemic Lupus rythematosus

Noel Narayanan

16.4 Approach to Vasculitis in hildren



G R

Raju P Khubchandani





16.2 Juvenile Idiopathic Arthritis



Surjit Singh



C

D

R

O



C

D



16.7 Allergic isorders in hildren mer encie S

S



s

ti e

g

s

are and

ection ditor : unit inghi E

S



I

nten ive

E

Section 17:

C



K Nagaraju



V d

ther heumatological isorders of hildhood

Surjit Singh





D



Noel Narayanan





16.5 Kawasaki isease

R

espiratory Failure





17.5

Uday B Nadkarni



D

E





C







C





Bala Ramachandran



Soonu Udani



C







ommon ye Problems

1019

ommon Problems of ar, Nose and Throat

1026



1011

C

ommon rthopedic onditions O

C



E

C

Divya Prabhat



E

C

C

R

S

C



972

990

Upreet Dhaliwal



s

bs

C

E

P





940

ommon urgical Problems

Prakash P Kotwal

18.5

932

982

Ketan Parikh

18.4

927

ommon Behavioral Problems

Manju Mehta

18.3

926

981

ection ditor : P amachandran

18.2

921

978

Praveen Khilnani

S

C





17.10 Transport of ritically Ill hild

18.1

919

974



C





17.9 Neurocritical are





17.8 Monitoring in the Pediatric Intensive are Unit

pecialtie

916

969

Suchitra Ranjit

ediatric Su

914

958

Sunit Singhi

Section 18:

911

955

17.6 Fluids, lectrolytes and Acid-base isorders 17.7 Assisted Ventilation in hildren

907

947





S

hock

M Jayashree



C

C





Late Yogesh C Govil

17.4



n U

ardiopulmonary esuscitation R



S Balasubramanian

17.3



O

ommon Poisonings in hildhood

C

17.2



S



Krishan Chugh





17.1 The Need, cope and rganization of Pediatric Intensive are Units





Raju P Khubchandani

16.6

O

902

heumatolo y

ection ditor : urjit ingh

16.3

S

894



C

ommon Health Problems in Adolescents

Sukanta Chatterjee





15.7



892

MKC Nair





15.6 Adolescent exuality









Paul Swamidhas Sudhakar Russell



889





15.5 Adolescent Mental Health





C





Sushila Russell

E

886



15.4 Adolescent ounseling





884

Jugesh Chhatwal

c NT NT

15.3 Adolescent Nutrition

xxxiii

vip.persianss.ir



C

D

S



R

1059 1073



C

E

nvironment and hild Health

H Paramesh

ediatric

1080



P

ommunity

s



C



D

C





Section 19:

E

S





19.1 Vital tatistics

1081



S

ection ditor : Piyush Gupta

C





19.2 Indicators of hild Health

1083





Joseph L Mathew

C

C





19.3 Primary Health are and hild Health

1087





Piyush Gupta

C





19.4 Integrated Management of Neonatal and hildhood Illness R





C

hild Labor

C

SR Banerjee



O

ti e

C



19.12 Prevention and ontrol of Injuries

rocedure

s

E

n U

SS

Kamath



21.1 Basics of Pediatric Therapeutics

D

D

R



D

E



D

D

1122

1155 1158

1167



S

afe Injection Practices

Annexure: rugs and osages



1119

1161

SS Kamath

Jeeson C Unni



C

D

R



ssential rugs in Pediatrics

Jeeson C Unni

21.5



D







21.4

1116

1151

Archana Kher

ational rug Therapy in hildren

1112

1150

21.2 Adverse rug eactions, rug Interactions, Therapeutic rug Monitoring Arun Phatak

1107

1145

Sandeep B Bavdekar

21.3

1105

1126





s

herapeutic

ection ditor : E

P

ediatric

T



Somu Sivabalan

Section 21: S

R



20.2 Physiotherapy and ehabilitation

1096

1125

ommon Procedures in Pediatric Practice

Baldev S Prajapati





20.1

C

S

ection ditor : A Parthasarathy



P

ediatric

P



K Nedunchelian

1093





RD Potdar



C





19.11 Adoption and are of rphans





V d

Meenakshi Mehta



19.10



hild Abuse and Neglect

C

19.9







S Yamuna





19.8 Art of Parenting



C

R

R

ights of the hild

Swati Y Bhave





19.7

Section 20:







Pukhraj Bafna





RK Agarwal

19.6 National Health Programs elated to hild Health



G R





Shashi N Vani

19.5 National ural Health Mission



1090





Piyush Gupta



1053

Arun Kumar Gupta

18.9



ommon ental Problems

18.8 Pediatric adiology



k

1032

PK Baskar





iat f

oo o Ped

18.7

tb

Tex

kin iseases in hildren

Jayakar Thomas



i



r cs

18.6

1175

xxxiv

vip.persianss.ir

Section

1

G R

Basic Concepts of Child Care

ti e

V d

Pediatrics—Yesterday, Today and Tomorrow: YK Amdekar History Elicitation: T Ravikumar Physical Examination: C Thangadorai Communication and Counseling: Parang N Mehta Legal and Ethical Issues to Pediatric Practice: George F Moolayil Research in Office Practice: Narendra Kumar Arora Child Health in Saa Countries: Zulfiqar A Bhutta rc















1.1 1.2 1.3 1.4 1.5 1.6 1.7

Section Editor : Piyush Gupta

n U

vip.persianss.ir





trics

Pediatrics—Yesterday, Today and Tomorrow YK Amdekar

Introduction Care, survival and development of children have always been our concern. The Government of India and Indian Academy of Pediatrics have undertaken several projects over last few decades to improve health of children and it is time to introspect their impact on the ultimate goal.

emography

isease Profile

In the 60s and 70s, antibiotics and vaccines appeared to have controlled infectious diseases. However, infectious diseases have returned with vengeance. Tripartite interaction between environment, microbes and host status decide outcome of infectious disease. There is complex struggle for survival between humans and microbes and microbes are exploiting human behavior. Early and frequent exposure to infections, varying nutritional and immune status, lifestyle changes, global travel and misuse of antibiotics have contributed to resurgence of old infections. Microbes have an inherent ability to mutate, change virulence and acquire resistance to antibiotics. This has made battle against infections more difficult. Besides, new organisms are being recognized. Forty new microbes have been recognized over 2 last 30 years. They include viruses such as rotavirus, ebola virus, hantavirus, parvovirus B19, human T-lymphotropic

Technological dvances A

D

ti e

n U

G R

V d

Due to a rapid rise in population in India that stands at 1.2 billion at present, there exists shortage of health personnel and facilities. Though there has been steady decline in infant and under-5 mortality rate, it is still high and neonatal mortality rate has not much changed over years. Breastfeeding is initiated within first hour only in 25% of neonates and exclusive breast-feeding in first 6 months is practiced in 45% of infants. Only 50% infants are timely weaned. More than 60% of children do visit health facilities for common illnesses but even then 40% have remained underweight, 45% stunted, 23% wasted, 75% anemic with 5% having severe anemia. Two to three percent children under 3 years of age suffer from acute severe malnutrition with 20–30% mortality in this group. In spite of availability of free vaccines in expanded program on immunization (EPI) program, there is no change over last decade in number of fully vaccinated children that stands at less than 50%. Similarly, only 45% children use oral rehydration solution (ORS) during diarrheal episodes. Thus, health indices have not much changed over last decade. It suggests that medical and paramedical personnel do not follow standard simple cost-effective preventive measures, in spite of contact opportunity with the community.

viruses type 1 and 2, hepatitis C and E, H5N1 avian strain of influenza, SARS, human metapneumovirus, H1N1 swine reassortent influenza, bacteria such as Legionella, Campylobacter jejuni, toxin producing staphylococci and streptococci, Borrelia, Helicobacter pylori and parasites such as Cryptosporidium and Cyclospora. Changing epidemiology has been observed in dengue fever, leptospirosis, brucellosis and Rickettsial fever. Due to a breakdown in public health measures, there is resurgence of malaria and tuberculosis. Co-infection with HIV has worsened scenario of tuberculosis. Many malignant conditions are now being attributed to an infectious agent. Hepatocellular carcinoma induced by hepatitis B and C infection and cervical cancer due to human papilloma virus are some of the examples of infection related malignancy. Many diseases of unknown etiology (Kawasaki syndrome, sarcoidosis and diabetes) are suspected to be induced by infections. Infections caused by antibiotic resistant organisms are on the increase. Multidrug resistant Staphylococcus aureus (MRSA), first detected in 1961 in UK, is now known both in nosocomial and community settings. Streptococcus pneumoniae has become resistant to penicillin almost all over the world; surprisingly, penicillin sensitivity is still maintained in India. Problem of resistance is mainly due to misuse of antibiotics in human as well as veterinary medicine and also due to genetic mutation by bacteria. Empirical use of antibiotics, non-adherence to standard protocols by physicians and noncompliant therapy by patients have resulted in multidrug resistant TB, malaria, and typhoid in the community. “ESKAPE” pathogens are problem pathogens—Enterococcus, Staphylococcus aureus, Klebsiella, Acinetobacter, Pseudomonas and enterobacteria species—and are difficult to eradicate. Besides, methicillin and vancomycin resistant Staphylococcus aureus, coagulase negative staphylococci, penicillin resistant pneumococci, macrolide resistant streptococci, multidrug resistant Salmonella and Shigella and extended spectrum betalactamase producing enterococci and Acinetobacter are also being reported more frequently. Non-infective illnesses such as lifestyle diseases, asthma, malignancy, autism spectrum disorders, metabolic disorders and immune deficiency disorders are being increasingly recognized. With a threat of epidemic of obesity, dual burden of malnutrition poses a stiff challenge.

D

e

T x

tboo

o

e

i

k fP d a

1.1

Etiological diagnosis of infections is possible today with modern culture techniques such as BACTEC; and polymerase chain reaction (PCR). Reversed transcription (RT-PCR)—is for amplification of RNA and real time PCR allows quantitative measurement of DNA or RNA. Southern blot is a method of probing a specific DNA sequence in DNA

vip.persianss.ir

sic o cepts o hil re

We need to change focus from disease to health. We must combine traditional wisdom with modern science. Indian Academy of Pediatrics (IAP) has formulated guidelines for standard management of many common diseases that should be followed meticulously. Constant updating is necessary to keep up with scientific advances and they should be used selectively. Science will undergo frequent changes as nothing is constant and ignorance is far more than knowledge. Hence we need to learn, unlearn and relearn.





1. Cavazzana-Calvo M, Thrasher A, Mavilio F. The future of gene therapy. Nature. 2004;427:(6977):779-81. 2. Gupta P, Shah D, Sachdev HPS, et al. National workshop on development of guidelines for effective home based care and treatment of children suffering from severe acute malnutrition. Indian Pediatr. 2008;43:131-9. 3. National Family Health Survey-3. Mumbai: IIPS; 2006. Available from: www.nfhsindia.org/nfhs3.shtml. Accessed 30 July, 2011.





Bibliography



A

d Ca

Pediatricians Must Change

Change in Clinical Practice Before the advent of modern science, physicians healed their patients with commitment, concern, and compassion.

fC

Before the era of super-specialization and technological advance, pediatricians were well trained in basics in clinical medicine. While super-specialization is a boon, it is expected that super specialist is an excellent generalist. New generation of pediatrician has a challenging task of keeping abreast to scientific advances but it should not be at the expense of basics. National Board of Examination has modified examination pattern that tests the candidates more thoroughly in actual life situations and hence training also has been suitably modified. This has been a better step forward in pediatric education.

V d

ti e

n U

G R

Change in Educational Reforms

dvances in Curative and Preventive Management Following a 40-year hiatus in discovering new classes of antibacterial compounds, three new classes of antibiotics have been brought into clinical use: (i) cyclic lipopeptides (daptomycin); (ii) glycylcyclines (tigecycline); and (iii) oxazolidinones (linezolid). Other “new” antibiotics are merely chemically modified old molecules. With increasing antibiotic resistance, “phage” therapy needs to be revived. Better drugs and improved understanding of their usage has modified outcome of malignant diseases. While minimal invasive surgery is fast replacing conventional open surgery, interventional radiology and cardiology provide better alternative to surgery in many conditions. Liver or kidney transplants are now available for such end-stage diseases, besides bone marrow transplant and stem cell therapy. The 20th century saw development of many new vaccines. One-third infectious disease mortality can be prevented by vaccination. Indian EPI program covers only 7 diseases unlike 12 or more diseases covered in national programs of many developed countries. Poor coverage of susceptible population and non-affordability of nonEPI vaccines are major hurdles. It is heartening that polio eradication efforts are nearing success. Till date, there has been just a single case of wild poliomyelitis reported in India in 2011. The time has come to phase out oral polio vaccine (OPV) slowly over next few years and replace with inactivated polio vaccine (IPV).

C n



They treated the child and family and not just the disease. As science developed, physicians started treating disease and not the child. Subsequently, they have learnt to treat diagnostic tests and not disease. Thus, modern clinical practice revolves around diagnostic tests, often without clinical correlation. Unfortunately, scientific advances have not translated into better health indices or quality of life for children at large. This is mainly because majority children need simple approach to their common problems. Modern science cannot replace basic clinical bedside methods. For example, kangaroo care and exclusive breastfeeding will save far more neonates than mechanical ventilation and modern facilities. Oral rehydration solution (ORS) has saved many lives. Nutritional advice, growth monitoring and immunization will contribute to better quality of life. Hence priority in clinical practice should be implementation of basic care and modern advances should be reserved only for most indicated situations.

Ba

sample. Western blot measures antibodies against specific protein as for HIV diagnosis. Gene therapy—introducing functional gene into host genome, replacing abnormal gene—has been successful in cystic fibrosis, hemophilia, sickle cell disease and muscular dystrophy. Transfer of genes in brain using liposomes may prove to be useful for degenerative brain disorders. Inborn errors of metabolism can be diagnosed with certainty at birth and cost-effective screening of common metabolic disorders is now feasible. Availability of several immunological tests have opened up new vista to diagnose otherwise fatal immune deficiency disorders. Imaging modalities have improved tremendously. Ultrasonography (USG) has undergone great advances over last five decades. Color Doppler, digital subtraction angiography and transesophageal USG are some of the newer modalities. Computed tomography (CT) and MRI give anatomical diagnosis while positron emission tomography (PET) scan offers metabolic or functional profile. Many advances have taken place for better resolution and clarity such as diffusion MRI scan, FLAIR image, MR angiography and MR spectroscopy.

3

vip.persianss.ir

E





History licitation

for the presenting complaint and other symptoms that will help in detecting associated conditions and in differential diagnosis should be enquired. The details of treatment given so far and the response should be noted.

ntroduction

The clinical interview and history elicitation are very important tools in the field of Pediatric Medicine. Though it is of much diagnostic value, the very process of interaction with the parents and the child during history taking also has therapeutic value. A pleasant and patient interaction is what any parent desires. There should be fewer distractions during the interview. It is good to use lay terms when talking to the parents and avoid medical terminologies as far as possible. While interviewing, the pediatrician should also observe the child to look for any clinical clues. In pediatrics the most important and distinct aspect is the fact that the person giving the history is usually not the patients (unless the child is about 4 or 5 years old). The parents are the usual source of information and in certain cases when caretakers (other than the parents) are bringing up the children then they will be the source of information.

Past History

C

ti e

n U

ontact History

History of contact with communicable diseases (e.g. TB and, chickenpox) must be elicited with tact and patience. It is often denied and repeated probing with leading questions may be necessary.

ntenatal History

As many illnesses in children have their origin in the womb it is important to get a good history about the period of pregnancy of the index child. A good guide for history elicitation would be the mnemonic of the three “I”s for the mother during pregnancy—illness, irradiation and injections (i.e. drugs). Maternal illness like syphilis, toxoplasmosis, AIDS, rubella, cytomegalovirus and herpes virus infections (STARCH) are associated with specific syndromes in the child. Folic acid supplementation during early pregnancy (the first trimester) prevents neural tube defects like meningomyelocele. B













The main problem or complaints for which the child has been brought for medical attention should be recorded in the informant’s own terms and should be recorded in chronological order with the duration of each complaint. Example: Fever—5 days Vomiting—4 days Loose motions—4 days Decreased urine output—2 days Lethargy—1 day Fast breathing—1 day

C

D

Presenting omplaints

G R

V d

emography

Make a note of the name of the child, his/her age in years (with months and days), parents’ names, address, date and time of interview, informant’s name and relationship to child and their reliability (with regard to the consistency of the information they provide).

Ask for details of relevant past illness, whose knowledge will help you in diagnosis or management. History of occurrence of similar complaints in the past should be noted. For example, in a child with chronic suppurative lung disease or malnutrition, a history of previous exanthematous illness or whooping cough will help; in failure to thrive, a history of recurrent diarrhea; and in a child with fever and seizures, a history of febrile fits will be supportive. Past medication history will also be helpful, e.g. past history of antiepileptic drugs or antituberculous drugs [history of red urine (rifampicin) while on treatment with antiTB drugs]. Information on previous significant hospitalization, accident or surgeries may also be helpful.

A

I

T Ravikumar



t

t

Tex book of Pedia rics

1.2

I

History of Present llness It is important to gather more information and elaborate on the specific presenting complaints. Find out the onset of the complaints (the time up to which the child was apparently well). The evolution of the problems should then flow in a clear, concise, temporal sequence, leading up to the present moment. They should be recorded in order of occurrence and an account of any repeated episodes of 4 symptoms (like seizures or respiratory infections) should be given. Symptoms pertaining to complications expected

irth History

The actual events occurring during delivery must be enquired. The period of gestation, duration of labor, nature of delivery, drugs administered during labor and any complications during delivery (like cord around neck, low Apgar score) should be noted. The duration of every stage of labor (especially the second stage) is important as prolonged labor may result in fetal hypoxia. The normal first stage of labor, from the onset of labor pains to the rupture of membranes, is about 12–24 hours and 6–12 hours in a primi and multigravida respectively. The second stage of labor, from the rupture of membranes to

vip.persianss.ir

D

D

Development is one aspect of pediatrics that makes it unique as compared to adult medicine. The developmental

 

ti e

Gross motor

Fine motor

1 month

Grasp reflex

Starts to smile

2 months

Hands closed

Personal social

Language

Social smile

Cooing

Sustained social smile

Says ‘aah’

Head mostly held up but still Hand open most often bobs forwards

4 months

Head held steady

7 months

Rolls over, creeping-crawling, sits Reaches out and grasps larger Smiles at mirror with hands leaning forwards objects, palmar grasp, transfers objects from hand to hand

Babbling

10 months

Sits without support, cruises

Pincer grasp

Says ‘Baba, mama’

1 year

Walks with one hand held

Releases objects to other person Plays simple ball game on request/gesture

Says 2-3 words with meaning

15 months

Walks alone, crawls upstairs

Makes tower of 3 cubes

Follows simple commands

18 months

Runs stiff, goes upstairs by Makes tower of 4 cubes, initiates Feeds self. Dry by day holding the rails vertical stroke scribbles

2 years

Runs well, walks upstairs and Makes tower of 7 cubes, initiates Handless spoon well and helps Puts sentence of 3 words downstairs one foot at a time, jumps horizontal stoke to undress

2 1/2 years

Goes upstairs with alternating Makes tower of 9 cubes feet

3 years

Rides tricycle, stands momentarily Draws circle, tower of 10 cubes, Dresses and undresses fully Knows age and gender on one foot constructs bridge or 3 cubes when helped with buttons, joins in play

4 years

Hops on one foot, throws ball Draws cross and square, copies Plays with several children with Tells story overhead, climbs well a bridge, constructs a gate of 5 beginning of social interaction, cubes goes to toilet alone

5 years

Skips

Reaches for objects, grasps Excited at sight of food objects and brings to mouth, hands in midline

Draws triangle

B C pt

This history is highly problem oriented and age dependent. Details of the food and dietary patterns help in diagnosing

3 months

n U

Ch

ietetic History

V d

Table 1.2.1 Normal developmental milestones Milestones

G R

ild are

evelopment History

s of

The neonate and its state after birth should be enquired. The term of the child, birth weight, cry, activity and color immediately after birth should be noted. Presence of jaundice or cyanosis, resuscitation steps used (if any) and whether hospitalized after birth must be detailed. Poor cry and lethargy suggest perinatal depression. Paucity of movements of one side or a particular limb may suggest stroke or birth injury. The sucking effort of a child after delivery, usually gives a clue to the neurological status of the child. All infants pass meconium within the first 24 hours, any delay would suggest cystic fibrosis while absence of passage would indicate intentional obstruction or anal atresia. Most infants void urine on the first day while all will void within 48 hours, any delay would point towards an obstruction or agenesis of the renal system.

C

Postnatal History

milestones that a child attains are a good reflection of its physical and neurological maturity. They may be divided into gross motor (head control, rolling over, crawling, sitting, standing, walking, etc.), fine motor or adaptive (grasping reaching transferring object, scribbling, etc.), social (smile, recognition, response to calls, etc.) and language (cooing, babbling, saying syllables, vocabulary, etc.). Tailor the development history to the child’s age. In case of more than one child in the family and if the other siblings are normal, the parents may be asked if the index child’s development mirrored that of the other siblings. The pace of development differs from child to child. As the child grows older the age range of attainment of specific developmental milestones usually widens. For example, a normal child may begin to sit without support between 5 months and 8 months as compared to a normal young infant developing social smile between 6 weeks and 8 weeks. Notice the range of normality becoming more in the older child. Always tabulate the attained milestones against the normal age for attainment of that particular milestone (Table 1.2.1).

asic once

the delivery of the child, is about 1–2 hours in a primi and ½–1 hour in a multigravida. The third stage, which is the delivery of the placenta, lasts about 15 minutes.

Waves bye-bye

Asks for objects by pointing

Helps put things away

Laughs out aloud

Speaks 10 words, identifies parts of the body

Knows full name

Dresses and undresses self. Asks Names 4 colors, repeats sentence question about meaning of words of 10 syllables

5

vip.persianss.ir

amily History

S

three full generations should be recorded. Individuals of the same generations should be recorded in the same horizontal line and numbered from left to right using Arabic numerals. Males are usually placed on the left side of the pedigree and sib-ship listed in both orders. The maternal age at the time of child delivery is also important. Young mothers (less than 18 years) have more chance of preterm, IUGR babies while older mother (more than 32 years) have more chance of having children with Down syndrome and Klinefelter syndrome. In children with disease showing hereditary traits, an enquiry of a much wider circle of relatives must be made.

1 year

1000 kcal/day

20 g/day

2 years

1100 kcal/day

3 years

1200 kcal/day

4 years

1300 kcal/day

30 g/day

5 years

1400 kcal/day

30 g/day

6 years

1500 kcal/day

30 g/day

7 years

1600 kcal/day

40 g/day

8 years

1700 kcal/day

40 g/day

9 years

1800 kcal/day

40 g/day

10 years

1900 kcal/day

40 g/day

11 years

2000 kcal /day

50 g/day

12 years

2100 kcal /day

50 g/day

Adolescent boy

2400 kcal/day

70 g/day

Adolescent girl

2100 kcal/day

65 g/day

20 g/day

I

It is important to note down any known specific drug or food allergies in the child.

6





20 g/day

History of llergies A

2 g/kg

B

100–110 kcal/kg/day

ibliography 1. Barness LA. Pediatric history and physical examination. In: Oski’s Pediatrics—Principles and Practice, McMillan JA, DeAngelis CD, Feigin RD (Eds), 3rd edition. Philadelphia: JB Lippincott Williams and Wilkins; 1999. pp .39-52. 2. Harris R. The examination of children. In: Hutchison’s Clinical Methods, Swash M (Ed), 20th edition. London: ELBS-WB Saunders Co; 1995. pp .365-86. 3. Rees PE. Evaluating the newborn infant: Diagnostic approach. In: Pediatric Clinical Skills, 2nd edition. Philadelphia, Churchill Livingstone; 1997. pp .47-76. 4. Singh M. Pediatrics clinical methods, 1st edition. New Delhi: Sagar Publication; 1992;1-94:174-211.

1 month–year



Protein/day



 

Calories required/day

mmunization History

It is important to record the details of vaccines given to the child in chronological order. The vaccination schedule of the Indian Academy of Pediatrics or at least the Universal Immunization Program should have been followed. Special vaccines (e.g. pulse polio vaccine) must also be enquired. Look for BCG scar at the outer aspect of the left arm at the insertion of the deltoid. If any vaccine has not been given, note the reason for not doing so.



n U

Age

G R

V d

ti e

Table 1.2.2 The recommended caloric and protein requirements

This has a bearing on the type of disease the child might be suffering from and it also helps in planning rehabilitation and treatment options, in addition to helping in giving preventive advice. The following points are worthwhile noting: • Type of family: joint or nuclear • Occupation and employment history • Per capita income (total income divided by the number or dependent family members) • Type of housing, ventilation, toilet and potable water facilities • Psychiatric illness and substance abuse (alcoholism, drugs) in the family • Marital stability • Traditional beliefs and child rearing practices.



The health details of all the family members must be obtained. This includes their gender, present and past health status, treatment taken and their proximity to the child. History of similar illness in the family must be looked for. History of stillbirths or abortions in the family should be noted (habitual abortions occur in maternal syphilis). The birth of abnormal children or children with illness in the family and the reasons for death, if any, of children or young adults in the family should be specifically enquired into. The consanguinity pattern with the degree of relationship may be helpful for genetic disorders. The pedigree chart will help record the family history in a pictorial manner helping us to derive the inheritance pattern of a particular illness. Usually

ocio-economic History



F

t

t

Tex book of Pedia rics

protein energy malnutrition and failure to thrive. In addition it helps us to formulate a diet plan for nutritional rehabilitation of the child for a specific disease. The calculation of the dietary values of the food consumed should provide the actual value of proteins, calories and fats and must mention whether it is sufficient in vitamins, minerals and other micronutrients. Always state the amount of calories and proteins the child is getting for that age as compared to what is recommended (Table 1.2.2) which will help us to calculate the calorie and protein gap. Any problem like feeding difficulty, regurgitation or vomiting should be noted. Any possible natural toxins in the food consumed (fungal aflatoxins, copper, etc.) and feeding patterns during times of illness should also be mentioned. In young children, the complete breastfeeding history including whether colostrum was given, duration of exclusive breastfeeding, weaning pattern, etc. must be elucidated. In children given other milk, it is important to note its dilution, bottle-fed or cup and spoon-fed, frequency and the amount taken during each feeding.

vip.persianss.ir

I

C Thangadorai

ntroduction

C

C

The physical examination in a child is distinct in certain areas from that of the adult. In this discussion, only the facts that differ from that of an adult examination have been stated. As far as possible, no child should cry or get irritated while you are examining. “If a child cries when you examine it, then it’s probably your fault”. This statement by John Apley sums up the care one should take, while handling the child. Physical examination starts even as the child and the attendant walk into the room. Even while eliciting history an observant eye needs to be kept on the child, to watch for clinical clues. There is no definite order to be followed while examining a child. Individualize the examination for every child. Do the invasive and potentially discomforting examinations at the end. Allow the child to be in its most comfortable position, and place it in the mother’s lap. Both the child and the mother must feel secure and confident about the examining doctor.

C

E

P



hysical xamination

Basic oncepts of hild are

1.3

E

General xamination

­



Figure 1.3.1 The different shapes of the head  



Before starting general examination; analyze the history and based on that, look for the specific features that would be relevant to the history which will help you to give a perfect diagnosis. Examining aimlessly is unhelpful, time consuming and irritating to the child and parents. General examination must be thorough from head to foot. Always examine the child’s throat irrespective of the complaint. The golden rule is “Head to foot and back, but forget not the ear, throat and urine”. The sensorium (e.g. stuporous or unconscious in intracranial pathology), posture and attitude (e.g. frog like and limp in a floppy infant), activity (e.g. apathetic in kwashiorkor), looks (e.g. acutely or chronically ill looking) and nutrition (i.e. marasmic, undernourished or moderately nourished) need special mention. Note the shape of the head (Fig. 1.3.1) whether microcephaly, macrocephaly, plagiocephaly (asymmetrical due to lying of the normal infants with their heads persistently on one side), scaphocephaly (boat shaped with increased AP diameter due to premature closure of the sagittal suture), brachycephaly (decreased AP diameter) and oxycephaly (tower-shaped skull). The size of the anterior fontanelle (AF; normal of about 2.5 cm × 2.5 cm) must be measured across the borders as shown in Figure 1.3.2. It normally closes by 9–18 months. Delayed closure is seen in rickets, hypothyroidism, hydrocephalus, Down syndrome, achondroplasia and mucopolysaccharidoses. The AF in a quiet child usually shows a very slight depression from the surface and may pulsate. It is bulging when the child cries and in

hydrocephalus, intracranial hypertension and pseudotumor cerebri, (i.e. after drugs like nalidixic acid, tetracyclines and steroids, and hypervitaminosis A). A sunken fontanel is a sign of dehydration. The posterior fontanel can be felt by running the finger along the sagittal suture to its junction with the lambdoid suture. It normally closes by 2–4 months of age. Ridging and overriding of sutures may normally be seen in the first few hours after birth, due to moulding of the skull during delivery. It may also be seen in craniostenosis due to premature fusion of the sutures. Sutures normally 7 get ossified by 6 months of age (Fig. 1.3.3).

vip.persianss.ir

b

P

ext ook of ediatrics T

 

Figure 1.3.3 The different cranial sutures at birth

 

Figure 1.3.2 The method of measuring anterior fontanelle size

 

Table 1.3.1 Few of the features to be looked for in the head and the associated conditions Features

Important associated conditions

Microcephaly

Familial, craniostenosis, intrauterine infections Trisomy 13 and 21 (Down syndrome)

Macrocephaly

Hydrocephalus, hydranencephaly, porencephaly, some neurodegenerative disorders like metachromatic leukodystrophy, Alexander and Canavan disease, certain intrauterine infections

Frontal bossing

Rickets, congenital syphilis, mucopolysaccharidoses, polysaccharidoses

Cranio-tabes (ping-pong skull)

Physiological (in preterm), rickets, congenital syphilis

Increased inter-pupillary distance (hypertelorism)

Genetic (racial), Down syndrome, Cri-du-chat syndrome, hypothyroidism

Proptosis (Sclera is visible above and below the cornea)

Thyrotoxicosis, orbital leukemic deposits, orbital cellulites, arteriovenous aneurysm (pulsatile), cavernous sinus thrombosis, neurofibromatosis, Crouzon disease

Cataract

Idiopathic, traumatic, intrauterine infections, galactosemia, diabetes mellitus, Down syndrome

Mongoloid eyes (upward slant)

Down syndrome, racial, Prader-Willi syndrome

Antimongoloid slant (downward slant) Turner syndrome, Cri-du-chat syndrome, Treacher-Collins syndrome Depressed nasal bridges

Down syndrome, mucopolysaccharidoses, hypothyroidism, familial

Low set ears

Down syndrome, mucopolysaccharidoses, Turner syndrome, Potter facies (renal agenesis)

Facial puffiness

Renal disorder, kwashiorkor, congestive cardiac failures, angioneurotic edema, cavernous sinus thrombosis

Large tongue

Hypothyroidism, mucopolysaccharidoses, glycogen storage disorders, Down syndrome (relative)

Small mandible

Pierre Robin syndrome

Short neck

Turner syndrome, Down syndrome, mucopolysaccharidoses, hypothyroidism





The Macewen’s sign is useful in clinically detecting raised intracranial tension after the sutures have closed. It is the crack pot sound elicited by percussing the skull. Transillumination of the skull in a dark room is useful in children below one year, to detect subdural effusion or hematoma, if translucency extends beyond 2 cm in the frontal and 1 cm in the occipital region. The face must be observed for any dysmorphic features 8 that may suggest chromosomal or developmental anomalies.

Table 1.3.1 shows some common abnormal features and a few conditions where they are seen. The inter-palpebral line of the eyes when continued horizontally backwards, normally divides the ears into the upper one-third and lower two-thirds. If the line passes above the ears, it is suggestive of low set ears. The neck should be examined for lymph node enlargement, short neck (normal neck length: height ratio is 1:13) and low hair line (below C5). The examination should also include the hair (e.g. pale hair with “flag sign”

vip.persianss.ir

emperature

 

Figure 1.3.4 Method of restraining a child for ENT examination

C

 



R

espiratory ate

The rate of respiration in children is important for diagnosing respiratory disease and certain other nonrespiratory conditions like acidosis and congestive cardiac failure. The rate varies different age groups (Table 1.3.2). But for practical purposes, the guidelines offered in the Reproductive and Child Health (RCH) Program, serve as a good guide to clinically suspect respiratory disease (Table 1.3.3). The pattern of respiration must also be noted whether regular, irregular, Cheyne-Stokes respiration (oscillation of breathing between apnea and hyperpnea with a crescendodecrescendo pattern usually seen in congestive cardiac failure, stages 1 and 2 REM sleep), acidotic, etc. In children the respiration is predominantly abdominothoracic. P

Oral temperature should be taken in children older than 5 years while in infants and younger children the thermometer

may be placed in the axilla. The groin or the rectum can be used. The temperature in the axilla or the groin is about 0.5° C lower and the rectal temperature about 0.5° C higher than the oral temperature. The normal temperature in children is between 36.5° C and 37.5° C. Temperature above 41° C is hyperpyrexia. In conditions like PEM, where hypothermia is a problem, special low reading thermometers (30–40° C) must be used.

ulse ate R

T

S

Vital igns

Figure 1.3.5 Method of examining skin turgor in a child with dehydration

R





C

C

Basic oncepts of hild are

in kwashiorkor), eyes (signs of vitamin A deficiency, icterus, pallor, etc.), ears (examine tympanic membrane for acute otitis media or chronic suppurative otitis media), oral cavity (with special reference to the dentition), extremities (limb deformities in skeletal dysplasia, widened wrists in rickets), nails (koilonychia in anemia) and skin (for pallor, icterus, scabetic lesions, impetigo, etc.). Fundus examination is important to make out papilledema, optic atrophy or retinitis pigmentosa. The mouth is examined for the state of the gums, dental caries and dentition. Delayed dentition may be familial or due to rickets or osteogenesis imperfecta. The correct position for doing the ear, nose and throat examination is shown in Figure 1.3.4, but this should be done preferably at the last. While examining lymph nodes, note the site, size, consistency, tenderness, warmth, matting and scarring. Always remember to examine the drainage areas, for focus of sepsis, if there is significant lymph node enlargement. In older children, discrete and non-tender lymph node enlargement up to 1.5 cm in the cervical and inguinal region may not be significant. The skin is examined (Fig. 1.3.5) by rolling a fold of loosely adherent skin on the abdominal wall between the thumb and forefinger to determine its consistency, the amount of subcutaneous tissue present and the degree of hydration. Examination of the hips must always be carried out in younger children and infants, to look for dislocation. The Ortolani or Barlow procedure is done and the typical clunk of the hip moving in and out of its socket is looked for. Infants and younger children do not exhibit classical pedal edema as they are confined the bed hence sacral edema should be looked for in them.

The pulse is felt mainly over the radial artery at the wrist. The character, regularity and volume must be observed. All the peripheral palpable vessels must be examined. The superficial temporal, carotid, brachial, radial, femoral, popliteal, posterior tibial and dorsalis pedis arteries are easily accessible. In infants and very young children, it may not be possible to palpate the peripheral vessels and in such situations, the heart rate must be counted by auscultation. The normal heart/pulse rates in the different age groups in children are given in Table 1.3.4. For practical purposes, a 9 heart rate of more than 200 per minute in newborns, more

vip.persianss.ir

T

P

Blood ressure

 

Age groups

Normal respiratory rate (per minute)

Newborn

40

1 year

30

5 years

20

10 years

18

b

P

ext ook of ediatrics

Table 1.3.2 Normal respiratory rate in children of different age groups

 

Table 1.3.3 Tachypnea indicating significant respiratory disease (From RCH Program) Age groups

Normal respiratory rate (per minute)

Below 2 months

60 or more

2 to 12 months

50 or more

12 months to 5 years

40 or more

 

Table 1.3.4 The normal heart rates in children of different age groups Age groups

Normal heart rate (per minute)

Newborn

140

1 year

110

3 years

100

8 years

90

10 years

80

than 150 per minute in infants and more than 120 per minute in older children can be taken as significant tachycardia. The radial and femoral pulse must be palpated simultaneously to look for any radio-femoral delay. Remember, the heart rate in a struggling or crying child will be more.

Recording of the blood pressure is one of the most important aspects in a pediatric examination. Yet it is surprising, how often it is neglected. The correct size of the cuff must be used, i.e. the cuff should be two-thirds size of the arm. A large cuff will give an erroneously low reading while a small cuff will give a high reading. In infants, the “flush method” may be used to check the pressure. Here the child’s arm is raised and a tight bandage is applied up to the level of the cuff so as empty the blood from the upper limb. Now, the cuff is inflated and the bandage is removed so that the limb will be pale and bloodless. Deflate the cuff slowly and note the reading at which the skin flushes and the limb becomes red again. This corresponds, approximately to the systolic pressure. In younger children where auscultation at the cubital fossa is difficult, the systolic reading obtained by palpation may suffice. The Doppler technique of measuring blood pressure is more accurate and can be used in children, if available. For every pediatric examination, both the upper limb and lower limb pressures must be recorded to detect coarctation of aorta, while in any child with a suspected cardiac illness, the pressure must be recorded in all four limbs. Normally, the pressure recorded in the lower limbs is about 10 mm Hg higher than the upper limbs. Reserve recording the pressure to the last in order not to irritate or scare the child. Normal blood pressure readings in children in the different age groups are given in Table 1.3.5. Normal blood pressure is defined as systolic and diastolic pressure, less than 90th percentile for that age and sex. Hypertension is defined as average systolic and/or diastolic blood pressure equal to or greater than the 95th percentile for that age and sex, on at least three occasions. As per the American Heart Association (Pediatric Advanced Life Support Course) recommendations, a formula has been devised to calculate the 50th percentile of systolic pressure in children over the age of 2 years [(90 + (2 × age in years)]. The lower limit of

 

Table 1.3.5 Weech’s formulae for estimating weight and height for age of normal children Weights

Kilograms

At birth

3.25

3–12 months

Pounds 7

age in months + 9

age in months + 11

2 1–6 years

(age in years × 2) + 8

7–12 years

(age in years × 7) + 5

(age in years × 5) + 17 (age in years × 7) – 5

2 Heights

Centimeters

Inches

At birth

50

20

At 1 year

75

30

2–12 years

(age in years × 6) + 77

(age in years × 2) + 30

10

vip.persianss.ir

C

eight

The height of the child is a good indicator of the chronicity of any debilitating illness. Height is ideally measured using Harpenden stadiometer. The child should stand against a wall with his bare feet touching each other, the heel, calf, buttock, upper back and occiput touching the wall and

C

 



The child must be weighed during every examination. The weight of the child is also useful for calculating the right dosage of the drugs to be given. The newborn loses up to 10% of its weight during the first week, but regains it in the next few days. The child doubles its birth weight by 4 months, triples it by 1 year and increases it 4 times by 2 years. For calculating expected normal weight, the formula shown in Table 1.3.5, may be used. While interpreting the weight of the child, the present weight must be compared to the expected weight for age and the percentage must be calculated in order to find out which grade of nutrition the child falls under (as per Tables 1.3.6 and 1.3.7). Weight is recorded on a weigh scale which should be frequently checked with standard weights and zero error must be adjusted before weighing.

the child looking straight ahead. A firm scale is pressed to the head to mark the point indicating height. The standing height can be measured for children more than 2 years old, while for younger children, the recumbent length should be measured using the infantometer (Fig. 1.3.6). In exceptions like a child with quadriplegic cerebral palsy, where the height or length could not be measured, the length of various segments of the body are measured separately and added together to get the length. The formula shown in Table 1.3.5 may be used for calculating the height for the age or alternatively the increase in height as shown in Table 1.3.8 may be used for calculating the expected height. While measuring the height, it is also important to measure the upper segment (from the vertex to the pubic symphysis) and the lower segment (from the pubic symphysis to the sole of the foot). The rate of growth of the upper and lower segments varies with age as shown in Table 1.3.9. Hence, any difference in the proportion expected for that age may suggest the presence of specific growth disorders (Table 1.3.10). Stature should also be defined with parent’s height being taken into account, referred to as the mid-parental height.

Weight

Figure 1.3.6 The infantometer method of measuring the length of child

C

A

nthropometry

The measuring of the various anthropometric data is essential for assessing the growth of the child and its nutritional status. It is also important for planning the diet and following up the child especially while recuperating from an illness or during nutritional rehabilitation.

H

Basic oncepts of hild are

the systolic blood pressure has been approximated by the formula 70 + (2 × age in years). An observed fall of 10 mm Hg in systolic pressure suggests a shock.

 

Table 1.3.6 The Wellcome classification of nutritional status Nutritional status

Expected weight for age

Presence of edema

Normal

More than 80%

No

Undernutrition

60–80%

No

Kwashiorkor

60–80%

Yes

Marasmus

Less than 60%

No

Marasmic kwashiorkor

Less than 60%

Yes

 

Table 1.3.7 Indian academy of pediatrics—classification of nutritional status Level of undernutrition*

Expected weight for age

I degree

60–69%

II degree

60–69%

III degree

50–59%

IV degree

Less than 49%

*The prefix ‘k’ is added to indicate presence of edema

11

vip.persianss.ir

ext ook of ediatrics

Age

Height

At birth

50 cm

6 months

+12 cm (62 cm)

1 year

75 cm

2 years

85 cm (86–87 cm)

2–5 years

6–8 cm/year

5 years and above

5 cm/year

b

P

 

Table 1.3.8 Rate of increase in height in children

 

T

Table 1.3.9 Normal upper segment/lower segment ratio in children Age

Upper segment/Lower segment

At birth

1.8/1

3–4 years

1.3/1

9 years

1/1

18 years

0.9/1

 

Figure 1.3.7 Method of measuring head circumference Table 1.3.11 Head circumference growth velocity

 

 

Table 1.3.10 Conditions with altered upper segment/lower segment ratio

Delayed adolescence, hypopituitarism, constitutional dwarfism, nutritional dwarf

High ratio (Upper segment > lower segment)

Hypothyroidism, chondrodystrophy, achondroplasia, Ellis-van Creveld syndrome, Turner syndrome

Low ratio (upper segment < lower segment)

Hurler syndrome, Morquio syndrome, hypogonadism

For girls: Approximate projected adult height (in cm)



Mother’s height + (Father’s height-13) 2

For boys: Approximate projected adult height (in cm) (Mother’s height + 13) + Father’s height













=



2

Till 3 months

2 cm/month

3 months to 1 year

2 cm/3 months (1/3rd of initial velocity)

1 to 3 years

1 cm/6 months (1/12th of initial velocity)

3 to 5 years

1 cm/year (1/24th of initial velocity)

Table 1.3.12 Formula for estimating head circumference in the first year (after Dine et al. ) Normal range of head circumference in cm (5th to 95th percentile) (Length in cm + 9.5) + 2.5 2

35 cm at one year, from 43 cm to 46 cm and from 48 cm to 51 cm at 5 years. The expected head circumference for the age may be calculated from (Tables 1.3.11 and 1.3.12). The adult head size is reached between 5 years and 6 years. Microcephaly is defined as head circumference, more than 3 standard deviations below the mean or less than the 5th percentile for the age and sex. Head size more than the 95th percentile for age suggests macrocephaly.

ead ircumference C

H

The size of the head is a good indicator of the size of its contents, viz. the brain and the ventricles. Any abnormality in the head circumference should alert the doctor towards any problem with the brain or its related structures. Head circumference is measured with a non-stretchable tape passing through the maximum point of the external occupational protuberance posteriorly and a point just above 12 the glabella anteriorly (Fig. 1.3.7). It varies from 32 cm to

hest ircumference C

C









=

Head circumference growth velocity

 

Proportionate (normal ratio for age)





Upper segment/Lower segment Probable disorder ratio

Height for age

This is measured at the level of the nipples (Fig. 1.3.8). In the infant, the chest circumference is lesser than the head circumference by about 2.5 cm, and the two become equal by one year after which the chest circumferences exceeds the head circumference. In undernutrition, the chest circumference remains lower than the head circumference even beyond one and half years whereas in well-nourished children, the chest circumference may exceed the head circumference even before one year.

vip.persianss.ir

This is calculated as shown below: Weight for height (WFH) = (weight of child ÷ weight corresponding to height of child) × 100

Values above 90% are normal, while values below 90% indicate malnutrition and values above 120% indicate overweight. I

C C

Body Mass ndex

C

H

Weight for eight

Basic oncepts of hild are

young children it is 1 cm to 2 cm less than the length or height. It equals the height at 10 years, and after 10 years it is from 1 cm to 2 cm more than the height. Increased arm span is seen in Marfan syndrome and homocystinuria.

It is calculated as: Body mass index (BMI) = Weight in kg ÷ (height in meter)2

For adults, BMI of 18.5–24.99 is considered normal, 25–29.99 is overweight (pre-obese), above 30 is obese and less than 18.5 is underweight (WHO). Body mass index (BMI) less than 16 is severe thinness, 16.00–16.99 is moderate thinness, 17.00–18.49 is mild thinness, 30.00–34.99 is obese class-I, 35.00–39.99 is obese class-II and more than 40 is obese class-III. For children, BMI 85th–95th percentile is overweight while that greater than 95 percentile is obesity.

rm pan S

See Table 1.3.13. E

evelopmental xamination

Here, the developmental history obtained must be confirmed by examining the child for the milestones attained or lost. A large number of accepted methods are available for assessing development. Of this, the Gesse ll developmental scale and the Bayley developmental scale are commonly used. The Baroda Developmental Screening Tests and the Trivandrum Developmental Screening Chart are useful for field assessment of children’s development. The development quotient (DQ) must be calculated separately for motor and mental development. DQ = Developmental age × 100





It is the distance between the tips of the middle fingers with both arms held wide open, i.e. spread apart. Normally, in

Growth atterns

S





Chronological age Developmental evaluation is of special value in children with neurological diseases like neurodegenerative disorders and chromosomal anomalies like Down syndrome. It is also useful for following up children with birth asphyxia or established cerebral palsy and mental retardation. The common developmental/primitive reflexes to be examined are shown in Table 1.3.14. The absence of appearance of the primitive reflexes at the expected time or their persistence beyond the time that they should normally disappear should lead to a suspicion of significant brain damage.

 

Figure 1.3.9 Method of measuring midarm circumference

exual Maturity ating R

A

The midarm circumference (MAC) is taken as the name suggests, at the midpoint between the acromion and the olecranon with the arm hanging by the side of the body (Fig. 1.3.9). It is useful to detect malnutrition in young children (1–4 years). Values more than 13.5 cm may be considered normal, while values less than 12.5 cm indicate significant wasting and undernutrition. Shakir tape is used for measuring MAC in the community and has color bands. Green color indicates MAC is greater than 13.5 cm, yellow color a MAC between 13.5 cm and 12.5 cm and red color a MAC lesser than 12.5 cm.

P

C

Midarm ircumference

D

 

Figure 1.3.8 Method of measuring chest circumference

Sexual maturity rating (SMR) in boys and girls is shown in 13 Table 1.3.15. Please refer to Chapter 3 for more details.

vip.persianss.ir

ext ook of ediatrics T

b

P

 

Table 1.3.13 Growth Patterns Age

Approximate daily weight gain (g)

Growth in length (cm/ month)

Growth in head circumference (cm/month)

0–3 months

30

3.5

2

3–6 months

20

2

1

6–9 months

15

1.5

0.5

9–12 months

12

1.5

0.5

1–3 years

8

1

0.25

4–6 years

8

3 cm/year

1 cm/year

 

Table 1.3.14 Primitive reflexes to be examined during developmental assessment Reflexes

Age of appearance

Age of disappearance

Stepping

Birth

6 weeks

Placing

Birth

6 weeks

Moro

Birth

3 months

Sucking and rooting

Birth

4 months while awake

Palmar grasp

Birth

6 months

Plantar grasp

Birth

10 months

Tonic neck

2 months

4–6 months

Landau

3 months

24 months

Neck righting

4 months

24 months

Parachute

9 months

Persists

SMR Stage

Pubic hair (boys/girls)

Breasts

Penis

Testes

1.

Preadolescent

Preadolescent

Preadolescent

Preadolescent

2.

Sparse, slightly pigmented

Breast and papilla Slight enlargement elevated, areolar diameter increased

Enlarged scrotum, pink

3.

Darker, beginning to curl

Breast and areola enlarged, no contour separation

Longer

Larger

4.

Coarse, curly, abundant but less than adult

Areola and papilla form secondary mound

Larger

Larger, scrotum dark

5.

Adult distribution, spreads to medial surface of  thighs

Mature, nipple projects

Adult size

Adult size

ystemic xamination E

S

 

Table 1.3.15 Sexual maturity rating (SMR) in boys/girls

It is beyond the scope of this chapter to cover the examination of every system in detail, it can be obtained from any standard textbook of clinical examination. Here we have attempted to give salient points in clinical examination that are different in children when compared to adults. S

R

espiratory ystem 14 Inspect the chest wall for any deformities. Costochondral beading is seen in rickets (broad and dome shaped), in scurvy (sharp due to posterior subluxation of the sternum)

and in chondrodystrophy. Look for working of the accessory muscles of respiration, i.e. flaring of alae nasi, sternomastoid contraction, suprasternal and subcostal and intercostal retractions which would indicate dyspnea. Observe for indrawing of the lower ribs (Harrison’s sulcus) which indicates chronic obstructive airway disease like bronchial asthma. Vocal fremitus is rarely of value in young children. Grunting respiration in a child indicates severe respiratory disease. Percuss lightly in infants and small children, tap the chest wall directly rather than using another pleximeter finger. Due to the thin chest wall, the chest is more resonant than adults. Before starting to auscultate, allow the child

vip.persianss.ir

A

bdomen The best place to examine the child’s abdomen is the mother’s lap, preferably while the child is feeding. Even if the child is struggling, it may be put on the mother’s shoulder and the abdomen is palpated from behind by “ballottement”, i.e. palpation just when the child breathes and the abdomen relaxes. Unlike in adults, it is not necessary to fold the legs of the child while palpating the abdomen. Young children normally have a protuberant abdomen. Look for umbilical (which may be normally seen in infants) and inguinal hernia. The liver is normally palpable in children till the age of 4 years, i.e. up to 2 cm below the costal margin. In view of this it is necessary to measure the span of the liver in order to make out actual enlargement. It is carried out by percussing the upper margin of dullness and by palpating the lower edge of the liver in the midclavicular line. The liver span ranges from about 4.5–5 cm at 1 week of age to approximately from 7 cm to 8 cm in males and from 6 cm to 6.5 cm in females by 12 years of age. The spleen may be normally palpable in infants, up to from 2 months to 3 months. Examine the genitalia and scrotum for hydrocele/hernia, intersex, phimosis, undescended testis, hypospadias or epispadias. The anus is examined for anal excoriation and pinworms.

C C C

a



s



n



b







e











D

D

P

he ractice of ifferential iagnosis

During situations when the diagnosis of the child is not very clear (which may be the case quite often), it becomes necessary to make a set of most probable diagnoses. This is called differential diagnosis. This should be based on the history, clinical symptoms and clinical signs that have been elicited. The differential diagnoses thus made will help us to plan out investigations towards proving or disproving each probable cause. Hence, to be of practical value the list should be as short as possible and should only include conditions that could reasonably explain most of the child’s history, symptoms and signs. The list should be given in descending order of probability of the various likely diagnoses, based on the positive and negative points towards each.

­

S

15 Figure 1.3.10 Model diagrammatic representation of recording deep tendon reflexes  

N

ervous ystem Neurological examination of the young child is quite diffi cult, especially sensory examination and requires ingenuity on the part of the doctor to get the child’s cooperation. Developmental screening and assessing of the primitive reflexes should be carried out as already mentioned. Much information regarding the neurological status of the child can be learnt by just observing the child, as the history is being elicited. Coordination is best tested by watching the child,

T

S

C

ardiovascular ystem In a neonate the apical impulse is located slightly outside the midclavicular line in the 4th intercostal space. By 2 years, it comes to the midclavicular line in the 4th intercostal space and comes to the adult position, i.e. 5th intercostal space 1 cm medial to the midclavicular line between 4 years to 7 years. In infants the right ventricles are dominant as compared to adults (where left ventricle is dominant). Due to the short neck of infants and young children, it is difficult to see the jugular venous pulse and pressure. Use a pediatric stethoscope with a small diaphragm to auscultate, as the intercostal spaces are narrow. It is preferable to auscultate the heart while the infant is comfortably sleeping or feeding from the mother. It is easier to hear the normal splitting of the heart sounds and P2 is louder in young children, i.e. less than 5 years. Functional systolic flow murmurs and venous hum are often heard in normal children.

play. Orientation is best tested only in children above 4–5 years. Handedness becomes apparent at about 3 years of age. Signs of meningeal irritation, i.e. neck stiffness, Kernig’s sign, Brudzinski’s sign must be looked for. They may not be present in infants and in the presence of severe undernutrition or overwhelming sepsis. Fundus may be normally pale in infants. Lifting the child gives a good idea about the muscle tone. If it is hypotonic the child will slip through the hands. The plantar reflex may be extensor up to 1 year of age. But persistence of extensor plantar beyond 2 years is definitely pathological. Tendon reflexes in young infants tend to be brisk. The deep tendon reflexes may be diagrammatically represented as shown in Figure 1.3.10, using the notations shown below: 0 = bsent + = luggish ++ = ormal +++ = risk ++++ = xaggerated History taking and clinical skill development in pediatrics are therefore to be learnt by repeated exposure to case interviews and hands on training in physical examination. The more a student gets this type of exposure, the more he can engage himself in self-analysis, which will help him to carry out the clinical examination thoroughly.

Basic oncepts of hild are

to play with your stethoscope, to allay its fears. Often it is less threatening to examine the back of the chest first. Due to the thin chest wall, breath sound are louder in children than in adults and their character is more like the bronchial breathing of adults. This is called puerile breathing. Do not be disheartened with a crying child, as breath sounds can be auscultated better in them. Be careful to distinguish the conducted sounds from the upper respiratory tract as in laryngomalacia, upper respiratory tract infection, etc.

vip.persianss.ir

C

C



ommunication and ounseling

ntroduction

 

Table 1.4.1 Advantages of good communication

S

ommunication kills



The chief goals of communication are: • Creation of a good interpersonal relationship • Facilitating exchange of information • Including patients and parents in decision making. Communication skills contribute to a doctor’s respect, a patient’s belief, and adherence to treatment, among other advantages (Table 1.4.1). A doctor with these skills is more likely to have happy, satisfied patients, than an equally technically competent doctor who does not bother about communication. Even if a pediatrician’s diagnosis and treatment are accurate, thoroughly rational, and successful, poor communication leaves parents unhappy and resentful. 16 This is especially so with chronic or incurable diseases, which are associated with anxiety, stress, and uncertainty for the whole family.



C

B

arriers to Good ommunication

A

The two most important barriers are not knowing what patients want from us (Table 1.4.2), and not realizing the importance of good communication. Some other barriers are: • Lack of time: History taking, physical examination, and prescription writing are seen as essential parts of a clinical encounter. When time is short, it is the communication with parents that is sacrificed • rrogance: Doctors expect patients and their parents to follow commands unquestioningly. Explanations are considered unnecessary • hyness: Shyness, from the patient or the doctor, stands in the way of adequate information being exchanged • Language and jargon: Communication with people speaking different languages can be problematic, and needs a special effort. If an interpreter is used, he should

Table 1.4.2 What patients want  

C

S

C

I

The mportance of ommunication and ounseling kills

Patients and parents do not have the knowledge to judge the quality of a doctor’s examination, diagnosis, or prescription. Most complaints in health care systems, both public and private, arise from poor communication. Parents are angered by the doctor’s refusal to spend time with them, refusal to give explanations, and a lack of courtesy and care. When these are followed by a poor treatment outcome, complaints, quarrels, and legal action are likely. Good communication can play a significant part in avoiding complaints and malpractice claims.

S

These are, quite simply, the skills that allow human beings to communicate effectively. For the pediatrician, communication skills consist of: • The ability to talk with parents. Not to parents, not at parents, but with them. Listening is an essential part; communication must be a two-way process • The ability to communicate well enough with patients and parents to understand their concerns, problems, and beliefs, and to elicit relevant information • The ability to explain the child’s illness clearly, and in language that the parents can easily understand. The treatment options should be explained clearly and completely, so that they can make informed decisions about treatment • The ability to convince parents to follow a treatment plan. This is especially important when embarking on prolonged, expensive, difficult, or culturally unacceptable treatment for a child • The ability to establish a relationship with the parents and child, based on mutual respect and trust • “Soft skills” like being able to put all classes of parents at their ease and being able to generate confidence. These components of a “good bedside manner” were once considered an important attribute of a successful practitioner.

Facilitation of comprehension of medical information Better identification of patients’ needs, perceptions, and expectations Patient satisfaction, leading to regular visits and referrals Feeling of empowerment and control Adherence to treatment plans Loyalty even if treatment is not immediately effective Less chances of complaints and legal action in the event of a mistake Doctors with good communication skills have better clinical and commercial success, less stress and more job satisfaction 

•  •  •  •  •  •  •  • 

Communication skills are a core competence for clinicians. Good communication enhances patients’ understanding and adherence to therapy, and improves outcomes. Poor understanding of the disease and treatment issues by parents is associated with poor outcomes.

•  •  •  •  •  •  •  •  • 

Clarity and directness Listening Honesty More and better information about their illness, treatment plan, and expected outcome More openness about the hazards and side-effects of treatment More information about the relief of symptoms and other concerns Advice on what they can do to help themselves Information on other treatments available A supportive, nonjudgmental, empathetic doctor 

I

Parang N Mehta

C

t

t

Tex book of Pedia rics

1.4

vip.persianss.ir

nformation eeds

B pt C

B

uild confidence: The parents’ efforts and views deserve respect. A little specific praise for the parents’ efforts helps significantly in building confidence and helping parents to cope. Some suggestions for future care improve their confidence that they will be able to manage the situation. Giving false hope is wrong, but information can be given in a positive manner. • peak truth: Parents deserve the truth, but the bald truth can be harsh and shocking. Disclosure must be tempered with common sense and empathy. Sometimes, the whole picture may need to be delivered in small parts spread out over two or more visits. However, withholding information leads to distrust. • e simple and clear: Many people do not comprehend words like “growth” and “tumor”. “Cancer” sounds shoc king, but may be necessary to drive home the problem to parents. • e tolerant: Blame, anger, a sudden outpouring of grief—these are common reactions. They should be met with understanding and support. • mpathize: Parents of sick children are going through a difficult experience. They appreciate the fact that their doctor understands their situation and their difficulties. Many other factors affect communication positively or negatively (Table 1.4.3). Good communication and counseling is an art that is acquired, developed and improved by experience. Efforts in this direction will lead to better patient/parent satisfaction and perhaps better clinical outcomes. It is well to remember that compassion, explanation, and reassurance are valued by patients and their families as much as a diagnosis, treatment, and cure. S

•



C

I

C

S

trategies for mproving ommunication and ounseling

­

B E











1. Ammentorp J, Mainz J, Sabroe S. Patients’ priorities and satisfaction with acute pediatric care. Arch Pediatr Adolesc Med. 2005;159:127-31. 2. Bartel DA, Engler AJ, Natale JE, et al. Working with families of suddenly and critically ill children. Arch Pediatr Adolesc Med. 2000;154:1127-33. 3. Bull SA, Hu XH, Hunkeler EM, et al. Discontinuation of use and switching of antidepressants: influence of patient-physician communication. JAMA. 2002;288:1403-9. 4. Fong J, Longnecker N. Doctor-patient communication: a review. The Ochsner Journal. 2010;10:38-43. 5. Moore PJ, Adler NE, Robertson PA. Medical malpractice: the effect of doctor-patient relations on medical patient perceptions and malpractice intentions. West Med. 2000;173:244-50.

A

ibliography

17



A

B

C

heck what the parents know: Many parents have faulty knowledge, acquired from magazines, lay books, and the internet. • ssess what the parents want to know: Some parents want to know every little fact and detail about their child’s condition. Others simply want a prescription and assurance. • ssess understanding: Emotional distress, poor comprehension skills, language problems, etc. can hinder parents’ comprehension. Understanding can be improved by giving time to absorb, and by repetition. • evelop listening skills: Listening well is an essential part of communication. This requires the provision of adequate time and patience, and the willingness to listen to parents’ concerns. A quiet room, lack of interruptions, provision of chairs for the parents, sitting at an appropriate distance, good eye contact, etc. are helpful. D

•



When faced with a chronic/permanent condition, most parents want to know: • What treatment can achieve for their child-relief of symptoms, prolongation of life, shortening of the course of the disease, etc. • Expected progress of the child during treatment • What to expect by way of improvement, side effects, fresh problems • Chances of complete cure; and • Treatment options. Parents also often want to know about advertised alternative medicine, and it is necessary to explain the unscientific and unproven nature of such “magic remedies”.

­

B

N

I

D

 

be given small bits of information to translate at a time, especially when the prescription is dealt with. Speaking in technical/medical language leaves the parents confused and uninformed. It is important to talk in language that a non-medical person can understand • eafness: Speaking loudly, slowly and distinctly helps parents with hearing impairment. Other useful measures are voice amplification devices, a quiet room, and the use of written communication • Phones: Phones are ubiquitous now, and calls can interrupt and hinder communication terribly.

C

Look at your watch frequently Appear to be in a hurry Use too many medical terms Talk with your hand on the door handle, or foot outside the door Interrupt all the time Start examination and then write out a prescription before the main problem has been identified Give long lectures as explanation Ignore concerns mentioned by parents

l

Don’ts

Greet the child and parent by name Smile Sit down when talking Try to talk in the patient’s language Direct the conversation to relevant directions At the end of the consultation, ask if the parents have any questions Engage the parents in a dialogue Give time for the parents to absorb and understand the content of your explanations, then to ask questions

s of hi d are

Dos

C

 

asic once

Table 1.4.3 Dos and don’ts of communication

vip.persianss.ir

I

E





Legal and thical ssues to Pediatric Practice

Doctor-patient relationship has undergone a sea change from the olden times. Decades back, medical professionals were never questioned in their actions. If any death or other mishaps used to occur, it was attributed to the act of God and the public used to accept it without any adverse reactions. As the times have gone by, medical professionals are being viewed with suspicion and are being dragged into the court for genuine or frivolous reasons. Deterioration in moral values, increased value for materialistic things, commercialization of the profession, higher expectation of the patients, awareness of rights and privileges of the patients, increasing consumer activism, media sensationalism, undue interference by the politicians, etc. contribute to worsening of the doctor-patient relationship. It is prudent on the part of the doctor to have an understanding of what constitute medical negligence and how to prevent it along with improvement in doctor-patient relationship by good ethical practice.

Medical Negligence Medical negligence has been divided into civil negligence and criminal negligence. In civil negligence the affected party takes the doctor to the court for monetary compensation whereas in criminal negligence the prosecution is done by the State and the punishment is usually fine or imprisonment. C

ivil Negligence A doctor can be found guilty under civil negligence by an act of omission or commission in a situation where he has failed to act in a manner which an ordinary professional of his standing would have been expected to act; or acted in a manner which an ordinary professional of his standing would not have been expected to act. The essential components of the modern tort of negligence propounded by Percy and Charlesworth are as follows: • • •

The existence of a duty to take care, which is owed by the defendant to the complainant. The failure to attain that standard of care, prescribed by the law, thereby committing a breach of such duty; and Damage, which is both causally connected with such breach and recognized by the law, has been suffered by the complainant.



Standard of care is the standard of the ordinary skilled man exercising and professing to have that special skill at that particular time. A man need not possess the highest expert skill. It is a well established law that it is sufficient if he exercises the ordinary skill of an ordinary competent man exercising that particular art. He is not negligent if he has acted in accordance with the practice accepted as proper by a responsible body 18 of medical practitioners skilled in that particular art. Failure

in attaining that average standard, leads to a claim for civil negligence. Compensation can be claimed under Law of Contract, Law of Tort or under Consumer Protection Act. C

ntroduction

riminal Negligence

The jurisprudential concept of negligence differs in civil and criminal law. What may be negligence in civil law may not necessarily be negligence in criminal law. For an act to amount to criminal negligence, the degree of negligence should be much higher, i.e. gross or of a very high degree. Negligence which is neither gross nor of a higher degree may provide ground for action in civil law, but cannot form the basis for prosecution. To prosecute a medical professional for negligence under criminal law, it must be shown that the accused did something or failed to do something which in the given facts and circumstances no medical professional in his ordinary senses and prudence would have done or failed to do. The hazard taken by the accused doctor should be of such a nature that the injury which resulted was most likely imminent.

O

I

George F Moolayil

ther Penal Provisions

Action can be taken against the medical professional under the Indian Medical Council Act for failure to follow the provisions of Code of Medical Ethics or under infamous conduct. Penal action also can be taken against the doctors for failing to follow various other rules concerning medical professionals like PNDT, Drugs and Cosmetic Act, Transplantation of Human Organs Act, Biomedical Waste Act, MTP Act, etc.

Precautions to be Taken during Treatment In the practice of medical profession, some precautions have to be taken to make the defense strong in cases of litigations. A doctor may be treating the patient in the correct manner. But many cases have been lost due to failure in proving the same in the Judicial Forums. D

t

t

Tex book of Pedia rics

1.5

ocumentation

A physician should document the following facts: • Name, age, gender, religion and address of the patient • Date, month and year along with the time of examination • All relevant history including details of previous illness along with any history of drug allergy should be noted. If there is no history of drug allergy, it has to be noted. If the patient develops allergy to a drug subsequently, the date of occurrence • The complete examination details including the positive and relevant negative findings

vip.persianss.ir

R

•

•

S

A good ethical practice should have the following elements: • tandard: A doctor should keep up high standard of behavior as prescribed in Code of Medical Ethics, 2002 of Indian Medical Council.

B

C

C pt

n

A

C l C

A I

•

D

•

B



To conclude, a good doctor-patient relationship will decrease the adverse incidents in the practice of medical profession. A good understanding of the laws involving the medical profession and taking proper precautions will help the medical professional in dealing with litigations.



ibliography 1. Bag RK. Medical Negligence and Compensation, 2nd edition, Calcutta: Eastern Law House Pvt. Ltd; 2001. 2. Lahoti RC. Jacob Mathew Vs. State of Punjab. KLT. 2005; 3:967.

thics in Practice and Good octor-Patient elationship

•

D

E

Patient has the right to refuse treatment. In situations where there is refusal of treatment the consequences should be explained to the patients/parents in front of a witness and it is better to get the refusal signed by them. The doctor has also got the right to refer the patients elsewhere if the treatment is refused. If the refusal involves the welfare of a minor or an unborn baby, the courts can override the objections of the parents. When a medical personnel advances a plea that the patient did not give his consent to the treatment suggested by him, the burden is on him to prove that non-administration of the treatment was on account of the refusal to give consent thereto.

•



R

efusal of Treatment

•

hoice: The patient should be given the choice of treatment and doctor. If there is request for reference, proper reference letter should be given. ccessibility: The patient should have accessibility to the best treatment in the hospital or referred to a higher center for better care, if necessary. The doctor will earn the respect of the patient and the relatives by doing so. Nondiscrimination: No discrimination should be shown to the patients on the basis of religion, caste or social standing. Transparency: The doctor should be transparent with the patients and relatives on all possible matters. All matters regarding the bill, etc. should be explained and there should not be any practice of dichotomy (receiving commission from scan centers, lab, etc. for sending the patient for investigations and for referrals). ccountability: The doctor should be accountable to the patient for the treatment. mparting information: The parents and the relatives should be made aware of the condition of the patient. A proper communication can avoid misunderstandings later on. Imparting of information should be done in a sensitive manner. Quality of service: Expectations on the part of the patients and relatives are quite high regarding the quality of medical service. So a medical professional should update his medical knowledge by regular attendance at continuing medical education programs. ealing with complaints: A medical professional should give serious consideration to complaints brought by the patients and relatives and do proper investigation on the complaints. I f the complaint is genuine, an apology to pacify them is necessary and if the complaint is due to some misunderstanding, the matter should be explained.

s of hi d are

Currently the courts unanimously treat lack of informed consent as a matter of negligence of the doctor to disclose necessary information. So the doctor is duty bound to disclose the information as to the risk which can arise from the treatment of the patient. Risk is defined as exposure to a chance of an injury or loss. A majority of the courts require the doctor to disclose information that other physician possess of the same skills or practicing in the same community would disclose in the same situation. Any person of sound mind who has attained the age of 18 years may give a legally valid consent. A consent given by a child under 12 years is invalid, between 12 years and 18 years is valid if the court feels that the patient has understood the implication of the consent. In a situation where there was no proper informed consent and if some medically acceptable complication occurs as a treatment outcome, the doctor can be punished under medical negligence as it can be always claimed that if there was an awareness of the complications the consent would not have been given and hence the doctor will be held responsible for the complications.

•



nformed onsent C

I

• •

A record of investigations advised with reports A provisional diagnosis and a working diagnosis should be there before starting a particular line of management Final diagnosis; and Proper follow-up advice with review dates should be given.

asic o ce

• •

19

vip.persianss.ir

C

S

Next step in the process is to locate the best evidence that attempts to answer the research question identified. There are a number of online information and electronic data bases that the clinician may tap to find the evidence. ‘Medline’ is the most commonly accessed resource through “PubMed”. ‘Google Scholar’ is another search engine to find current evidences to your scientific queries. There are several websites that are modeled to facilitate evidence based medicine. These websites provide consolidated information on a wide range of clinically relevant areas and questions. The critical appraisal of the literature is an important step. The literature should be relevant to the research question and the findings valid and applicable to the settings in which the physician is working. Study design and how the potential biases have been handled are critical factors determining the validity of the findings of an article. Findings of a study on the compliance of anti-tubercular therapy done in Latin America may not be applicable in Indian settings due to various social, cultural and economic factors but do give an idea about the methods for studying the problem in your practice environment.

Generating Hypothesis and tudy esigns D

It is important to distinguish between descriptive and analytic studies. Descriptive studies ask simpler questions about what is going on in the practice environment. For example: • How many children with diarrhea have visible blood in my practice? • What proportion of patients with cough longer than 10 days benefit from bronchodilator therapy? • How many typhoid patients have I seen during MarchJune of the current year? Analytic studies compare one or more interventions or exposures. For example: • What is the duration of diarrhea if antibiotic is not given versus given? • How many doses of anti-pyretic are sufficient after DPT shot?

earching Literature and its ritical ppraisal

S

I

R

D

eveloping esearch dea

A

The majority of pediatric patients in India and developing countries are being treated in ambulatory clinics. If we assemble a cohort of 1000 children, less than 2 are likely to receive treatment in a teaching hospital. Major differences exist between the two settings. Patients seen in office practice have a wide spectrum of severity and differ significantly to that seen in hospitals. Such patient mix precludes generalization and application of results from studies that are mostly done in teaching hospitals. Equally important is the frequently encountered psycho-social morbidity in primary care office practice but uncommon in hospital settings. Thus, research in primary care office practice has several advantages. The sample represents the true population sample and the prevalent problems; accurate character of the disease and natural history; research on psychosocial issues is enabled; allows for health care services research; large sample sizes can be accrued; and it helps to improve quality of care. Office based research makes a clinician feel “alive”; prevents boredom of sitting in the OPD and writing prescriptions and broadens the scope of physician beyond the basic service delivery; excitement of office based research also includes intellectual stimulation, collaboration with academic colleagues, presentations in academic and scientific forums, and a sense of personal fulfillment. Office based research has the potential for improving the effectiveness, efficiency, delivery, feasibility, and reach of clinical care and behavioral interventions.

How is malnutrition associated with increased risk of infections? The best ideas for research come from everyday clinical problems. When an idea comes, think, and reflect on this for a few days or weeks, and think it through your colleague(s). Once research question is defined, it is important to think about how it will be answered. A good research question has four characteristics: feasibility (availability of adequate number of subjects, technical expertise, availability of ancillary services and investigations, time, funds, and scope); interesting to the investigator (passion of physician is essential); newness (confirms or refutes previous findings in your settings, provides new insights to the subject); ethical and relevant (to your practice, scientific knowledge, policy, future research directions).

•

ntroduction



I

Narendra Kumar Arora



20

O

R





esearch in ffice Practice



t

t

Tex book of Pedia rics

1.6

All research questions should lead to formulation of hypotheses. Every hypothesis has also four components: the research question, patient population and its description, study design to be applied and expected outcome. Most of the practicing physicians will require consulting an academic colleague to choose the appropriate study design.

vip.persianss.ir

B C pt



Ch l

R

A ­

­ ­



B

O

R

F

Where to Get unds for ffice ased esearch? Funding for practice based research is definitely a major barrier but not that is insurmountable. Obtaining grants by individuals is more difficult compared to when PBRN approach for funding. Professional associations such as Indian Academy of Pediatrics may be able to facilitate and mobilize funds from donors, foundations and government agencies. Collaboration with universities or medical schools is other option to generate resources. International agencies like AHRQ (The Agency for Healthcare Research and Quality) support PBRN. The support from the industry is justified provided issues related to conflict of interest are taken care of and independence along with scientific rigor of the work are consistently maintained. C

N

R

B

The practice based research network can be considered the research laboratory of the primary care setting. PBRN were initially set-up as a surveillance network to report on common diseases and clinical problems or diseases of public health importance. Subsequently these networks have been involved to answer and shed new light on the complex, adaptive processes of primary care practices and imperatives. The key elements in a PBRN are: participation of moti vated practicing pediatricians, research projects, com munication and the academic framework. The design of such network studies have to be kept simple and easy; study duration should preferably be short with quick results to keep the motivation high, budgetary require ments need to be reasonable and they should not have ethic related complexity. The research coordinator is of critical significance; the person ought to have good research method knowledge, be able to carry network partners together and accomplish the task with consistent implementation of quality assurance steps during execution of study protocol. Managing network brings with it some unique challenges as well. PBRN must receive approval from many ethics committees to conduct research in several locations and practice settings. In a network, there are issues of selection bias, sampling errors, and data collection standardization, which are method aspects that may not be easy to control in busy and varied practice settings.

Office based practitioners can answer many important clinical questions that are not necessarily important for those attending big hospitals. Some examples may include: assessing severity of diarrhea and respiratory tract infections; determining indications for giving antibiotics in febrile child; when to order for investigations after a head concussion; assessing and managing first time pain abdomen which does not respond to antispasmodics; positive Mantoux test in a child with recently treated pulmonary tuberculosis; and many other such difficulties. Sentinel surveillance; counting and characterizing clinical encounters; understanding primary care encounters; comparing approaches to manage patients; modifying patient/clinician behavior; conducting pragmatic clinical trials; comparing approaches to deliver services in practice and monitoring outcomes are some of the broad areas where questions may be developed and research done. Studies such as these most often result from joint contributions of office based physicians and hospital based consultants, and has their major impact in community practice, where the results are most applicable.

C

O

F

R

S

N

S

B R

Practice ased esearch etwork

reas of esearch

i d are

There are difficulties in accomplishing good primary care research: perceived lack of time; translating potential research ideas into coherent research questions and proposals; training in research methods; obtaining ethical clearances; funding; analysis and writing of manuscripts; and working out research collaborations with academic colleagues. Loneliness of solo practitioners and emotional involvement with patients are additional barriers. This has restricted the opportunity for pediatricians and family physicians to improve the quality of care in office practices. During last two decades, there is worldwide movement to encourage and rope in clinicians in primary care to engage in the much needed research. Research networks have been set-up. Practice Based Research Network (PBRN) is a number of primary care clinics grouped together in a structure of a network for the purpose of performing research in the community.



s of

arriers to esearch in ffice Practice and ome olutions- ormation of esearch etworks

Collecting research data in geographically dispersed network environment requires proactive efforts at coordination, accuracy and timely transmission of data. Data collection must not put too much burden on the busy practitioner. Electronic data collection at the point of care is a feasible solution. It is important that data collection methods match the study design for accuracy and comfort.

asic once

Descriptive study designs are: cross-sectional studies; casecontrol; case reports and case series. Natural history of common childhood diseases can also be easily researched. Clinical trials are examples of analytic studies. Simple trials can be done in office practice.

onclusion

This is an era of shifting patient care from hospitals into the community and provides new and challenging opportunities to young and experienced alike for office based research and improved education. Keeping in mind the methodological limitations and potential biases, office 21 based patient material represents the “real world” situations

vip.persianss.ir

t













1. Ipp M. Office based research: a new era and new opportunities. Pediatric Child Health. 2002;7:445-6. 2. John M. Westfall. Card Studies for Observational Research in Practice. Ann Fam Med. 2011;9:63-8. 3. Lindbloom EJ, Ewigman BG, Hickner JM. Practice based research network—the laboratories of primary care research. Medical Care. 2004;42:III45-49.





ibliography

4. Pace WD, Fagnan LJ, David WR. The Agency for Healthcare Research and Quality (AHRQ) Practice Based Research Network (PBRN) relationship: Delivering on an opportunity, challenges, and future direction. J Am Board Fam Med. 2011;24:489-92. 5. Slora EJ, Harris DL, Bocain AB. Wasserman RC. Pediatric Clinical Research Networks: Current Status, Common Challenges, and Potential Solutions. Pediatrics. 2010;126:740-45. 6. Wasserman R, Slora E, Bocian A, et al. Pediatric research in office settings (PROS): A national practice based research network to improve children’s health care. Pediatrics. 1998;102:1350-7.

B

t

Tex book of Pedia rics

and research findings have the possibility of wide community applicability to improve effectiveness, efficiency and equity of care particularly in resource constraint environments.

22

vip.persianss.ir

C

C

S

H

hild ealth in aarc ountries

C





Basic oncepts of hild are

1.7



C C

S

P

ommon eonatal roblems in aarc ountries N

C

Although there has been a reduction in child mortality globally, annually around 8.8 million children die before reaching their fifth birthday, the majority within the neonatal period (Fig. 1.7.1). During the neonatal period, preterm/low birth weight birth, perinatal asphyxia, and sepsis are the leading causes of death whereas infectious diseases like pneumonia, diarrhea, and malaria are the leading causes of death beyond the neonatal period. The majority (82.6%) of these deaths are taking place in South Asia and Africa. Southeast Asia including majority of the SAARC countries accounts for 27% of these deaths. The SAARC region represents countries with overlapping WHO and UNICEF regions and includes parts of Asia with the highest mortality rates. The combined population of SAARC countries is around 1.57 billion, representing approximately 23% of the total world’s population. With the inclusion of Afghanistan, the region now houses countries with the highest child mortality rates in Asia. Of the eight SAARC countries, Afghanistan ranked 2nd (under-5 mortality rate of 199) and Sri Lanka ranked 128th (under-5 mortality rate of 13) in the list of 257 countries (Table 1.7.1). In the year 2000 the Millennium development goals (MDG) were set with

specific targets for mortality reduction by two-thirds by the year 2015. A recent countdown review indicates that out of the five SAARC countries surveyed, only two (Bangladesh and Nepal) are “on track”, two (India and Pakistan) have “insufficient progress”, and one (Afghanistan) had “no progress”.

C

ntroduction

Neonatal deaths account for 50–60% of all infant deaths and the majority of these deaths and simple interventions to address prematurity are available and can be scaled up. These include appropriate care of the mother in pregnancy, use of antenatal steroids in preterm labor, antibiotics for preterm premature rupture of membranes, appropriate care in the first 48 hours after birth. The principal causes of neonatal mortality in the region include perinatal asphyxia, prematurity and sepsis. The exact burden of prematurity is unknown in the region. While it may be difficult to prevent prematurity, cost effective care at birth and prevention of hypothermia are possible. There have been moderate to large scale evaluations of appropriate skin to skin care after birth in

I

Zulfiqar A Bhutta

23  

Figure 1.7.1 Leading causes of death in children under 5-year of age in Southeast Asia

vip.persianss.ir

1990

2009

2

260

199

Pakistan

37

130

87

Bhutan

41

148

79

India

48

116

66

Bangladesh

57

149

52

Nepal

59

142

48

Maldives

118

111

15

Sri Lanka

128

29

13

P P

N

neumonia

Community acquired pneumonia, is one of the leading causes of death in children in the region (majority due to Hib and pneumococcal infections). Hib and pneumococcal vaccines are being rolled out in many SAARC countries either through national investments or GAVI funding. Additional preventive interventions include strengthening of routine immunization, addressing low birth weight, promotion of exclusive breastfeeding, environmental hygiene and reduction in exposure to indoor air pollution. Notwithstanding the role of preventive strategies, there is the importance of appropriate management. Currently most SAARC countries have policies in place for appropriate management through IMNCI trainings of all health care providers looking after children. The basic emphasis of IMNCI training is to recognize pneumonia at an early stage (using a classification on the basis of respiratory rate and presence or absence of subcostal recessions) and initiation of treatment at first or second level of health care. There is also the provision of referral in the event of deterioration or danger signs at presentation. Given the difficulties in referral in some instances, there is also an increasing focus on community-based care (dete ction and management of pneumonia) and preliminary findings from several studies in the region indicate promising outcomes.

24

­

D

iarrhea

Despite vast improvements in our understanding of the risk factors and strategies for the control of diarrheal disease as well as economic growth, diarrhea remains a leading cause of death in children in the SAARC region. Almost half of the childhood diarrhea deaths globally are in five countries of which two (India and Pakistan) are in the SAARC region. A major reason for poor progress is in this area is the relative lack of investment in large scale water and sanitation projects and the fact that between 20% and 30% of the population still does not have access to improved and safe water and over a third of the rural population practices open defecation. Prevention and treatment of dehydration is the key for successful management. Provision of diet and zinc during the diarrheal episode helps not only to treat current episode but prevent malnutrition as well as respiratory morbidity. Given findings that rotavirus infections account for almost a third of all diarrhea deaths, the newer rotavirus vaccines may offer a unique opportunity for prevention of severe diarrheal disease and mortality in the region. O

H

P

ddressing eterminants and ublic ealth ptions D





rural India as well as the use of insulated beds. The use of surfactant and mechanical ventilation may only be available in larger cities in the private sector hospitals but there is great potential for development of low cost surfactant and equipment, especially CPAP units which could make a lot of difference to survival. Perinatal asphyxia can account for up to half of all newborn deaths in the first week of life and can also be associated with significant neonatal morbidity and developmental disability. Many cases of perinatal asphyxia cannot be predicted and hence appropriate facilities for recognition and neonatal resuscitation must be made available in all birthing facilities. While there have been efforts at promoting domiciliary resuscitation in the hands of birth attendants, there are encouraging trends of reduction in perinatal mortality with facility based births. At the very basic level, encouraging facility-based births in the hands of skilled birth attendants with appropriate basic equipment is a key intervention. Although there have been remarkable reductions in neonatal tetanus, neonatal sepsis remains a major cause of morbidity and mortality in newborn and although vertical transmission is possible, the majority of these infections are community acquired and hence potentially preventable. Hand hygiene, and the use of birth kits, followed by appropriate cord care are important interventions to prevent infection. Emerging evidence from various SAARC countries indicates that the use of cord chlorhexidine may be associated with significant reduction in the risk of neonatal omphalitis and sepsis. Given the high rates of infections in community settings and potential delays in recognition and referral, there is increasing attention to community based detection and management of potential neonatal infections and other problems. This includes the training of health care providers in IMNCI, as this approach can be used to recognize and treat serious infections at an early stage and impact outcomes.

A

*Source: State of the World’s Children 2011





Afghanistan

C

 

b T

Value

Rank

P

Country

ommon roblems Beyond eonatal eriod P

ext ook of ediatrics

Table 1.7.1 Comparison of under 5 mortality rates in 1990 and 2009 with present ranking according to current under 5 mortality rates*

Maternal and child undernutrition is an important determinant of child mortality and long-term adverse outcomes including

vip.persianss.ir

C



C C

























1. Acuin CS, Khor GL, Liabsuetrakul T, et al. Maternal, neonatal, and child health in Southeast Asia: towards greater regional collaboration. Lancet. 2011;377:516-25. 2. Arifeen SE, Hoque DM, Akter T, et al. Effect of the Integrated Management of Childhood Illness strategy on childhood mortality and nutrition in a rural area in Bangladesh: a cluster randomised trial. Lancet. 2009;374:393-403.





Bibliography

3. Baqui AH, Arifeen SE, Rosen HE, et al. Community-based validation of assessment of newborn illnesses by trained community health workers in Sylhet district of Bangladesh. Trop Med Int Health. 2009;14:1448-56. 4. Bhandari N, Mazumder S, Taneja S, et al. Effectiveness of zinc supplementation plus oral rehydration salts compared with oral rehydration salts alone as a treatment for acute diarrhea in a primary care setting: a cluster randomized trial. Pediatrics. 2008;121:e1279-85. 5. Bhutta ZA, Ahmed T, Black RE, et al. What works? Interventions for maternal and child undernutrition and survival. Lancet. 2008;371:417-40. 6. Bhutta ZA, Chopra M, Axelson H, et al. Countdown to 2015 decade report (2000-10): taking stock of maternal, newborn, and child survival. Lancet. 2010;375:2032-44. 7. Black RE, Cousens S, Johnson HL, et al. Global, regional, and national causes of child mortality in 2008: a systematic analysis. Lancet. 2010;375:1969-87. 8. Darmstadt GL, Bhutta ZA, Cousens S, et al. Evidence-based, cost-effective interventions: how many newborn babies can we save? Lancet. 2005;365:977-88. 9. Million Death Study Collaborators, Bassani DG, Kumar R, et al. Causes of neonatal and child mortality in India: a nationally representative mortality survey. Lancet. 2010;376:1853-60. 10. UNICEF. State of the world’s children, New York. 2011.

Basic oncepts of hild are

development. Given the high burden of low birth weight, stunting and wasting in the SAARC region, even among countries where mortality rates have improved, there is much need to focus on these determinants. In addition to poverty and maldistribution of resources, the status of women in society, empowerment, ethnicity and race play a critical role in existing inequities in care and access. Most people living in rural areas and urban slums live in abysmal conditions and have limited access to quality health care services. Appropriate targeting, poverty alleviation strategies such as conditional cash transfers, employment schemes and good governance are essential elements in provoking change. An essential element in the quest for targeting the poor, especially in areas with shortage of trained medical staff, is the strategy for task shifting and scaling up of interventions through lay workers and community health workers.

25

vip.persianss.ir

Section

2 Care of the Newborn





















2.1 2.2 2.3 2.4 2.5 2.6 2.7 2.8 2.9 2.10 2.11

























Section Editor : Siddarth Ramji Neonatal Nomenclature: Siddarth Ramji Neonatal Resuscitation: Siddarth Ramji Care of the Normal Newborn: B Vishnu Bhat Identification and Approach to a Sick Newborn: Swarna Rekha Bhat Low Birth Weight: Vikram Datta Parent Counseling: Ranjan Kumar Pejaver Neonatal Hyperbilirubinemia: Rhishikesh Thakre Neonatal Infections: Naveen Jain Neonatal Seizures: Ruchi Nimish Nanavati Respiratory Distress: Ashok Kumar Bleeding Neonate: JN Sharma

vip.persianss.ir

Neonatal Nomenclature

e of t e Ne

ar





C

2.1

h

Siddarth Ramji

Perinatal Period

F

The period from 28 weeks of fetal life up to the first 7 days after birth is the perinatal period.

etal Period

Early Fetal Period

wb rn

Post-term Neonate A neonate who is born after 41 weeks of completed gestation is a post-term neonate.

Birth Weight Related Low Birth Weight Any neonate who at birth has a weight of less than 2,500 g irrespective of gestation is a low birth weight (LBW) neonate.

Very Low Birth Weight A neonate with a birth weight of less than 1,500 g is a very low birth weight (VLBW) neonate.

xtremely Low Birth Weight

A neonate with a birth weight less than 1,000 g is an extremely low birth weight (ELBW) neonate.

Small for Dates (S D)/Small for Gestation Age (SGA)/ Intrauterine Growth Retardation (IUGR) F

This refers to a period of gestation up to 22 weeks. Usually the fetus at this period weighs less than 500 g and has a crown rump length (CRL) of less than 25 cm.

A neonate who is born before 37 weeks of completed gestation is a preterm neonate.

E



Gestational Age Related

Preterm Neonate

o

There are several terms that are used in reference to newborn infants and their care. This section will briefly summarize the commonly used terms in reference to the newborn infant. Newborn/Neonate: Any infant who is up to 28 days of life is termed a newborn or neonate. Early neonatal period: The period from birth up to 7 days of life is the early neonatal period. Late neonatal period: The period from day 7 up to day 28 of life is the late neonatal period.

This refers to the period of gestation from 22 weeks to 27 weeks of fetal life. The fetus usually weighs 500–999 g and has a CRL of 25 cm up to 35 cm.

Late Fetal Period This refers to gestations greater than 27 weeks and the fetus usually weighs 1,000 g or more and has a CRL of at least 35 cm.

Appropriate for Dates (A D)/Appropriate for Gestation Age (AGA) It refers to a neonate whose birth weight is between 10 centile and 90 centile or –2 and +2 standard deviation of the reference standard for a given gestational age.

Large for Dates (L D)/Large for Gestation Age (LGA)

T

F

Neonate

These are synonymous terms often used interchangeably and refer to a neonate whose birth weight is less than 10th centile or less than –2 standard deviation of the reference standard for a given gestational age. F

Intermediate Fetal Period

erm Neonate

A neonate born between 37 weeks and 41 weeks of completed gestation is a term neonate.

It refers to a neonate whose birth weight is greater than 90 centile or greater than +2 standard deviation of the reference standard for a given gestational age.

27

vip.persianss.ir

R





Neonatal esuscitation Siddarth Ramji

B

I R

To identify neonates who would need resuscitation at birth, ask the following two questions: 1. Is the baby crying or breathing? (Identified by observing chest rise, which should be visible and regular) 2. Is there a good muscle tone? (Identified by noting the posture, which should show generalized flexion at upper and lower limbs).

Apgar score is traditionally used to identify birth asphyxia (Table 2.2.1). Birth asphyxia has been defined variously as Apgar score less than 7 at 1 min, no breathing at birth or gasping respiration at birth. It is the most common neonatal emergency in the delivery room. It is estimated that about 5–10% of newborn infants do not establish adequate breathing efforts at birth and need assistance to establish adequate breathing or ventilation. Asphyxia contributes to almost 25% of neonatal deaths.

dentifying Newborns Needing esuscitation at irth



A

B

What is irth sphyxia?

If the answer to both the questions is “Yes”, then the newborn needs to be dried and kept warm. Both these actions can be performed with the newborn lying on the mother’s chest and should not require separation of mother and baby. If the answer is “No” to any of these questions, then the neonate is depressed or nonvigorous and needs resuscitation. The newborn must be assessed to determine the need for one or more of the following actions in sequence:

tiology

The risk factors associated with the need for neonatal resuscitation are given in Table 2.2.2. Not all infants born depressed at birth have these associated risk factors. Thus, all personnel in the delivery room must be trained in basic neonatal resuscitation and every birth must be treated as a potential emergency needing resuscitation at birth.



E



t

t

Tex book of Pedia rics

2.2

 

Table 2.2.1 Apgar score Parameters

0

1

2

Respiratory effort

Absent

Gasping

Good cry

Heart rate

Zero

< 100/min

> 100/min

Color

Central cyanosis

Peripheral cyanosis

Pink

Tone

Flaccid

Partial flexion of extremities

Complete flexion

Response to nasal catheter

None

Grimace

Sneeze

 

Table 2.2.2 Risk factors associated with need for neonatal resuscitation

28

Antepartum factors

Intrapartum factors

•  •  •  •  •  •  •  •  •  •  •  •  •  •  •  • 

•  •  •  •  •  •  •  •  •  •  • 

Maternal diabetes Pregnancy-induced hypertension Anemia Antepartum hemorrhage Maternal infection Maternal cardiac, renal or pulmonary disease Polyhydramnios Oligohydramnios Premature rupture of membranes Post-term gestation Multiple gestation Fetal malformation Maternal substance abuse Diminished fetal activity No antenatal care Maternal age < 16 or > 35 years

Emergency cesarean section Forceps or vacuum-assisted delivery Breech or other abnormal presentation Premature labor Chorioamnionitis Prolonged labor (> 24 hours) Fetal bradycardia Use of general anesthesia Narcotics administered to mother within 4 hours of delivery Meconium-stained amniotic fluid (MSAF) Abruptio placentae or placenta previa

vip.persianss.ir

C

I

A

I

nitial ssessment and ntervention

t

Dry all babies soon after birth. Assess for the breathing and tone of the baby while drying is being done. Neonates who are vigorous should not be separated from the





Initial steps for stabilization (dry and provide warmth, position, assess airway, stimulate to breathe) • Ventilation • Chest compression • Medications. Progression to the next step is initially based on the simultaneous assessment of respiration and heart rate. Progression to the next step occurs only after the successful completion of the preceding step. Approximately 30 seconds are allotted to complete each of the first two steps successfully, re-evaluate and decide whether to progress to the next step. The first minute of life is termed the “golden minute” and is critical to minimize postnatal hypoxia to the neonate (Flow chart 2.2.1 provides the algorithm for neonatal resuscitation). Successful resuscitation in the delivery room needs appropriate equipment. The list is provided in Table 2.2.3.

are of he Ne bor

•

 

Table 2.2.3 List of resuscitation equipment

n

w

Radiant warmer Oxygen (central supply or cylinder) Suction device (mucus extractor or central suction facility) Self-inflating resuscitation bag (250–500 mL) with oxygen reservoir and pop-off valve Face masks (sizes 0 and 1) Laryngoscope (straight blade No. 0, 1) Endotracheal tubes (sizes 2.5 mm, 3 mm, 3.5 mm internal diameter) Drugs: Adrenaline (1:1000), naloxone, normal saline and Ringer’s lactate Intravenous cannula, umbilical catheters, syringes, needles

 

Flow chart 2.2.1 Algorithm for neonatal resuscitation

29

vip.persianss.ir

Positive Pressure Ventilation



Rate of Ventilation If adequate chest rise has been established, the rate of ventilation must be sufficient to provide 40–60 breaths/ min.

Initiate Ventilation with 21% Oxygen (Room Air)



Current evidence strongly supports initiating resuscitation with room air rather than 100% oxygen in infants greater than 32 weeks. In preterms less than 32 weeks, resuscitation may be initiated with 100% oxygen or lower inspired oxygen concentration if blenders are available and oxygen delivery titrated if there is access to pulse oximetry in the delivery room. Infant color in the delivery room is a poor marker of hypoxia. Good response to assisted ventilation after 30 seconds of ventilation is indicated by: • Appearance of spontaneous breathing efforts • Rise in heart rate to more than 100/min. C

hest ompression

Chest compression (cardiac massage) is indicated when the heart rate is less than 60/min after 30 seconds of assisted ventilation. Chest compression requires two personnel—one to continue assisted ventilation and the other to perform chest compressions. The thumb for compression is placed over the lower-third of the sternum. Assisted ventilation and chest compression are coordinated in a ratio of 30 ventilations to 90 chest compressions (3:1). Chest compression is discontinued when heart rate rises to above 60/min. D

Positive pressure ventilation (PPV) can be provided with bag and mask, or bag and endotracheal tube. A small towel 1 inch thick is placed under the infant’s shoulder. The self-inflating bag used for neonates must have a volume

between 250 mL and 500 mL, with an oxygen reservoir. One must select the correct-sized mask (zero size for preterm and size one for term infants) for resuscitation. The correct fit mask when placed over the infant’s face should cover the chin, mouth and nose and not the eyes. Ensure that the mask makes a good seal around the mouth and nose such that when the bag is inflated there is visible chest rise in the infant. If the chest does not rise, the possible reasons could be: • The seal is inadequate • Airway is blocked • Insufficient inflation pressure.

C









t

t

Tex book of Pedia rics

mother and should be kept warm by placing on mother’s chest and covering the baby with a sheet of cloth. In vigorous babies, cord clamping should be delayed for at least 1 min, as there is strong evidence of the benefit of the additional transfer of blood to the baby in preventing anemia in the latter months of infancy. For nonvigorous babies who need resuscitation, clamp and cut the cord immediately and place the baby under a radiant warmer to provide warmth. Open airway and position the head. To open the airway, slightly extend the neck and maintain this position by placing a folded towel (about 1 inch thick) under the shoulder. Suction the baby’s mouth and then the nose using a mucous extractor or Dee Lee trap. If the amniotic fluid is meconium stained, current evidence does not recommend peripartum suctioning. Suctioning mouth should only be restricted to nonvigorous neonates irrespective of the color of the amniotic fluid. If amniotic fluid is meconium stained and the baby is not vigorous, suction the baby’s mouth and nose. If one has the expertise, the trachea may also be cleared by suctioning under direct laryngoscopy. Provide tactile stimulation. If the infant is not breathing even after suction, provide tactile stimulation by flicking the sole or gently rubbing the back (Fig. 2.2.1). Do not slap the back or squeeze the rib cage. Reassess the neonate. A prompt increase in heart rate remains the most sensitive indicator of resuscitation efficacy. Auscultate over the precordium for 10 seconds and multiply by 10 to get the infant’s heart rate. If the heart rate is below 100/min or the neonate is gasping or apneic, it is an indication to initiate positive pressure ventilation.

rugs

Adrenaline It is indicated whenever the heart rate remains less than 60/ min in spite of chest compression. The dose is 0.1–0.3 mL/ kg of 1:10,000 solution given intravenously or intratracheal. The dose may be repeated after 3–5 min as indicated.

Naloxone

Figure 2.2.1 Methods to provide tactile stimulation  

30

It is a narcotic antagonist. It is indicated to reverse respiratory depression in an infant whose mother has received narcotics within 4 hours of delivery. The dose is 0.1 mg/kg given intravenously, intratracheal or if perfusion is adequate, intramuscular or subcutaneously.

vip.persianss.ir

K

It is indicated in neonates in shock—poor pulses, pale and cold extremities. Dose is 10 mL/kg of normal saline or Ringer’s lactate given by intravenous (IV) push over 5–10 min. Nonresponse to volume expansion with 20 mL/kg of crystalloid would be indication for ionotropes such as dopamine or dobutamine. At present there is not sufficient evidence to recommend use of sodium bicarbonate in the delivery room.

C

ey Messages

are of he Ne bor

Volume Expanders

It may be appropriate to consider discontinuing resuscitation if no heart rate is detected for 10 min after birth.

B

w







1. McDonald SJ, Middleton P. Effect of timing of umbilical cord clamping of term infants on maternal and neonatal outcomes. Cochrane Database Syst Rev. 2008;(2):CD004074. 2. Perlman JM, Wyllie J, Kattwinkel J, et al. Part 11: Neonatal resuscitation: 2010 International Consensus on Cardiopulmonary Resuscitation and Emergency Cardiovascular Care Science With Treatment Recommendations. Circulation. 2010;122:S516-S538. 3. Rabi Y, Rabi D, Yee W. Room air resuscitation or the depressed newborn: a systematic review and meta-analysis. Resuscitation. 2007;72:353-63. 4. Taeusch HW, Ballard RA, Gleason GA. Avery’s Diseases of the Newborn, 8th edition. Philadelphia: Saunders; 2005.



R

D

When to iscontinue esuscitation

ibliography



Neonates who needed only initial steps of resuscitation can be provided observational care by monitoring them when roomed-in with their mothers. Neonates who needed more intensive resuscitative assistance such as assisted ventilation, chest compression or drugs need to be shifted to an neonatal intensive care unit (NICU) or special care newborn care unit, and monitored more intensely with clinical and biochemical monitoring. Many of these infants would need IV fluids, ionotropes, supplemental oxygen and even mechanical ventilation.



C

Post-resuscitation are

n









t



• About 5–10% neonates would require resuscitation at birth and almost half occur amongst women with no risk factors. • Resuscitation sequence should be swift, and in apneic infants assisted ventilation should start within 1 min. • Sequence of interventions is initial steps, assisted ventilation, chest compression and medication. • Initiate PPV with room air. Use oxygen supplementation cautiously in preterm infants. • All infants who need advanced resuscitative intervention should be shifted to an NICU for postasphyxial care.

31

vip.persianss.ir

C



are of the Normal Newborn B Vishnu Bhat suture lines. It is usually noticed after 2 days of birth and disappears after 6–8 weeks. It usually does not require treatment.

Normal eatures in Newborn

Urine and tool Passage

B

R

espiration, Heart ate and lood Pressure

kin hanges C

S

A newborn may have periodic breathing and the normal rate may vary from 40/min to 60/min. The heart rate is usually 120–160 beats/min. The systolic blood pressure will vary from 40 mm Hg to 60 mm Hg and diastolic 25–40 mm Hg.

mportant xamination indings in Newborn

Anterior and posterior fontanels are open at birth. Large posterior fontanel or both anterior and posterior communicating is usually abnormal. Similarly, if anterior fontanel is very small and there is ridging of suture lines, it may indicate craniosynostosis. Natal teeth may rarely be present. They may be loose and sometimes may result in injuries to mother’s nipple in which case, they may need to be extracted. Phimosis is normal during this period but the meatus is visible when prepuce is retracted in more than 85% of babies. Mucosal tags may be seen at the introitus in female babies. Liver palpable 2–3 cm below the costal margin and palpable spleen tip are normal. Usually both testes are in the bottom of the scrotum in term male babies. If the testes are not palpable or abnormally placed, it should be recorded and investigated.

32

Jaundice, which usually appears after 2–3 days and disappears by 7–10 days of life and does not stain the palms and soles is usually physiologic. Enlargement of the breast during first 3–7 days of life is known as mastitis neonatorum and does not require any treatment. This enlargement is related to maternal transfer of hormones. A female baby may have vaginal discharge and bleeding during that period, which is also hormone-related and disappears within a few days. There may be molding of the head and caput succedaneum over the presenting part, which disappears within 2–3 days. Caput should be differentiated from cephalhematoma. The latter is due to collection of blood under periosteum and hence does not cross the

mportant Neonatal eflexes R

E

Physiologic vents

I



Mongolian spots are bluish patches seen over the buttocks and back due to deposition of melanin pigment and disappear in 6 months to 2 years’ time. Milia are whitish pin head size papular lesions seen over the face due to obstruction to sebaceous glands. They disappear in 2–3 weeks. Erythema toxicum are erythematous papular, macular or rarely pustular lesions present over the body surface. They usually appear after 2 days and disappear during the first week. Epstein’s pearls are whitish papular lesions usually seen over the palate and sometimes over the prepuce and disappear after a few days. Small hemangiomas may be seen over the body surface, which disappear within 2 years of life.

F

The average birth weight of babies in India is 2,900 g. The length may vary from 47 cm to 52 cm and head circumference 32–35 cm. Generally, the head circumference is 2–3 cm more than chest circumference at birth and they equal by 1 year after which chest circumference exceeds that of the head.

A newborn usually passes urine within 48 hours and meconium during the first 24 hours of life. If there is no distension of abdomen, delayed passage of urine indicates inadequate feeding. Distension of bladder in a male baby suggests posterior urethral valve. When there is delay in passage of meconium, a soft rubber catheter should be passed into the rectum to confirm patency. The fingertip or thermometer should not be used for checking patency since these may result in injury. Prematurity, meconium plug, congenital megacolon, meconium ileus and hypothyroidism may result in delayed passage of meconium. A baby may pass frequent yellow-colored watery to semisolid stools during first 3–7 days of life known as transitional stools. Some babies may pass stools once in 2–3 days. Hypothyroidism should be excluded in babies with constipation. E

nthropometry

I

A

F

S

Normal newborn is an infant born at term with birth weight greater than 2,500 g without any asphyxia, major congenital malformations or birth injuries.

R

t

t

Tex book of Pedia rics

2.3

Rooting Reflex Rooting Reflex helps the baby to locate the mother’s nipple without her directing the baby’s mouth. The infant turns toward the point where the cheek is touched. When the corner of the mouth is touched, the lower lip is lowered and the tongue is brought forward toward the contact. If the finger is moved away, the head turns to follow it.

vip.persianss.ir

C

Sucking and Swallowing Reflexes

 

are of

Table 2.3.1 Danger signs in newborn

eeding

Temperature Maintenance and Monitoring

Vitamin

All babies should receive vitamin K prophylaxis of 0.5 mg (for < 34 weeks) to 1 mg (for > 34 weeks) intramuscularly after birth to prevent hemorrhagic disease of the newborn.

anger igns S

D

Normal body temperature of newborn ranges from 36.5°C to 37.5°C. Newborns are uniquely susceptible to hypothermia because they have a large body surface area, which causes heat loss and have reduced subcutaneous insulation. They are dependent on caregivers to keep them warm and dry. The temperature of the delivery room should be at least 26 ± 2°C and free from draft of air. The infant should be received in prewarmed sterile linen and should be dried thoroughly from head to foot. The wet linen should be removed and replaced with a dry cloth. The baby should be made to wear cap and socks. Kangaroo care position is the most ideal. Skin to skin contact with the mother not only prevents hypothermia, but also promotes breastfeeding and bonding. A bath is delayed until the temperature is stabilized. It is better if baby bath is not given in hospital for fear of cross infection. Mother can be taught the simple technique of monitoring the baby’s temperature by touching the periphery and observing for color change. Cold extremities with blue or pale color indicate inadequate warmth.

Mother should be informed about danger signs and the need to get medical advice as and when they are observed. Some of the danger signs are given in Table 2.3.1. D

It is elicited by touching the baby’s palm from the ulnar side with finger or any other suitable object. The fingers close and grasp the object. When the dorsum of the hand is touched, the fingers open. The grasp becomes stronger if the head is turned to the opposite side and the stimulating finger is moved toward the fingers. Similar reflex can be elicited in the lower limb by stimulating the sole. Persistence of grasp after 3 months of age may indicate cerebral palsy.

K

Management

Breastfeeding should be initiated soon after birth and exclusive breastfeeding is advised till 6 months of age in normal term babies. A baby should receive 7–8 feeds a day during the first few weeks. The pregnant mother should be explained the benefits of breastfeeding and the family members are encouraged to support her. It is important not to separate the newborn from the mother without a justifiable reason. The initial alert period is utilized to start breastfeeds as babies tend to sleep a lot after that. Health workers caring for newborns should learn the signs of good attachment; which include: mouth wide open, more areola seen above than below, chin touching the breast and lower lip everted. Excessive weight loss of more than 8–10% in the first 3–4 days indicates inadequate breastfeeding or illness in the baby.

Grasp Reflex

n

C

E

ord and ye are

The umbilical stump should be cleaned with spirit and kept dry. Local application of antiseptics is not required. Usually the stump will fall in 7–10 days’ time. The eyes should be cleaned with sterile wet cotton. There is no need to instill antibiotic drops into eye except in areas with high incidence of vertically acquired conjunctivitis. F

It is a vestibular reflex, which disappears by 3–5 months of age. It can be elicited by raising the shoulder for 45° from ground and then dropping by 30°. There will be abduction and extension of arms with opening of fingers. This is followed by flexion and adduction of arms. The reflex may be accompanied by crying, extension of trunk and neck with movements of legs. It is exaggerated when there is cerebral irritation caused by hypoxia, infection, hyperbilirubinemia, etc. It is decreased with sedation, central nervous system (CNS) depression or prematurity. Asymmetrical response is seen with Erb’s palsy, clavicular or humerus fracture, or shoulder dislocation.

C

Moro Reflex

Poor sucking/cry Fever/hypothermia Persistent vomiting Abdominal distension Pallor Jaundice involving extremities Bleeding Convulsions

th

•  •  •  •  •  •  •  • 

e Newbor

These are elicited by introducing clean finger or mother’s teat in the mouth. They disappear when voluntary control of feeding is achieved. They are decreased or absent when there is neurological depression, hypotonia or immaturity. A baby would have usually crossed 34 weeks of gestation when he/she can take full feed from breast.

ischarge Planning

It is ideal to discharge a normal newborn after 48–72 hours of life. The baby should be free of illness, significant jaundice, and the mother should be confident about breastfeeding. The baby should have received initial immunization with bacille Calmette Guerin (BCG), oral polio and Hepatitis B vaccines; and have passed meconium and urine. If the baby is discharged early, he or she should be reviewed at home

33

vip.persianss.ir

B









1. Behrman RE, Kliegman RM, Jenson HB (Eds). Nelson Textbook of Pediatrics, 18th editon. Philadelphia: Elsevier Saunders; 2007. 2. Bhat BV. Neonatal care. In: Parthasarathy A, Menon PS, Nair MK, Bhave SY (Eds). Partha’s Fundamentals of Pediatrics, 1st edition. New Delhi: Jaypee Brothers; 2007. pp. 29-62. 3. Cloherty JP, Echenwald EC, Stark AR (Eds). Manual of Neonatal care, 6th edition. Philadelphia: Lippincott Williams and Wilkins; 2008. 4. Illingworth RS. The Normal Child. Some Problems of the Early Years and their Treatment, 10th edition. Edinburgh: Churchill Livingstone; 1997. 5. Taeusch HW, Ballard RA, Gleason CA. Avery’s Diseases of the Newborn. 8th edition. Philadelphia: WB Saunders; 2005.



Temperature control and prevention of hypothermia is vital. Breastfeeding is initiated as soon as possible. All babies should receive vitamin K prophylaxis. Initial immunization should be given before discharge. Growth and development should be monitored during follow-up.



ey Messages

ibliography



K







• • • • •



t

t

Tex book of Pedia rics

or hospital after 48 hours. Babies should be followed up and anthropometry recorded in a growth chart. The growth and development should be evaluated monthly during the first few months and 3-monthly thereafter.

34

vip.persianss.ir

A

dentification and pproach to a Sick Newborn

I



Care of the Newbor

2.4

Swarna Rekha Bhat

 

Table 2.4.2 Features of significant illness on history Danger signs •  Lethargy •  Breathing difficulty •  Temperature instability •  Failure to pass urine/meconium in first 24/48 hours •  Vomiting •  Diarrhea •  Cyanosis •  Jaundice •  Abdominal distension •  Convulsions •  Bleeding •  Excessive weight loss

ho is a Sick Newborn?

valuating a Sick Newborn istory E

H

E



A sick newborn may be described as one who is: • Not feeding well • Lethargic • Has any systemic illness and • Has poor weight gain. These are infants who require additional support such as oxygen, IV fluids, etc.

Following history should be elicited to determine nature of illness, and presence or absence of risk factors (Table 2.4.1): • Age of neonate in hours/days • Antenatal history to identify any risk factors • Intrapartum history to identify risk factors • Mode of delivery • Apgar score or crying or breathing immediately after delivery • Feeding history: Any feed given/is feeding adequate/is baby on breastfeeds • One should also elicit history suggestive of significant illness (Table 2.4.2).

xamination

Examination of a sick neonate can provide useful clues to the etiology of sickness and also provides a basis for the management plan. Important signs and their utility are summarized below.

Temperature Instability Hypothermia (< 36°C) could be due to environmental factors or as a result of sepsis. Hyperthermia is less common; could be environmental or a manifestation of sepsis.

Respiratory Distress Breathing problems in a neonate can be tachypnea (respiratory rate > 60/min), chest indrawing, stridor or apnea. The severity of respiratory distress (RD) can be monitored using the respiratory distress score (Table 2.4.3).

 

Table 2.4.1 Risk factors: maternal and fetal

n

W

Early identification of a sick newborn is the key to successful management and improving neonatal outcome.

Antenatal risk factors •  Maternal diabetes mellitus •  Pregnancy-induced hypertension (PIH); eclampsia •  Urinary tract infections (UTI) •  Any illness •  Rh negative mother •  Oligo- or polyhydramnios Fetal •  Intrauterine growth retardation (IUGR) •  Abnormal ultrasonogram (USG) •  Abnormal Doppler study •  Preterm delivery Intrapartum •  Premature rupture of membranes (PROM) •  Abnormal nonstress test (NST) •  Meconium-stained amniotic fluid (MSAF) •  Difficult delivery •  Operative delivery

Cyanosis The two most serious causes of cyanosis are congenital cyanotic heart disease and respiratory illness such as respiratory distress syndrome (RDS), meconium aspiration syndrome (MAS) or pneumonia. However, simpler problems such as polycythemia and hypoglycemia can also present as cyanosis.

Shock It can be due to fluid/blood loss, asphyxia, duct-dependent cardiac lesions, sepsis or inborn errors of metabolism (IEM).

Pallor Pallor may indicate anemia (due to blood loss or hemolysis), but may also indicate hypothermia, hypoxia, hypotension and sepsis.

35

vip.persianss.ir

cs

 

Features

0

1

2

Respiratory rate/min

< 60

60–80

> 80

Cyanosis

Nil

Nil on 40% O2

Requires > 40% O2

Retractions

Nil

Mild

Moderate to severe

Grunting

Nil

Audible with stethoscope

Audible without stethoscope

Air entry

Normal

Decreased

Barely audible

Convulsions The critical point is recognition of convulsions. Any abnormal movement needs to be reported, jitteriness and sleep myoclonus needs to recognized, subtle seizures are often missed. Seizure can be a pointer for several illnesses.

neonate, it could be due to thrombocytopenia. Intracranial bleed is a serious problem and is more common in preterm neonates.

Jaundice

Lethargic, Poor Feeding, Unresponsiveness

Neonatal jaundice is often physiologic, but high bilirubin levels can be associated with neuronal damage. In neonates with persistent jaundice after day 14, always check for color of stool. Pale colored stool could indicate cholestatic jaundice such as biliary atresia.

A sleeping neonate may appear lethargic. A simple method of determining whether a neonate is lethargic or sleeping is to observe if the neonate is responsive to stimulation. If a neonate does not change state during examination or handling, it indicates that the sensorium is not normal. The etiology of lethargy may range from simpler causes like hypoglycemia and polycythemia to more severe ones including sepsis, meningitis, intracranial bleed and hypoxic brain injury. Neonates with IEM usually present with lethargy and poor feeding.

The most likely reason is poor milk intake; however other more serious problems, which can present as poor weight gain include any systemic illness and IEM. A

V d

Vomiting

n U

A neonate presenting with vomiting may be having something as physiologic as regurgitation or it could be due to an intestinal obstruction. Bilious vomiting and persistent vomiting associated with abdominal distension or lethargy indicates need for further evaluation and admission.

Abdominal Distension

Abdominal distension usually indicates a serious underlying problem such as sepsis, paralytic ileus, intestinal obstruction or Hirschsprung’s disease. It is uncommon for a well neonate to have abdominal distension. Occasionally, medications used for colic (e.g. antispasmodics) can lead to abdominal distension and constipation.

Bleeding Bleeding can be from any site; it could be skin bleeds, mucosal bleeds (GI bleed) or visceral bleed. The most common cause of gastrointestinal bleed in a neonate is due to hemorrhagic 36 disease of newborn. Skin bleeds usually indicate sepsis, disseminated intravascular coagulopathy (DIC), but in a well



Inconsolable, excessive and incessant crying may indicate severe illness. Stage I hypoxic ischemic encephalopathy (HIE) is one example of a CNS problem presenting as excessive crying. Pain, as in arthritis, sepsis, myocarditis, intracranial bleed and hypoxia can all manifest as excessive cry and irritability in the initial stages of the illness.

ssessment of llness Severity

Severity of illness can be assessed at admission by assessing the physiologic alterations in the baby using the score for neonatal acute physiology and perinatal extension (SNAPPE II). Details of this score have been provided in Table 2.4.4. Scores greater than 15 are usually associated with higher mortality. Neonates who will require high dependency care or shift to a tertiary center will include: • VLBW and ELBW neonates • Any neonate requiring ventilator support • A sick neonate requiring constant monitoring (multiple seizures) • A sick neonate requiring cardiovascular support (shock) • Any sick neonate who needs constant monitoring (refractory hypoglycemia, oliguria) • Suspected or proven IEM. T

ti e

Excessive Crying, Irritability and Restlessness

G R

Inadequate Weight Gain

I

extbook of Pediatri T

Table 2.4.3 Respiratory distress (RD) score

riaging Sick Neonates

All neonates should be assessed for emergency signs: • Assess for hypothermia. If present, arrange for rewarming of the baby. • Check for severe respiratory problem and if present, arrange to oxygenate the baby. • Determine if the child is in shock or has encephalopathy or convulsions and if so initiate urgent steps to manage. The triaging process is outlined in Flow chart 2.4.1. Only after initiating emergency measures, proceed to investigate the neonate. Proceed to treat the neonate for underlying disorder based on the clinical examination and investigations.

vip.persianss.ir

T

E

 

Give mergency reatment

Value

Score

Maintain emperature

Mean blood pressure

29 mm Hg 20–29 mm Hg < 20 mm Hg

0 9 19

Core temperature

> 35.6°C 35–35.6°C < 35°C

0 8 15

Place the neonate under a warmer and bring the temperature to 36.5°C–37.5°C. Keep the baby dry, and the head, hands and feet should be covered. Maintain the temperature within this range.

Birth weight

999 g 750–999 g < 750 g

0 10 17

2.49 1–2.49 0.3–0.99 < 0.3

0 5 16 28

Lowest serum pH

7.19 7.1–7.19 < 7.1

0 7 16

Urine output

> 0.9 mL/kg/hr 0.10.9 mL/kg/hr < 0.1 mL/kg/hr

0 5 18

Multiple seizures

No Yes

0 19

Apgar at 5 min

>7 1,500 g

Birth weight 1,000 to 1,500 g

1

60

80

2

75

95

3

90

110

4

105

125

5

120

140

6

135

155

7 onwards

150

170

R

The delivery room management consists of an expert resu scitation—maintaining good thermoregulation, minimal handling, and use of nasal continuous positive airway pressure (CPAP) and intubation when required for preterm births. Low birth weight neonates who need care in a special care unit include those with birth weight less than 1,800 g, gestation less than 34 weeks, any neonate who is unable to feed from the breast and any sick neonate.

Low birth weight babies are more prone to develop hypothermia due to deficient heat regulatory mechanisms. Soon after delivery, the unclothed baby with the head and feet covered should be placed in between the breasts of the mother in skin-to-skin contact position (Kangaroo mother care). It not only maintains the temperature of the baby but also helps in the prompt initiation of breastfeeding. If the baby requires resuscitation or hospitalization, she may be placed under a servo controlled radiant warmer with the temperature probe attached to the trunk of the baby. In absence of a servo device, the baby should be covered with at least 3–4 layers of clothes, socks, mittens and cap. In a special care newborn unit, the infants may be kept warm using overhead radiant warmer or incubator. Regular monitoring of axillary temperature at least once every 6–8 hours should be carried out in all hospitalized babies. Devices that provide direct heating of the baby like hot water bags, hot air blowers and direct heaters should be avoided. F

luids

If a LBW needs fluids, then in the first 48 hours after birth, neonates less than 1,250 g should be provided 5% dextrose

B

F

E

V d

ti e

n U

G R

eight Neonate

The goal of nutritional management of the LBW infant should be to achieve full enteral nutrition as soon as possible. Breast milk is the best milk for the neonate and the mother should be supported and counseled for the maintenance of regular lactation and the need for expression and its technique. The guidelines for providing enteral feeds to the LBW neonate are summarized in Table 2.5.3. Neonates can undergo gradual transition from gastric feeding to spoon feeding and then onto breastfeeding. In infants less than 34 weeks, transition from tube to breast can be facilitated by allowing the infant to suck on empty breast of mother (nonnutritive sucking) before each gavage feeding session. Daily assessment of the sucking efforts of the neonate will provide indication of when and how fast the transit from one mode to another mode of feeding should be undertaken.

T

hermoregulation

nteral eeding of the Low irth

W

and those more than 1,250 g should receive 10% dextrose. The fluid requirements volumes are given in Table 2.5.2. Sodium and potassium should be added after 48 hours or earlier if there is more than 6% weight loss from the birth weight in the first 48 hours.

­

cs d

extbook of Pe iatri T

• • • • • • •

Specific problems of SGA infants include the following: Perinatal asphyxia Meconium aspiration syndrome Infections Hypoglycemia Polycythemia Hypothermia Dysmorphology.

Monitoring for Feed Intolerance The signs of feed intolerance are: an increase in the abdominal girth by more than 2 cm from the baseline, vomiting of feeds and a prefeed residue of greater than 25–50%. Presence of such signs in the baby may prompt a cessation of feeding and initiating investigation into the cause of feed intolerance.

 

Table 2.5.3 Guidelines for the modes of feeding for low birth weight (LBW) neonates

40

Birth weight (g) Gestation (week) Condition

< 1,200 < 30

1,200–1,800 30–34

> 1,800 > 34

Initial

Intravenous fluids; Try gavage feeds if not sick

Gavage

Breastfeeding; if unsatisfactory, give spoon or paladai feeds

After 1–3 days

Gavage

Spoon or paladai feeds

Breastfeeding

Later (1–3 weeks)

Try spoon or paladai feeding

Breastfeeding

Breastfeeding

After some more time (4–6 weeks)

Breastfeeding

Breastfeeding

Breastfeeding

vip.persianss.ir

Vitamin and Mineral Supplementation

F

Low birth weight neonate can be considered for discharge if the infant has had a smooth transition to breastfeeding or breast and spoon/paladai feeds, is gaining weight consistently for at least 3 days, maintains temperature when being cared with mother, is not receiving any oxygen





K





B







Discharge Policy

1. Agostoni C, Buonocore G, Carnielli VP, et al. Enteral nutrient supply for preterm infants: commentary from the European Society for Pediatric Gastroenterology, Hepatology and Nutrition Committee on Nutrition. J Pediatr Gastroenterol Nutr. 2010;50(1):85-91. 2. Bombell S, McGuire W. Early trophic feeding for the very low birth weight infants. Cochrane Database Syst Rev. 2009;(3):CD000504. 3. Lucas A, Fewtrell M. Feeding low birth weight infants. In: Rennie JM (Ed). Roberton’s Textbook of Neonatology, 4th edition. Edinburgh: Churchill Livingstone, 2005. pp. 314-24. 4. Papageorgiou A, Pelausa E, Kovacs L. The extremely low birth weight infant. In: Mhairi G MacDonald, Martha D Mullett, Mary M K Seshia (Eds). Avery’s Neonatology: Pathophysiology and Management of the Newborn, 6th edition. Lippincott: Williams and Wilkins; 2005. pp. 461-89.



I

Avoid routine oxygen administration, prophylactic use of IV immunoglobulins, antibiotics, indomethacin or high doses of vitamin E. Unnecessary blood transfusions (maintain hematocrit above 35% in sick newborns), formula feeds and rough handling, excessive light and sound should be avoided.

ibliography





A

hat to void

V d

ey Messages

• Care for LBW babies at health facilities where optimum care can be provided. • Provide breast milk to all LBW babies. Those who have poor sucking feeding by gavage or use spoon or paladai to assist feeding of expressed breast milk is recommended. • Use adequate vitamin (vitamin D) and minerals (calcium, phosphorus and iron) supplements till at least 12 months of age. • Use Kangaroo mother care at hospital and also train families to provide the same after discharge from hospital at home. • Follow-up LBW for growth, development (especially vision and hearing) and illness till at least 12–18 months of age.

ti e

n U

G R

Mortality of LBW babies is directly related to the birth weight and gestational maturity. Lesser the weight and gestation, the poorer the prognosis. In general, over 90% of all LBW babies have no major neurodevelopmental handicaps.

dequacy of Nutrition

All vaccines should be administered as per schedule according to the chronological age irrespective of birth weight or period of gestation. Hepatitis B, BCG and birth dose of oral polio vaccine (OPV) and can be safely and effectively given to LBW or preterm babies after stabilization. W

Prognosis

n

B

F A

According to the Cochrane review, multicomponent human milk fortification leads to short-term increase in weight gain, linear growth and head circumference growth. There is still insufficient evidence in contemporary literature to recommend routine fortification of human milk. The cost, greater risk of contamination and theoretical risk of hypercalcemia are some factors, which are to be borne in mind before prescribing human milk fortifier to LBW infants.

mmunization

ollow-up Protocol

After discharge from the hospital, babies should be regularly followed up and screened for the following parameters: • Feeding and nutrition • Anemia and osteopenia • Growth and development: Neurobehavioral problems • Immunization • Retinopathy of prematurity, vision, strabismus and hearing • Problems resulting from previous morbidities, e.g. bronchopulmonary dysplasia.

ortification of Preterm reast Milk

The key measure of optimal feeding is the weight pattern of the baby. A preterm LBW baby loses up to 1–2% weight every day amounting to 10% cumulative weight loss during the first week of life. Birth weight is regained between 10th day and 14th day. Babies start gaining weight by the second week of life at the rate of about 15–20 g/day and this is considered adequate. Excessive weight loss or inadequate weight gain indicates inadequate feeding, cold stress, excessive insensible water loss or systemic illness (like anemia, sepsis, late metabolic acidosis, etc.).

Care of the Newbor

Supplementation should be started as soon as the infant is receiving at least 120–150 mL/kg of enteral feeds. All preterm should receive daily supplement of 400 IU of vitamin D and 120–140 mg/kg/day (110–130 mg/100 kcal) of highly bioavailable calcium with 60–90 mg/kg/day (55– 80 mg/100 kcal) of phosphate is recommended. At 4 weeks of age, iron supplements should be started in a dose of 2–3 mg/kg/day. At present there is no evidence for routine zinc supplement or protein fortification of breast milk.

or antibiotics, and the mother is confident in handling the baby. Assessment of the home environment prior to discharge may be particularly useful in cold climates.

41

vip.persianss.ir

C





Parent ounseling Ranjan Kumar Pejaver

Parent counseling is the process of providing assistance and guidance by giving information to the parents at various points during the perinatal period. It is the art of presenting facts enabling the parents to make the decisions.

regarding possible sequelae, further continuation of care or withdrawal of care. C

Predischarge ounseling

Preconception

B

There is a history of recurrent pregnancy loss, stillbirths or neonatal deaths. Parents would want to know why that has happened and is there a way of preventing it in the next pregnancy.

Neonatal Period Preterm infant has had a stormy stay in the intensive care unit resulting in severe damage to various organ systems. 42 Intact survival is unlikely. Parents may need information



­



ounseling: pecific Technical spects A

Baby needed extensive resuscitation, has survived and what are the repercussions. Baby is extremely LBW or premature, or in case of antenatally undiagnosed abnormalities, parents may need information regarding the management and prognosis.

S

D

mmediately fter elivery A

I

Baby is receiving extensive resuscitation and does not seem to be responding. They may need counseling regarding continuation/stopping of resuscitation measures.



S

A

D

A

It has revealed some congenital abnormality or findings like growth restriction, oligo- or polyhydramnios and need for information regarding the seriousness of the same and fate of the current pregnancy and the fetus.

t elivery

V d

C

n U

bnormal ntenatal Ultrasound can

The counseling should be conducted in a quiet and comfortable room. It should be conducted by a senior member of the team. It is preferable that the session is conducted in a language the parents understand. If necessary, a reliable interpreter may be used. It should be done in strict privacy, preferably with both parents being present. Some key decision makers in the family or supportive elders may be allowed to be present with permission of the parents. It is good to involve nurses and other specialists who are involved or likely to be involved in the care of the infant. Counseling should be nondirective and nonjudgmental. Be careful not to hurt the local, traditional, family, cultural and religious sentiments of the parents. Provide information in simple, nontechnical language. The depth of the infor mation should be commensurate with the educational level and the understanding ability of the listeners. Be patient, be ready to repeat the advice, give time to the parents to understand, think and then convey the decisions. Calmly answer the queries and be sympathetic. As they are overwhelmed by the situation, they are confused, less receptive and may not be able to quickly process the information given. If there is any written material relevant to the discussion, it is helpful and may be given to the parents. It is a good habit to summarize the discussion and decisions arrived at; write it down and take the signatures of the parents after they have read it. Signatures of the counselor and witness are also important.

D

A A

Parents are of extreme ages and may be suffering from conditions like severe diabetes, hypertension and systemic diseases. Parents would like to know how the next pregnancy and baby will be affected by these diseases.

C

C

General Principles of Parent ounseling

ti e

ge and Health of Parents

G R

If a baby has demised or stillbirth has occurred.

Health of Previous hild Previous child of the parents or any members of the immediate family of either of the spouses suffering from inherited disorder, congenital abnormalities, developmental delay and dysmorphic features. Parents would like to know information like what are the chances of the next pregnancy resulting in the same.

ereavement ounseling C

Which Parents Need ounseling?

Parents of NICU graduates would need information regarding danger signs to watch out, follow up plans and other instructions like feeding, medications, immunizations, etc. or in case of postoperative state. Parents need information regarding home care and any follow-up procedures—timing, cost and prognosis.

C

t

t

Tex book of Pedia rics

2.6

iagnosis

Wherever applicable, diagnosis should be known as far as possible. Where it is not known, the same should be

vip.persianss.ir

S

C th



B









1. American Academy of Pediatrics Committee on Fetus and Newborn. The initiation or withdrawal of treatment for highrisk newborns. Pediatrics. 1995;96:362-3. 2. Baker K, Kuhlman T, Magliaro B. Homeward bound: discharge teaching for parents of newborns with special needs. Nursing Clin North Am. 1989;24(3):655-64. 3. Freer Y, Lyon A, Stenson B, et al. BabyLink—improving communication among clinicians and with parents with babies in intensive care. Br J Healthcare Comput Inform Manag. 2005;22(2):34-6. 4. Harmon RJ, Glicken AD, Siegel RE. Neonatal loss in the intensive care nursery: Effects of maternal grieving and a program for intervention. J Am Acad Child Psychiatry. 1984;23:68-71. 5. Leslie L, Harris LL, Douma C. End-of-life Care in the NICU: a family-centered approach. NeoReviews. 2010;11:e194-e199. 6. Munson D. Withdrawal of mechanical ventilation in pediatric and neonatal intensive care units. Pediatr Clin North Am. 2007;54:773-85.



ibliography



D

S

This is devastating to the family. There is a feeling of loss, guilt, shame, inadequacy and anger in the parents. Compassionate counseling is required stressing on the point that neither of the parent is responsible for it. It may be essential that a diagnosis be arrived at in view of future pregnancies even it may not help in the current one. Especially if genetic disorders or metabolic defects are suspected, investigations such as chromosomal

• Parents need counseling at several stages of perinatal period. It is the responsibility of care providers to support parents to make informed choices. • Counseling should be conducted by trained personnel respecting the privacy of families. • Counseling should be nonthreatening, nontechnical but supportive.



C

ti e

Pregnancy Loss, tillbirth, eath of a Neonate

ey Messages

V d

Withdrawal of are

n U

G R K

Resuscitation is not successful all the time. Discontinuation is justified if there are no signs of life (no heart beat and no spontaneous respirations) after 10 minutes of full resuscitation. Parents should be informed if possible about the progress and status of the baby at various steps. In major congenital abnormalities incompatible with life, discussion with parents is essential before taking decision to discontinue resuscitation. A concurrence of another colleague is ideal.

The same applies to question of parental request for withdrawal of care in babies receiving intensive care, having multiple organ systems involved, with remote possibility for ultimate survival or likelihood of severe neurological sequelae is almost certain. Clinical indicators, laboratory investigations and imaging studies showing bad prognostic signs should be documented and shown to parents. Decisions like “do not resuscitate”, “withdrawal of care” and “discharge against medical advice by parents” warrant due deliberation, proper documentation and authorized personnel’s and parents’ concurrence in black and white. Legal sanction for any of the above does not exist at present time!

w



esuscitation



R

It should be known and quoted where necessary, e.g. survival percentage of very LBW infants and premature infants. It will guide the parents to decide roughly on the outcome and whether to seek transfer to higher centers if available.



D

S

tatistical ata upports

e Ne bor

Family history, pedigree construction, knowledge of the prevalence of the disorder, modes of inheritance and recurrence chances should be ascertained.

n

A

C

ounseling on Genetic spects

analysis, autopsy, infantogram, clinical photographs, tissue biopsy, blood and other body fluid analysis should be done. Informed consent of parents is needed for this. In cases of neonatal deaths, a meeting with the parent should be arranged after a few weeks when they would be more receptive having gone through the stages of bereavement and results of all investigations are available to have a more fruitful counseling. Future reproductive options, perinatal preventive strategies like folic acid consumption, investigations, antenatal management as “high-risk” pregnancy, planning of delivery and neonatal care could be discussed. Visits by a social worker to the abode of the unfortunate couple at least during the first few months after the tragedy to counsel will be very beneficial. Parental counseling is an important aspect of perinatal care. It should be given its due priority. It is ideal that every unit has a person who has had some formal training in counseling.

are of

conveyed to the parents. If a visit to higher center would be helpful, then sufficient information about that should be supplied.

43

vip.persianss.ir





Neonatal Hyperbilirubinemia

efinition

 

Table 2.7.1 Causes of indirect (unconjugated) hyperbilirubinemia

tiopathogenesis

Hyperbilirubinemia is the result of an imbalance between bilirubin production and its elimination. In majority, early neonatal jaundice is due to rise in unconjugated (indirect) bilirubin. The etiological basis for progressive hyperbilirubinemia is usually multifactorial. Table 2.7.1 summarizes the important causes.

R

B

Jaundice within 24 hours of life, peak bilirubin greater than 15 mg/dL and persistence beyond 2 weeks are not physiologic and needs to be investigated. Rate of rise of bilirubin greater than 0.5 mg/dL/hour or bilirubin level greater than 25 mg/dL increase the risk of bilirubin toxicity in neonates. Bilirubin is a potential neurotoxin. Areas of the brain most commonly affected are the basal ganglia and brainstem nuclei for oculomotor and auditory function. Bilirubin toxicity or kernicterus can cause permanent sequelae characterized by tetrad of choreoathetoid cerebral palsy, high frequency central hearing loss, vertical gaze palsy and dental enamel hypoplasia. A

linical ssessment

All newborns should be examined for jaundice at least once a day during the first 72 hours. Jaundice is assessed 44 by inspecting the baby’s skin, sclera or mucous membranes





E

D

ssessment in Neonates ischarged arly

All newborns should be assessed for presence of risk factors at the time of discharge (Table 2.7.2). The more risk factors present, the greater the risk of severe hyperbilirubinemia, and the risk is extremely low if risk factors are absent. If neonates are discharged within 24 hours, those with risk factors should be seen within 24–48 hours, and those without risk factors within 72 hours of discharge. If facilities are available then serum bilirubin can be estimated by use of transcutaneous bilirubinometer on each of these visits.

Table 2.7.2 Risk factors for significant hyperbilirubinemia  

n U

Nonphysiologic Jaundice and isk of ilirubin Toxicity

V d

ti e

The functional immaturity in bilirubin metabolism viz. increased enterohepatic circulation, increased fetal erythrocyte breakdown, decreased hepatic excretion and immature hepatic conjugation predisposes to early jaundice which is transient, requiring no treatment. It usually appears after 24 hours, peaks between 3 days and 5 days in term and 5–7 days in preterm and disappears by 2 weeks of life. The peak bilirubin is under 15 mg/dL.

G R

preferably in natural light. The skin is blanched by digital pressure over bony parts to reveal underlying yellowing. Jaundiced newborns should also be examined for bruising, cephalhematoma, lethargy, vomiting, excessive weight loss, pallor, plethora and hepatosplenomegaly. Abnormality in tone, cry or sensorium should alert to possibility of bilirubin neurotoxicity. In infants presenting with jaundice extending beyond 2 weeks, one must enquire for pale or white stools, which may indicate obstructive jaundice such as biliary atresia. A

Physiological Jaundice

C

•    •    •    •  •   

Physiological jaundice Breastfeeding jaundice, breast milk jaundice Increased production –  Blood group incompatibility (Rh, ABO, minor blood group) –  RBC membrane defects (hereditary spherocytosis, elliptocytosis) –  RBC enzyme defects (G6PD deficiency, pyruvate kinase deficiency) Disorders of bilirubin uptake –  Gilbert’s syndrome Disorders of conjugation –  Crigler-Najjar Types I and II, hypothyroidism, pyloric stenosis Enhanced enterohepatic circulation –  Small or large bowel obstruction or ileus Idiopathic Others –  Prematurity, sepsis, polycythemia, infant of diabetic mother, extravascular blood (cephalhematoma, bruising) 

•  •  •       

Hyperbilirubinemia is a common neonatal problem. It is defined as an increased level of bilirubin in the circulation. A small proportion of these neonates develop jaundice that is significantly high to warrant treatment. Approximately, 60% of term and 80% of preterm babies develop jaundice during the first week of life. About 5% develop pathological jaundice. Studies suggest the possible impact of genetic polymorphisms on ethnic variation in the incidence and severity of jaundice.

•  •  •  •  •  •  • 

Primipara mother Visible jaundice at discharge Gestation < 38 weeks History of jaundice requiring treatment in previous sibling ABO/Rh incompatibility Geographic prevalence for G6PD deficiency Weight loss at discharge > 3% per day or > 7% cumulative weight loss. 

D

Rhishikesh Thakre

E

t

t

Tex book of Pedia rics

2.7

vip.persianss.ir

C



n

Treatment of Unconjugated Hyperbilirubinemia The decision making in jaundice management is based on gestation, weight, well-being and age of the infant. Tables 2.7.4 and 2.7.5 provide guidelines of how to decide if an infant needs treatment—phototherapy (PT) or exchange transfusion (ET).

 

Table 2.7.3 Investigations for significant hyperbilirubinemia •  •  •  •  •  •  • 

e Newbor

In all neonates with jaundice that is not considered physiologic, investigations need to be done to assess the severity of jaundice (for planning treatment) and etiology of the jaundice. Table 2.7.3 summarizes the important investigations that need to be done. Cord blood is collected for typing the baby blood group if mother’s blood group is Rh negative or O Rh positive or blood group is not known. If an Rh negative mother has an Rh positive baby, the cord blood is subjected to direct Coombs test, total serum bilirubin (TSB), reticulocyte count, peripheral smear and hemoglobin.

th

­

are of

I

End-tidal carbon monoxide measurement (ETCO) in exhaled air may serve as indirect marker of ongoing hemo lysis as equimolar concentrations of CO and bilirubin are formed following breakdown of RBC. Synthetic heme analogs, metalloporphyrins, are competitive inhibitors of heme oxygenase and their use has been proposed as an attractive alternative strategy for preventing or treating severe hyperbilirubinemia.

nvestigations

Total and direct bilirubin Mother and baby blood group Hemoglobin or packed cell volume (PCV) Peripheral blood smear (for RBC shape and evidence of hemolysis) Reticulocyte count Direct Coombs test (if mother is “O” or Rh negative) G6PD assay

G R

Phototherapy

Phototherapy is the mainstay of treatment. When bilirubin is exposed to blue light (in the range of 420–480 nm), it undergoes change in structure to a product called lumirubin, which can be excreted in the urine without undergoing conjugation in the liver. The efficacy of PT depends on spectrum of light (460–480 nm), irradiance (8–30 mW/cm2/nm) and surface area of infant’s skin exposed to light. The choice of device depends upon the severity of jaundice. For majority of infants, standard PT (six special blue lights of Philips TL 52, 20 W each) is effective. When the

V d

Sick infant with jaundice or prolonged jaundice (> 3 weeks) •  Complete blood count •  Urine examination and culture •  Evaluate for infection as indicated •  Urine for reducing substances •  Thyroid profile (T4, TSH) •  Evaluate for cholestasis (if direct bilirubin is elevated)

ti e

 

Table 2.7.4 Treatment guidelines for jaundiced neonates with less than 35 weeks of gestation Birth weight (g)

n U

Phototherapy (mg/dL)

Healthy infant

< 1,000

5–7

1,001–1,500

7–10

1,501–2,000

10–12

2,001–2,500

Exchange transfusion (mg/dL)

Sick infant

12–15

4–6

Variable

6–8

Variable

8–10

Variable

10–12

Variable

 

Table 2.7.5 Treatment guidelines for jaundiced neonates born at or greater than 35 weeks of gestation (beyond 24 hours) Total serum bilirubin levels (mg/dL)

Low risk

Medium risk

High risk

≥ 38 weeks and well

≥ 38 weeks + risk factors* or 35–37 6/7 weeks and well

35–37 6/7 weeks + risk factors*

Age (hours)

PT

Intensive PT

ET

PT

Intensive PT

ET

PT

Intensive PT

ET

24

9

12

19

7

10

17

5

8

15

48

12

15

22

10

13

19

8

11

17

72

15

18

24

12

15

21

10

13

18.5

96

17

20

25

14

17

22.5

11

14

19

> 96

18

21

25

15

18

22.5

12

15

19

i

*Risk factors: soimmune hemolytic disease, G6PD deficiency, asphyxia, significant lethargy, temperature instability, sepsis, acidosis or albumin less than 3.0 g/dL Abbreviations: PT, Phototherapy; ET, Exchange transfusion

45

vip.persianss.ir

t

 

Table 2.7.6 Causes of conjugated (direct) hyperbilirubinemia



Administering Phototherapy

•  Bacterial sepsis •  Neonatal hepatitis   –  Toxoplasmosis, cytomegalovirus, rubella, herpes, syphilis, parvovirus B19 •  Biliary obstruction   –  Biliary atresia, choledochal cyst, inspissated bile plug •  Metabolic disease   –  Hypothyroidism, galactosemia, alpha-1-antitrypsin deficiency •  Genetic disease   –  Dubin-Johnson syndrome, Rotor syndrome, cystic fibrosis, Alagille syndrome •  Others   –  Drugs, cholestasis associated with total parenteral nutrition •  Idiopathic

Phototherapy is administered continuously and interrupted only for nursing and feeding purpose. The infant is placed naked with genitalia and eyes covered. Close attention is paid to the infant’s temperature, daily weight and intake output. Breastfeeding is continued frequently. Hypoxia, hypothermia, hypoglycemia, acidosis and sepsis need to be prevented, and if present, treated aggressively. Intravenous fluids are given only to infants who have inadequate oral intake, significant weight loss (> 10%) or are dehydrated.



t

Tex book of Pedia rics

bilirubin is rapidly rising or nearing ET range, intensive PT [using high-intensity light-emitting diodes (LED)] lights or double surface standard PT tubes) is provided.

Monitoring

Prevention

V d

n U

I

ntravenous mmunoglobulin

Practice Points and Tips •

•

Treatment of irect Hyperbilirubinemia

•

D

Intravenous immunoglobulin (IVIG) 500 mg/kg is used when serum bilirubin is rising despite intensive PT or the value is within the ET range in antibody-mediated hemolysis (Rh, ABO) settings.

•

ey Messages

46







• Neonatal hyperbilirubinemia is usually multifactorial in origin. • The most common cause of neonatal hyperbilirubinemia is physiological jaundice, which is a diagnosis of exclusion. • Conjugated hyperbilirubinemia is always pathological, and expert help should be sought early, especially to identify biliary atresia. • Systematic approach in the form of clinical evaluation, assessment of risk factors and interpreting bilirubin with age in hours paves way to rational management.



­

Direct hyperbilirubinemia (Table 2.7.6) is defined as conjugated bilirubin level greater than 2 mg/dL or 15% of the TSB. It is an uncommon but potentially serious condition that indicates hepatobiliary dysfunction. Evaluation should include total and direct bilirubin, urine examination and urine culture, evaluation for infection (as indicated) and thyroid profile. Further evaluation should be tailored with expert consultation to rule out surgical cause. Treatment is directed at the specific underlying cause if any, and remains largely supportive with diet rich in calories, medium chain triglycerides, adequate proteins, and supplementation of fat and water-soluble vitamins. Early detection and diagnosis are important for successful treatment for a favorable prognosis.

Hyperbilirubinemia may develop both in the absence of identifiable risk factors and without clinically significant jaundice having been present at the time of discharge. Hence clinicians should remain alert for jaundice during first postnatal week. Do not rely on visual inspection alone to estimate the bilirubin level in a baby with jaundice. Interpret bilirubin levels according to the infant’s age in hours. Do not subtract conjugated bilirubin from TSB when making decisions. K

ti e





xchange Transfusion

Exchange transfusion is indicated for infants whose bilirubin levels cross the threshold indicated in Tables 2.7.4 and 2.7.5 or those who have clinical features of bilirubin encephalopathy. During ET, twice the infant’s blood volume (160 mL/kg) is exchanged; this procedure can decrease the bilirubin level by approximately 50%. The procedure is invasive and carries a small risk of complications (1– 5%)—fluid overload, infection, electrolyte imbalance, hypoglycemia, thrombocytopenia, thrombosis and death. I

G R

1. Test during pregnancy for ABO and Rh blood types and provide appropriate prenatal care. 2. Assess all infants for risk factors for jaundice prior to discharge, and re-evaluate on day 3–5 when the bilirubin level is highest. 3. Inform parents to look for jaundice and report if there are any concerns. 4. Provide appropriate support and advice to breastfeeding mothers. 5. Follow-up neonates with severe jaundice for hearing loss and other neurodevelopmental sequelae.

E

Total serum bilirubin is monitored every 4–12 hours depending on patient’s age and bilirubin level. Phototherapy is usually discontinued when TSB levels reach a level below PT threshold (Tables 2.7.4 and 2.7.5). It should not be used to treat infants with conjugated hyperbilirubinemia because of the risk of bronze baby syndrome.

vip.persianss.ir

C th n















B

e Newbor

1. American Academy of Pediatrics Subcommittee on Hyperbilirubinemia. Management of hyperbilirubinemia in the newborn infant 35 or more weeks of gestation. Pediatrics. 2004;114(1):297-316. 2. Bell R, Bhutani VK, Bollman DL, et al. (2005). Severe hyperbilirubinemia prevention toolkit, California Perinatal Quality Care Collaborative (CPQCC). [online] Available from http://www.cpqcc.org/quality_improvement/qi_toolkits/ severe_hyperbilirubinemia_prevention_rev_october_2005 [Accessed July, 2011].

3. Guruprasad G, Chawla D, Aggarwal S. (2010). Management of neonatal hyperbilirubinemia. In: NNF Clinical Practice Guidelines. [online] Available from http://nnfpublication.org/ Uploads/Articles/0b79b423-4675-4420-98e6-5d14dd6fceaa. pdf [Accessed July, 2011]. 4. Kaplan M, Wong RJ, Sibley E, et al. Neonatal jaundice and liver disease. In: Martin RJ, Fanaroff AA, Walsh MC (Eds). Fanaroff and Martin’s Neonatal-Perinatal Medicine: Diseases of the Fetus and Infant, 9th edition. Philadelphia: Mosby Elsevier publications; 2011. 5. Yaccha SK. Consensus report on neonatal cholestasis syndrome. Indian Pediatr. 2000;37:845-51.

are of

ibliography

47

vip.persianss.ir

Neonatal nfections I





t

Naveen Jain –

Suspected (clinical syndrome like perforation of viscus, petechial or purpuric rash, chest X-ray consis tent with pneumonia or white cells in normally sterile fluid). ­

D

efinition

–

Tex book of Pedia rics

2.8

pidemiology

•

-

• • • •

S

O

E

Maternal fever (> 37.8°C) in the period from onset of labor to delivery Prolonged rupture of membranes (PROM) for more than 18 hours Spontaneous preterm (< 37 weeks) onset of labor (SPTOL) Preterm (< 37 weeks) premature rupture of membranes (pPROM) Maternal sepsis, urinary tract infection (UTI) or diarrhea within 7 days to the date of delivery Clinical chorioamnionitis in the mother.

E

S

O

tiological gents acteria

Common pathogens in India are Klebsiella, Staphylococcus, Escherichia coli and Pseudomonas. This is very different from the developed nations where Group B streptococci, coagulase negative staphylococci (CONS) and fungi dominate.

–



–

F

–

–

–

–

–

–

B

Low birth weight Babies admitted to NICU and undergoing invasive procedures (ventilation, parenteral nutrition through central catheters, not feeding orally, admission to inadequately staffed units, poor compliance to unit policy on prevention of infections).

A

–

-

– –



isk actors for Late nset Neonatal epsis F

R

• •

-

-

-

-

–

-

-

-

•

isk actors for arly nset Neonatal epsis F

R

Classically sepsis has been differentiated into early onset neonatal sepsis (EONS) and late onset neonatal sepsis (LONS) based on onset before 72 hours of life or after. These two types of sepsis have different risk factors or settings.

-

-

–

-

–



E

t

Sepsis in the newborn is defined when it meets the following criteria: (1) any two clinical signs and (2) any two laboratory signs, (3) in the presence of or as a result of suspect or proven infection (European Consensus statement 2010). 1. Any two clinical signs: – Temperature instability - Core temperature greater than 38.5°C or less than 36°C – Cardiovascular instability - Tachycardia (heart rate 180 beats/min) in the absence of external stimulus or pain or drugs, i.e. “unexplained” increase in heart rate for 0.5–4 hours - Bradycardia (heart rate 100 beats/min) for 0.5 hours in the absence of heart block, external vagal stimulus, beta-blockers - Rhythm disturbances - Reduced blood pressure (systolic BP less than 65 mm Hg in first week and less than 75 mm Hg between 1 week and 1 month) - Mottled skin and impaired peripheral perfusion - Decreased urine output (less than 1 mL/kg/hour) – Respiratory instability - Apneic episodes - Respiratory rate greater than 2 SD (> 50 beats/ min in first week and 40 beats/min between 1 week and 1 month) - Increased oxygen need - Ventilation for acute process for causes other than neuromuscular or general anesthesia – Gastrointestinal - Feed intolerance, abdominal distension, poor sucking – Petechial rash or sclerema – Nonspecific: lethargy, irritability, hypotonia 2. Any two laboratory tests: – Abnormal leukocyte count (> 20,000 × 109/L or less than 4000 × 109/L) – Immature to total neutrophil (I/T) ratio (> 0.2) – Platelet count less than 100,000 × 109/L – C-reactive protein (CRP) greater than 15 mg/L – Procalcitonin (PCT) greater than 2 ng/mL – Metabolic acidosis; base excess (BE) greater than –10 – Blood sugar greater than 180 mg/dL or less than 45 mg/dL confirmed at least two times on ageappropriate infusions 3. Evidence of infection: – Proven (positive culture or microscopy or polymerase 48 chain reaction)

ungi

Fungal infections are more common in babies weighing less than 1,500 g and are likely to be associated in babies with parenteral nutrition, central catheters, abdominal surgeries, broad-spectrum antibiotics or steroids. Both Candida albicans and non-albicans are isolated.

vip.persianss.ir

Herpes infection should be considered in a baby with sepsis like syndrome (presenting after 1 week of life) if markers of bacterial infection are negative. Other viral infections that neonates can be exposed to include chickenpox, vertically transmitted rubella and cytomegalovirus infections.

Procalcitonin is a promising screening tool but the cost and availability are still limited. For the diagnosis of LONS, the PCT test showed better accuracy than the CRP test. Procalcitonin rises earlier in the course of infection.

Parasites

Although the gold standard, blood culture can be negative in infected neonates due to prior use of antibiotics, sampling issues or poor laboratory resources. The value of cultures lies mostly in guiding antibiotic changes in treatment failures and planning antibiotic policy for empiric therapy in that population.

Clinical features of systemic sepsis can be varied and nonspecific and have been described above under definition of sepsis. In neonates with systemic signs of sepsis, presence of convulsions, neck retraction or bulging fontanel must raise the possibility of meningitis. Neonates with septic arthritis or osteomyelitis may not have systemic symptoms and may present with painful limb movement and localized swelling with signs of inflammation. I

Localized nfections

iagnosis

ematological ndices I

H

t n

C

E

F

Meningitis requires modifications in the choice of antibiotics, dose and duration, and hence cerebrospinal fluid examination must be performed in all symptomatic neonates or CRP/ blood culture positive neonates on antibiotics. Cerebrospinal fluid cytology of greater than 30 cells (more than 50% polymorphs), with raised protein (> 100 mg/dL) and/or sugar less than 30 mg/dL may suggest meningitis.

Urine ulture It should be done in infants with failure to thrive, prolonged jaundice and fever since these could be features of UTI. In all infants with obstructive uropathy who are ill, urine cultures should be done. Samples for cultures should be collected by suprapubic puncture.

Total leukocyte count (TLC) has to be interpreted against age-specific normative data (Monroe, Zipurusky). The values as high as 24,000 and as low as 5,000 may be normal and do not suggest infection. Immature to total neutrophil (I/T) ratio (> 0.2) and absolute neutrophil counts have higher specificity, but are often normal early in the course of infection. R

- eactive Protein

C-reactive protein has value in ruling out infection. Once started on antibiotics on clinical suspicion, two negative CRPs 24 hours apart after the baby is asymptomatic gives 99% confidence to stop antibiotics. Also in settings of EONS where antibiotics are started empirically, CRP at 48–72 hours helps in differentiating infected sick infants from those symptomatic due to noninfectious causes. A positive CRP is usually a value greater than 10 mg/L.

A

Treatment

Neonatal sepsis is a serious disease and early diagnosis and treatment is crucial. Currently available tests like total white blood cell count, immature/total ratio and CRP do not have the ability to correctly identify or confirm sepsis early. There are newer tests like interleukin-6 (IL-6) and PCT, but they too have been disappointing.

C

erebrospinal luid xamination

ntibiotics

The indiscriminate use of broad-spectrum antibiotics without appropriate blood cultures and the practice of not stopping their use when no infection is documented have resulted in high antibiotic resistance rates amongst organisms isolated in India. The current data published from India suggests cefotaxime must be avoided as an empiric antibiotic. There is also high resistance to ampicillin and gentamicin. Some evidence suggests that use of amikacin and piperacillin-tazobactam may have low failure rates. There may be a justification in using cloxacillin if the incidence of Staphylococcus is high in a given set-up. Antibiotics like carbapenems and vancomycin should be treated as reserve drugs and be used only if primary treatment plan fails. The recommended duration of antibiotic therapy for uncomplicated culture positive neonatal sepsis (no meningitis, bone and joint or staphylococcal infections) is 7–10 days. Antibiotics may be stopped at 2–3 days in babies in whom these were started empirically, when cultures and CRP are negative and there is improvement symptomatically. In neonates with meningitis or staphylococcal sepsis, the duration of treatment may be 2–3 weeks and for up to 4–6 weeks in bone infections.

D

Infections of the eye (purulent discharge), umbilicus (pus discharge and/or erythema of surrounding skin) and pustules are superficial infections.

lood ulture

C

ystemic nfections I

S

Infection can be local or systemic.

C

F

C

linical eatures

are of he Newbor

Common parasitic infections that neonates can be exposed include toxoplasmosis, malaria and syphilis.

C

Procalcitonin

B

Viruses

49

vip.persianss.ir

S

Intravenous Immunoglobulins There is no proven benefit of IVIG in prevention or treatment of neonatal sepsis (INIS trial). This expensive blood product is not without side effects. Its expensive “off label” use for expected benefits must be discouraged.

•

•

50

Use appropriate drug doses and schedules; refer drug formularies for right diluents, storage, interactions and adverse events expected.















Practice Points and Tips



S

Prevention of epsis Sepsis in the neonate can be prevented by promoting exclusive breastfeeding and simple hand hygiene at the household level and also by preventing applications on the umbilical cord during the first few days of life. Hospital acquired infections can be minimized by goof hand hygiene, promoting provision of breast milk to sick LBW neonates, good adherence to asepsis protocols and strict antibiotic policy that limits its use when required.



Sepsis remains the leading cause of neonatal mortality world over. In the acute phase, hypoglycemia, coagulopathy, organ failures like pneumonia, pulmonary hypertension, shock due to myocardial dysfunction and capillary leaks, renal failure and cholestatic jaundice are not uncommon with Gramnegative sepsis. Meningitis can result in complications such as hydrocephalus and developmental delay.



C

omplications



C

S

upportive are Sepsis is a multiorgan disease that can result in death and disability. Antibiotics alone cannot change the outcome. Supportive care includes ventilation, inotropes, blood products, glucose, and acid-base monitoring and correction, and is the most important determinant of outcome.



Pentoxyphylline and recombinant human activated protein C have been tried, but with no demonstrated benefits.

1. Craft AP, Finer NN, Barrington KJ. Vancomycin for prophylaxis against sepsis in preterm neonates. Cochrane Database Syst Rev. 2000;(2):CD001971. 2. Darmstadt GL, Miller-Bell M, Batra M, et al. Extended-interval dosing of gentamicin for treatment of neonatal sepsis in developed and developing countries. J Health Popul Nutr. 2008;26(2):163-82. 3. Foster J, Richards R, Showell MG. Intravenous in-line filters for preventing morbidity and mortality in neonates. Cochrane Database Syst Rev. 2006;(2):CD005248. 4. Hofer N, Müller W, Resch B. Non-infectious conditions and gestational age influence C-reactive protein values in newborns during the first 3 days of life. Clin Chem Lab Med. 2011;49(2):297-302. 5. Isaacs D. Unnatural selection: reducing antibiotic resistance in neonatal units. Arch Dis Child Fetal Neonatal Ed. 2006;91(1):F72-F74. 6. Lubell Y, Ashley EA, Turner C, et al. Susceptibility of community-acquired pathogens to antibiotics in Africa and Asia in neonates-an alarmingly short review. Trop Med Int Health. 2011;16(2):145-51. 7. McClure EM, Goldenberg RL, Brandes N, et al. The use of chlorhexidine to reduce maternal and neonatal mortality and morbidity in low-resource settings. Int J Gynaecol Obstet. 2007;97(2):89-94. 8. Rossi P, Botgros R. (2010). Report on the expert meeting on neonatal and pediatric sepsis. [online] EMA. Available from http://www.ema.europa.eu/docs/en_GB/document_library/ Report/2010/12/WC500100199.pdf [Accessed September 2012]. 9. Webster J, Pritchard MA. Gowning by attendants and visitors in newborn nurseries for prevention of neonatal morbidity and mortality. Cochrane Database Syst Rev. 2003;(3):CD003670. 10. Wynn JL, Wong HR. Pathophysiology and treatment of septic shock in neonates. Clin Perinatol. 2010;37(2):473-9. 11. Yu Z, Liu J, Sun Q, et al. The accuracy of the procalcitonin test for the diagnosis of neonatal sepsis: a meta-analysis. Scand J Infect Dis. 2010;42(10):723-33.

Others

ibliography



B

•

­

Single-volume Exchange Transfusion A few studies (from India) have demonstrated good benefit in neonates with advanced sepsis—sclerema, persistent hypotension, coagulopathy and metabolic acidosis. Con cerns about use of blood products have limited research in this direction.

•



Granulocyte Colony Stimulating Factors or Granulocyte Monocyte Colony Stimulating Factors The routine use of granulocyte colony stimulating factors (GCSF) or granulocyte monocyte colony stimulating factors (GMCSF) is not recommended. When used in severely neutropenic neonates with proven sepsis, a survival benefit was demonstrated in some studies.

torage of antibiotics: Certain antibiotics like ampicillin, gentamicin and cefotaxime are so inexpensive (the disposable syringe costs more!) that keeping the vials for further use is only associated with risk of nosocomial sepsis. Some antibiotics like meropenem and teicoplanin have a short shelf life of less than 48 hours after reconstitution. Do not combine antibiotics with similar toxicities, e.g. vancomycin and gentamycin. Be willing to de-escalate to a lower antibiotic if culture suggests so. Stop antibiotics if cultures, infection markers and clinical signs allow. No benefit is known by completing a “course of 7–14 days”.



S

A

•



t

t

Tex book of Pedia rics

djunct Therapies in Treatment of epsis

vip.persianss.ir

Neonatal Seizures





Care of the Newbor

2.9

Ruchi Nimish Nanavati

 

   

 

 

 

 

 

 

 

Perinatal events •­ Hypoxic ischemic encephalopathy (HIE) •­ Intracranial hemorrhage (ICH): Germinal matrix intraventricular hem­ orrhage (IVH), subdural hemorrhage, primary subarachnoid hem­ orrhage (well baby with seizures) Metabolic •­ Hypoglycemia: Preterm, low birth weight (LBW), infant of diabetic mother (IDM), sick baby •­ Hypocalcemia –­ Early: Preterm, asphyxia, IDM –­ Late: Top feeding •­ Hypomagnesemia •­ Hyponatremia/hypernatremia •­ Pyridoxine dependency •­ Inborn errors of metabolism (IEM): Non ketotic hyperglycinemia, urea cycle defects, maple syrup urine disease (MSUD), glutaric aciduria II, propionic aciduria, methylmalonic aciduria, mitochondrial disease, Menkes disease, glucagon transporter deficiency Infections •­ Bacterial meningitis •­ Nonbacterial infections: Toxoplasmosis, herpes simplex, Coxsackie B, rubella, Cytomegalovirus Developmental problems •­ Cerebral cortical dysgenesis •­ Lissencephaly, schizencephaly •­ Neuronal migration disorders •­ Pachygyria, polymicrogyria Miscellaneous •­ Passive drug withdrawal •­ Accidental injection of local anesthetic into fetal scalp •­ Drug toxicity •­ Polycythemia •­ Hypertensive encephalopathy •­ Neonatal epileptic syndromes: Benign familial NS, benign idiopathic NS (fifth day fits), early myoclonic encephalopathy, early infantile epileptic encephalopathy (Ohtahara’s syndrome)

Causes

Relative deficiency of inhibitory neurotransmitter

Pyridoxine dependency

Membrane alteration with increased Na+ influx

Hypocalcemia and hypomagnesemia

-

 

Hypoxemia, ischemia and hypoglycemia

 

 

Excess of excitatory neurotransmitter

 

Failure of Na+ - K+ pump secondary Hypoxemia, ischemia and to reduced energy production hypoglycemia

 

 

Probable mechanism

 

 

Table 2.9.1 Mechanisms of seizure

 

 



A seizure results from excessive synchronous electrical discharge, i.e. depolarization of neurons produced by the inward migration of sodium ions. The probable mechanisms for excessive depolarization are shown in Table 2.9.1. The electrical discharges readily generated in the neonatal brain do not propagate sufficiently leading to fragmentary seizures whose electrical activity may not spread to surface electroencephalography (EEG) electrodes. The more advanced development within the limbic system with connections to the midbrain and brainstem explain the higher frequency of subtle features. Dramatic fall in brain glucose within 5 minutes of onset of seizures with concomitant rise in brain lactate may interfere with DNA synthesis, glial proliferation, differentiation and myelination. A single bout of seizures permanently inhibited DNA

Table 2.9.2 Etiology of neonatal seizures (NS)

 

tiopathogenesis

Benign familial neonatal seizure is an autosomal dominant disorder with primary generalized seizures on day 2 or 3 of life without any obvious risk factors. Seizures may recur and gradually resolve over weeks. Later epilepsy is seen in less than 10%.

 

The incidence of NS ranges from 2.8 per 1,000 in term to 57.5 per 1,000 in VLBW infants. Hypoxic ischemic encephalopathy, intracranial hemorrhage (ICH), intracranial infections and developmental defects account for 80–85% of all cases of NS. Indian data is limited.

F

B

enign amilial Neonatal Seizure

 

pidemiology

n

E

H

This is the most common cause; seizures generally start within 24 hours, increase in frequency over 24–36 hours and usually burn out by day 4–5. Subtle seizures are most common.

 

E

Seizure is a paroxysmal alteration in neurological function— behavioral, motor or autonomic.

E

ypoxic Ischemic ncephalopathy

 

Definition

synthesis in the neonatal rat brain. Evidence suggests that NS may predispose to impaired cognitive and behavioral functions or susceptibility to epilepsy later in life. The most common etiologies are listed in Table 2.9.2.

 

Seizures represent the most distinctive manifestation of neurological disease in the newborn period. The tendency toward recurrent seizures and status epilepticus is far greater in the newborn period. It is critical to recognize neonatal seizures (NS), determine their etiology and treat them, as they usually relate to significant illness requiring specific therapy, and seizures themselves may be a cause of brain injury and potential long-term sequelae.

51

vip.persianss.ir

E

arly Myoclonic ncephalopathy

It starts as focal motor fragmentary seizures and later evolves into typical infantile spasms. It is usually associated with non-ketotic hyperglycinemia. EEG reveals burst suppression pattern with poor long-term outcome.

­

­



Investigations The following tests are required with first seizure episode: • Blood sugar • Serum calcium, phosphorus, magnesium • Serum electrolytes • Cerebrospinal fluid study • Cranial ultrasonogram (USG)

 

Table 2.9.3 Neonatal seizures and time of onset Time of onset

Etiology

< 24 hours

Hypoxic ischemic encephalopathy (HIE), severe birth trauma, congenital central nervous system (CNS) anomalies, pyridoxine dependency, hypoglycemia, hypocalcemia, drug withdrawal, intracranial hemorrhage (ICH)

1–3 days

All above + intracranial infections, subarachnoid hemorrhage, inborn errors of metabolism (IEM), benign familial neonatal seizures

> 3 days

Late hypocalcemia, sepsis, meningitis, progressive hydrocephalus, epileptic syndromes, herpes encephalitis, IEM

­

T









Neonatal seizures are difficult to recognize because they are brief, fragmentary, asymmetrical, lack organization and may be associated with non-motor phenomenon. Four patterns are described which are as follows: 1. Subtle seizures: These are the most common sub types (50%), include broad spectrum of behavioral phenomena: • Ocular: Tonic horizontal deviation, ocular fixation, repetitive blinking • Oral-facial-lingual movements • Limb movements: Cycling, peddling • Autonomic phenomena: Tachycardia, bradycardia, irregular respiration, increased blood pressure • Apnea: Rarely the only manifestation, rarely lasts for more than 10–20 seconds, initial tachycardia is common At bedside, subtle seizures are distinguished by their insensitivity to tactile stimulation or restrain and frequent association with autonomic effects. 2. Clonic seizures: These are well localized stereotypic and repetitive biphasic movements. They may be unifocal, multifocal (progression from one part to another in nonordered fashion) or generalized, and are usually not associated with loss of consciousness. Primary generalized clonic seizures are extremely rare. They are provoked by metabolic disturbances, focal traumatic injury, subarachnoid hemorrhage and focal infarct. The most common cause of clonic seizures that remain unifocal is neonatal stroke. EEG reveals unifocal or multifocal abnormality and prognosis is good. 3. onic seizures: They are characterized by sustained extension or flexion of axial or appendicular muscle group and resemble decerebrate or decorticate posture. They may be focal or generalized, often associated 52 with eye deviation or apnea and are most common in preterms with ICH or diffuse CNS disease. Generalized



F

Clinical eatures



These are brief tonic spasms presenting between days 10 and 90 of life. They are usually associated with structural disorders. EEG reveals burst suppression pattern with poor long-term outcome.





E

E

O

E

arly Infantile pileptic ncephalopathy ( htahara’s Syndrome)

tonic seizures may not be associated with timesynchronized EEG discharges. EEG may depict burst suppression pattern and prognosis is poor. 4. Myoclonic seizures: These are characterized by syn chronous single or multiple slow jerks and are asso ciated with diffuse CNS pathology. Focal and multifocal myoclonic seizures are most commonly unassociated with, while generalized myoclonic seizures are more likely to be associated with time-synchronized EEG discharges. EEG may reveal burst suppression or hypsarrhythmia pattern and prognosis is poor. Seizure mimics certain behavioral phenomena, which may be confused with NS. 1. Jitteriness is characterized by tremulous movements (5–6/s), is stimulus sensitive, not associated with abnormal autonomic changes or eye movements and is terminated by passive flexion of extremities. EEG is normal. 2. Benign sleep neonatal myoclonus usually presents in the first week and resolves spontaneously over weeks. It occurs during non-rapid eye movement (non-REM) sleep, abolishes on arousal and never occurs during wakefulness. Neurological examination and EEG are normal. Transient dysmaturity of the brainstem reticular activating system is postulated. Anticonvulsants are not indicated. Long-term outcome is normal. Later epilepsy does not develop. 3. Hyperekplexia is a rare autosomal dominant disorder characterized by hypertonia, hyperreflexia and an exaggerated startle response. The mutation of inhibitory glycine receptor is described. Therapy with clonazepam/ diazepam causes marked improvement. The timing of seizure can also provide some clue to etiology (Table 2.9.3).

F

F

B

Benign idiopathic neonatal seizures have onset between days 4 and 6 of life in term infants with normal neurological state and diagnostic testing. They are probably related to transient zinc deficiency. Long-term outcome is favorable and later epilepsy does not develop. E

ics

atr di

extbook of Pe T

enign Idiopathic Neonatal Seizures ( ifth Day its)

vip.persianss.ir

G: Indicated in all cases of NS requiring anticonvulsant therapy. Ictal EEG is useful for the diagnosis of suspected seizures, while interictal EEG is useful for predicting long-term prognosis. Abnormal background activity indicates a high risk for long-term neurological sequelae. EE

have raised concerns. Attempts should be made to stop AEDs and wean the baby to only phenobarbitone (Flow chart 2.9.2). Monotherapy is most appropriate.

Prognosis

dditional Investigations

Prognosis mainly depends upon gestational age, nature and cause of seizures, neurological examination, EEG and neuroimaging findings. A rough guide of prognosis is provided in Table 2.9.4.

•

ecent dvances

n

R

• Neuroimaging: Computed tomography (CT) scan is advisable when etiology is undetermined after first line investigations and is especially good for hemorrhages and calcifications. Magnetic resonance imaging (MRI) provides better resolution of anatomy and details of function and perfusion with diffusion-weighted MRI, spectroscopy and angiography. Neuroimaging has more value in prognostication. • Metabolic work-up: Inborn errors of metabolism should be suspected with family history of unexplained fetal or neonatal deaths, mental retardation, seizures occurring after introduction of feeds, or those asso ciated with unexplained lethargy, coma and vomiting. Initial work-up includes blood gases (arterial blood gas), blood ammonia, serum and urinary amino acids, serum lactate and pyruvate, and urinary reducing substances. • Sepsis work-up • TORCH screen • Karyotyping • Toxic drug screen • As per suspected etiology.

A

A

Care of the Newbor

•

Criteria for determination of adequacy of therapy had always remained controversial. Current research highlights that the elimination of all seizure activity

­

 

Flow chart 2.9.1 Algorithm for the management of neonatal seizures

T

reatment







Neonatal seizure is a medical emergency and mandates prompt treatment. The algorithm for management of NS is given in Flow chart 2.9.1. Other drugs used in resistant seizures include: • Lidocaine: Intravenous bolus dose of 4 mg/kg followed by 2 mg/kg/hour • Paraldehyde: Intramuscular 0.1–0.2 mL/kg/day or rectal paraldehyde 0.3 mL/kg/day • Pyridoxine: Therapeutic trial of pyridoxine is warranted in refractory seizures. Pyridoxine dependency is diagnosed on rapid cessation of EEG seizures following 50–100 mg IV pyridoxine. • Valproic acid: As adjunctive or maintenance therapy; 20 mg/kg IV/PR/PO is followed by 5–10 mg/kg/day in two divided doses. Concern about hepatotoxicity, hyperammonemia and hyperglycinemia limits its use. • Primidone, carbamazepine, lamotrigine and vigabatrin have been tried in refractory seizures. Importance of history and physical examination to detect the cause of seizures cannot be overemphasized. Treatment of the underlying cause is essential.

Long-term Planning and Prognostication The possible adverse effects on developing CNS of continuing antiepileptic drugs (AEDs) for several months

53

vip.persianss.ir

should be the goal of therapy and not merely elimination of clinical seizures. Fosphenytoin has proved to be a major advance in therapy of neonatal status epilepticus. Topiramate and bumetanide causing inhibition of excitation at excitatory amino acid receptors with potent anticonvulsant and neuroprotective properties hold promising future.

 

atr

ics

Flow chart 2.9.2 Weaning of anticonvulsant therapy

di

•

K

extbook of Pe

•

ey Messages

-











1. du Plessis AJ. Neonatal seizures. In: Cloherty JP, Eichenwald EC, Stark AR (Eds). Manual of Neonatal Care, 6th edition. Philadelphia: Lippincott Williams and Wilkins; 2008. pp. 483-98. 2. Laoria N. Controversies in diagnosis and management of neonatal seizures. Indian Pediatr. 2000;37:367-72. 3. Rennie JM. Seizures in the newborn. In: Rennie JM, Roberton NR (Eds). Textbook of Neonatology, 3rd edition. Edinburgh: Churchill Livingstone; 2000. pp. 1213-22. 4. Tekgul H, Gauvreau K, Soul J, et al. The current etiologic profile and neurodevelopmental outcome of seizures in term newborn infants. Pediatrics. 2006;117:1270-80. 5. Volpe JJ. Neonatal seizures. In: Volpe JJ (Ed). Neurology of the Newborn, 5th edition. Philadelphia: Saunders Elsevier; 2008. pp. 203-44.



ibliography



B









T



• The diagnosis of seizures requires a high index of suspicion, careful clinical observation and often EEG. • Neonatal seizures may predispose to cognitive, behavioral or epileptic complications later in life. • Elimination of all seizure activity should be the goal of therapy and not merely elimination of clinical seizures. • Phenobarbitone is the preferred anticonvulsant for initial and maintenance therapy. • Long term prognostication should be guarded in recurrent or resistant seizures or those with EEG abnormalities.

 

Table 2.9.4 Prognostic outcome in neonatal seizures % Normal

Hypoxic ischemic encephalopathy (HIE)

50

Intracranial hemorrhage (ICH) with hemorrhagic infarct

< 10

Primary subarachnoid hemorrhage

90

Hypocalcemia - Early

50

Hypocalcemia Late

100

Hypoglycemia

50

Bacterial meningitis

50

Central nervous system (CNS) malformation

0

Background EEG

Neurosequelae

Normal

< 10

Severe abnormalities

≥ 90

Moderate abnormalities

~ 50

-

Neurologic disease

54

vip.persianss.ir

Ashok Kumar

Meconium spiration Syndrome (M S)

R

Respiratory distress affects nearly 5–10% of all newborns. The etiology of RD depends on the age of onset of symptoms, gestational age, and maternal factors. Table 2.10.1 summarizes the common causes of RD in newborn. The pathogenesis of the most common causes is outlined in this section.

Clinical eatures F

TT

T

T

ransient achypnea of the Newborn (

N)

It is a benign, self-limiting disorder which commonly occurs in term and late preterm (34–36 weeks) newborns. It is

 

Table 2.10.1 Etiology of respiratory distress (RD) in newborn

                     

 

 

 

 

   

 

 

 

 

 

 

 

 

 

 

 

 

•  Respiratory system – Respiratory distress syndrome (RDS) – Transient tachypnea of the newborn – Intrauterine pneumonia – Meconium aspiration syndrome – Pneumonia – Aspiration pneumonia – Air leak syndromes – Surgical causes: Tracheoesophageal fistula, diaphragmatic hernia, bilateral choanal atresia, and congenital lobar emphysema •  Cardiovascular system – Congestive heart failure •  Central nervous system – Perinatal asphyxia – Intracranial hemorrhage •  Metabolic – Hypoglycemia – Metabolic acidosis •  Miscellaneous – Hypothermia – Polycythemia

In a newborn presenting within the first 6 hours of birth with RD, one should consider the possibilities of RDS, TTN, MAS, congenital pneumonia, air leaks or malformations as the etiology. Neonates with RDS are preterms who present with tachypnea, chest retractions, expiratory grunt, cyanosis, and apneic spells. Neonates with TTN usually have mild to moderate RD with tachypnea, chest retractions and flaring of alae nasi. However, the baby remains active and alert despite severe tachypnea. Quick recovery by 2–3 days helps to differentiate this condition from pneumonia or MAS. In MAS, RD develops soon after birth, manifesting as tachypnea, retractions, and hyperinflated chest, and occasionally grunting. There is history of meconium stained amniotic fluid and/or meconium staining of the skin and babies are often born depressed at birth, requiring resuscitation in the delivery room. If there is history of chorioamnionitis in the mother one may consider the possibility of congenital pneumonia. Air leak should be suspected in any newborn that has received positive pressure ventilation at birth and would have features of decreased air entry and increased resonance on percussion on the side of the air leak and mediastinal shift to the opposite site. If facilities for transillumination are available then air leak can be confirmed on the bed side by the bright transillumination on the side of the air leak. Cardiac disease usually presents beyond the first day of life and features of congestive heart failure, murmurs on cardiac auscultation with or without cyanosis may provide clues to a cardiac etiology.

Surgical Causes of espiratory Distress Suspect esophageal atresia (with/without tracheoeso phageal fistula) if a newborn presents soon after birth with increased salivation, and choking during feeds. Failure to pass a feeding tube into the stomach confirms the diagnosis. Respiratory distress in a baby with scaphoid abdomen and 55 a mediastinal shift should suggest diaphragmatic hernia. Bilateral choanal atresia presents as cyclic RD, particularly ­

The incidence is inversely related to the gestational age. Approximately 50% of newborns delivered at 26–28 weeks of gestation develop RDS, whereas the disease becomes infrequent after 34 weeks. Compared to the West, incidence of RDS is relatively less in India. Factors like male gender, infants of diabetic mothers, multiple gestation, perinatal asphyxia, and cesarean section increase the risk of disease. It is caused by deficiency of surfactant in lungs which is a composite mixture of phospholipids and proteins produced by type II epithelial cells of alveoli.

R

R

espiratory Distress Syndrome

 

Meconium staining of amniotic fluid (MSAF) occurs in 10–15% of deliveries. Approximately, 5% of babies born through MSAF develop MAS. It is common in term and post-term babies, especially those with intrauterine growth restriction.



E

tiopathogenesis of espiratory Distress

A

Respiratory distress (RD) in newborn is the presence of one or more of the following features: respiratory rate greater than or equal to 60/min, chest retractions and grunt.

caused by delayed clearance of fetal lung fluid. Risk factors for Transient tachypnea of the newborn (TTN) include poor respiratory effort at birth, cesarean delivery without the benefit of labor, multiple gestations, delayed cord clamping, male gender, and maternal diabetes. A

Definition

n

R





espiratory Distress

Care of the Newbor

2.10

vip.persianss.ir

ssessment of the Severity of espiratory Distress R

A

cs d

extbook of Pe iatri T

during feeding that disappears on crying or opening mouth, as it bypasses nasal obstruction. Inability to pass a catheter through nose into nasopharynx establishes the diagnosis.

The severity of RD can be judged clinically by the Respiratory Score (Downe Score) as given in Table 2.10.2. The score is used in babies who are breathing spontaneously, including those receiving CPAP. A score of less than 5 indicates mild RD, a score of 5 to 8 moderate RD, and a score of greater than 8 severe RD.

Diagnosis Chest Skiagram

Figure 2.10.1 Chest X-ray of respiratory distress syndrome (RDS) showing diffuse haziness, blurring of cardiac margins and air bronchogram  

This is the most useful investigation that can help in the etiological diagnosis of RD in the newborn. In RDS the radiological features include symmetrical fine reticulogranular pattern, reduced lung volume, diffuse haziness (ground glass appearance), air bronchograms (Fig. 2.10.1) and complete white out of lungs in late stages. In TTN the chest X-ray shows normal or increased lung inflation, streaky perihilar infiltrates, and fluid in horizontal fissure (Fig. 2.10.2). In MAS, chest X-ray shows hyperinflation, coarse irregular opacities and sometimes pneumothorax (Fig. 2.10.3). Air leaks, esophageal atresia, diaphragmatic hernia can all be diagnosed by typical radiological features. Pneumonia may show varying degrees of lung opacities and would have to be correlated clinically. T

Shake est

ther ests T

O

The test is based on the premise that if there is sufficient amounts of surfactant present in the amniotic fluid (or gastric aspirate taken within 30 min of birth), it would generate a stable foam layer at air-liquid interface when mixed with ethanol. Inadequate foam layer could suggest insufficient surfactant and an indirect support for the diagnosis of RDS.

In case pneumonia is suspected then blood culture, CRP, total leukocyte count and absolute neutrophil count should be done.

 

Figure 2.10.2 Chest X-ray of transient tachypnea of the newborn (TTN) showing streaky perihilar infiltrates

 

Table 2.10.2 Respiratory score (Downe score) Score

0

1

2

Respiratory rate

40–60/min

60–80/min

> 80/min

Oxygen requirement

None

≤ 50%

> 50%

Retractions

None

Mild to moderate

Severe

Grunting

None

With stimulation

At rest

Breath sounds

Normal

Decreased

Barely heard

Prematurity

> 34 weeks

30–34 weeks

< 30 weeks

56

vip.persianss.ir

n

Care of the Newbor Figure 2.10.4 Chest X-ray of the same baby as in Figure 2.10.1, showing considerable clearing of lungs 5 hours after giving surfactant

R

xygenation

Provide oxygen by head box to maintain target oxygen saturations (SpO2) in the 88–95% range. Oxygen therapy must be monitored by pulse oximeter to avoid hypoxia or hyperoxia, both of which are harmful to the baby. A

Continuous Positive irway Pressure In neonates with RDS, continuous positive airway pressure (CPAP) is a simple, safe, and effective method to improve oxygenation. It works by preventing alveolar atelectasis, thereby reducing work of breathing. Continuous positive airway pressure is utilized in spontaneously breathing babies. Intractable apnea is a contraindication for CPAP. Early CPAP use is more effective and may reduce the need of mechanical ventilation. Continuous positive airway pressure is best delivered through short nasal prongs. Begin CPAP at 5–6 cm H2O pressure (maximum 8 cm H2O). Insert orogastric tube to prevent gastric distension from swallowed air (CPAP belly). Inability to maintain SpO2 greater than 85% at 60– 80% FiO2 indicates need for mechanical ventilation.

Mechanical Ventilation Mechanical ventilation should be considered in infants with RD who fail CPAP or have recurrent apneas or are in shock or have evidence of respiratory failure (blood gas revealing PaCO2 greater than 50 mm Hg and PaO2 lesser than 50 mm Hg).

­

Surfactant replacement therapy (SRT) should be consi dered in neonates with RDS. Surfactant is instilled directly into the lungs through endotracheal tube. In symptomatic babies early rescue therapy (within 2 hours) is better than delayed therapy. Prophylactic SRT (before the onset of RD) is indicated for newborns under 26 weeks’ gestation and to those preterms who require intubation for stabilization in delivery room. Natural surfactants are better than synthetic preparations. The dose is 100– 200 mg/kg. Single dose suffices for most infants. Dose may be repeated 6–12 hours later, if significant distress persists. Monitor baby carefully after SRT. Within a few minutes, there is improvement in gas exchange, oxygen requirement comes down and ventilator settings must be adjusted to prevent ventilator induced lung injury. Figure 2.10.4 shows radiological improvement in lung status after surfactant therapy.

ntibiotics

When pneumonia is suspected, appropriate antibiotics such as ampicillin/cephalosporin and an aminoglycoside should be initiated along with other supportive care.

Complications Acute complications include air leaks, patent ductus arteriosus (PDA), intracranial hemorrhage, and infection. Long term complications in preterms include retinopathy of prematurity, bronchopulmonary dysplasia (BPD), neuro developmental impairments, and other complications of prematurity. ­

O

Treatment is largely supportive. The infant should be nursed under a radiant warmer. Most newborns with RD are not usually capable of being fed and need to be initially kept on IV fluids.

T

Surfactant eplacement herapy

reatment

A

T

 

 

Figure 2.10.3 Chest X-ray of meconium aspiration syndrome (MAS) showing patchy hyperaeration and heterogeneous opacities in lung fields

57

vip.persianss.ir

B







1. Guha’s Neonatology. Principles and Practice (Volume 2). In: Guha DK, Saili A, Bhat S, Shenoi A (Eds), 3rd edition. Jaypee Brothers: New Delhi; 2005. 2. Sweet DS, Carnielli V, Greisen G, et al. European consensus guidelines on the management of neonatal respiratory distress syndrome in preterm infants–2010 update. Neonatology. 2010;97:402-17. 3. Warren JB, Anderson JM. Core concepts: respiratory distress syndrome. NeoReviews. 2009;10:e351-61. 4. Welty S, Hansen TN, Corbet A. Respiratory Distress in the Preterm Infant. In: Taeusch HW, Ballard RA, Gleason CA (Eds). Avery’s Diseases of the Newborn, 8th edition. Philadelphia: Saunders; 2005. pp. 687-703.



• The most common cause of RD in preterm newborns is RDS and TTN in late preterm and term newborns. • Good supportive care improves outcome. • Monitor oxygen therapy with pulse oximetry. Unmonitored oxygen use is dangerous. • Oxygen administration by hood is effective in mild RD. • Continuous positive airway pressure is effective and cheap method to provide respiratory support in mild to moderate RD.



K

ey Messages

ibliography



R

cs

• Mechanical ventilation is the modality of choice for treating severe RD. • Always obtain blood culture and sepsis screen before starting antibiotics. • Single course of antenatal steroids between 24 weeks and 34 weeks of gestation reduces the incidence of RDS by 50%. • Surfactant replacement therapy is most effective when it is given within 2 hours of development of symptoms (early rescue therapy).

Respiratory distress syndrome can be prevented by using antenatal corticosteroids to pregnant women between 24 weeks and 34 weeks of gestation with threatened preterm labor. A complete course consists of two doses of betamethasone (12 mg IM) at 24-hourly interval or four doses of dexamethasone (6 mg IM) at 12-hourly intervals. Betamethasone is better than dexamethasone. Multiple courses of steroids are not recommended in view of modest incremental benefit and real risk of brain damage to the fetus.

T

d

extbook of Pe iatri

Prevention of espiratory Distress Syndrome

58

vip.persianss.ir

B





Care of the Newbor

2.11

leeding Neonate JN Sharma

The hemorrhagic disorders in the newborn are a group of disorders with different etiologies, which have in common an abnormal tendency to bleed. This abnormal tendency to bleed is due to a defect in the mechanism of hemostasis.

A hemorrhagic disorder should be suspected when there is: • Spontaneous bleeding into the skin, mucous memb­ rane and internal tissues, joints. Bleeding from gastrointestinal tract is a common form of mucosal bleeding in the newborn due to hemorrhagic disorders. • Excessive or prolonged bleeding following trauma or surgery: A postoperative or traumatic bleed may be the first manifestation of a coagulation disorder • Bleeding from more than one site • Associated family history of abnormal bleeding. Points in history should include gestation and birth weight, day of onset of bleeding, type of bleeding viz. skin, bleeding per rectum, hematemesis, bleeding from IV sites, family history of bleeding disorder, maternal history of idiopathic thrombocytopenic purpura (ITP), systemic lupus erythematosus (SLE), maternal drug ingestion, history of birth trauma and complicated delivery and administration of vitamin K at birth. What is not a bleeding disorder? There are some conditions which present with bleeding or mimic bleeding but are not due to a bleeding disorder. They include the following: • Swallowed maternal blood by the newborn • Bleeding from an umbilical granuloma • Hormone withdrawal vaginal bleeding • Urate crystals which stain the nappy • Subconjunctival hemorrhage and retinal hemorrhage and petechiae of the skin of head and neck region, which are common during passage through the birth canal or may result from venous obstruction. In vascular disorders, the bleeding is usually confined to the skin and may cause petechiae and ecchymosis. In platelet disorders, petechial bleeding is common, ecchymosis present is usually not larger than 2 cm in diameter and bleeding from mucous membranes is prominent. In coagulation disorders, petechial hemorrhage is rare. Ecchymosis tends to be larger than in platelet and vascular disorders. Bleeding more frequently occurs in deeper tissues. Time of onset of bleeding manifestations often gives a clue to diagnosis. Onset of bleeding between 2 days and 6 days indicates classical hemorrhagic disease of newborn. Immune mediated thrombocytopenia usually manifest within first 48 hours of age. Early onset bleeds are associated with intrapartum events and maternal status whereas late onset bleeds are usually secondary to infections.

Pathogenesis The newborn has reasons to be susceptible to bleeding complications due to physiological handicaps. None of the coagulation factors cross the placenta from the mother to the fetus. At birth, the concentration of vitamin K dependent factors (factors V, factor VII, factor IX and factor X) and contact factors (factor XI and factor XII) are reduced to about 50% of normal adult values and are further lower in preterm infants. Both thrombin generation and thrombin inhibition are reduced in the neonatal period and there are low levels of plasminogen.

n



-

–

–

–

–

–

–

–

–

The causes of bleeding in the neonate may be: • Hemorrhagic disease of newborn (HDN) • Platelet disorders – Qualitative disorders, e.g. Glanzmans disease (throm­ bosthenia), use of aspirin by mothers – Quantitative disorders, e.g. autoimmune and alloim­ mune thrombocytopenia, disseminated intravascu­ lar coagulation, giant hemangioma, necrotizing en­ terocolitis, renal vein thrombosis • Inherited deficiency of clotting factors – Sex linked recessive (expressed in males): Hemo­ philia, Christmas Disease – Autosomal dominant (expressed in both sexes with one parent affected): von Willebrand disease, dysfibrinogenemia, factor XI deficiency. – Autosomal recessive (expressed in both sexes with parents as carriers): Deficiency of clotting factors I, II, V, VII, X, XII, XIII, variants of von Willebrand disease. • Increased capillary fragility and supporting structures causing bleeding into skin (e.g. breech delivery, trau­ matic delivery) intraventricular hemorrhage. • Traumatic causes – Rupture of liver and spleen associated with breech delivery – Retroperitoneal or intraperitoneal bleeding – Subdural hematoma, cephalhematoma, subgaleal hemorrhage.



tiology

F

Clinical eatures

E

Introduction

59

vip.persianss.ir

•

The management of a bleeding neonate includes supportive treatment with blood component therapy and identification and treatment of the cause.

If there is significant hemorrhage and the infant is in shock, it will present with cold peripheries, tachycardia, CRT greater than 3 seconds and a blood pressure less than 35 mm Hg. The hemoglobin level may not fall for 2–3 hours. The infant in shock requires a rapid transfusion of 15–20 mL/kg of uncross matched O negative blood or 10–20 mL/ kg of normal saline over 5–15 min. If there are no signs of recovery from shock another bolus should be given over 15–20 min. S

Infant not in hock • •

• •

If the infant is not in shock, administer vitamin K 1 mg IV immediately if not given at birth. The infant may need a transfusion with packed red cells 10 mL/kg over 2–3 hours to raise Hb to 10–12 g/dL. It needs mention that 10 mL/kg of packed red cells will raise Hb by 2–3 g/dL or the hematocrit (Hct) by 10%. All critically ill infants should be given IV fresh frozen plasma (FFP), 10 mL/kg if PT and PTT are prolonged. Platelet transfusion should be given when platelet count is less than 20,000/cumm.

Neonatal lloimmune hrombocytopenia T



Infant in hock S

Essential investigations for all cases include Hb esti­ mation, red cell morphology, total leukocyte count, differential leukocyte count, platelet count and reti­ culocyte count. • pt test: This test should be performed when there is only gastrointestinal bleeding in a well neonate in the first 48 hours of life and is used to distinguish maternal from neonatal blood. One part of vomitus is mixed with five parts of distilled water and centrifuged. To the pink centrifuged supernatant fluid, 1 mL of 1% sodium hydroxide is added and wait for 1–2 min. If the solution changes to yellow brown color it favors possibility of swallowed maternal blood (HbA gets denatured by alkali while HbF stays pink). • Screening tests for hemorrhagic disorders include platelet count and examination of blood film for number, morphology and presence of platelet clumping, the bleeding time, prothrombin time (PT), activated partial thromboplastin time (APTT) and thrombin time. • Special tests are required to (a) identify the deficient coagulation factor, (b) to determine the degree of deficiency and (c) to detect and quantitate immune inhibitors. They include prothrombin consumption test, coagulation factor assays and platelet function tests. A neonate who has a positive bleeding history or is having active bleeding should have a platelet count, bleeding time, PTT and PT done. If the results are normal, a thrombin time and von Willebrand factor (vWF) testing should be considered. If the initial test results are abnormal, special tests should be planned. Table 2.11.1 provides a differential diagnosis of the causes of bleeding based on clinical status and screening laboratory tests. Table 2.11.2 provides a differential diagnosis of coagulation disorders based on laboratory tests while Table 2.11.3 gives the etiology of neonatal thrombocytopenia.

A

D

Management

A

i

ic

extbook of Ped atr s T

Laboratory iagnosis

Therapeutic interventions include platelet transfusion and administration of IVIG. Platelet transfusion is indicated when platelet count is less than 30 × 109/L. In infants with no bleeding, but platelet counts between 30–50 × 109/L use of IVIG in the dose of 1 g/kg/day on two consecutive days can effectively raise the platelet count.

 

Table 2.11.1 Diagnostic possibilities based on laboratory tests Clinical evaluation

Platelet count

PT

APTT

Diagnostic possibilities

Sick neonate

Decreased

Increased

Increased

DIC

Decreased

Normal

Normal

Platelet consumption (infection, NEC, renal vein thrombosis, giant hemangioma, polycythemia)

Normal

Increased

Increased

Liver disease, heparin

Normal

Normal

Normal Neonate

Altered vascular integrity (hypoxia, acidosis, extreme prematurity, hyperosmolarity)

Decreased

Normal

Normal

Immune thrombocytopenia, thrombosis, bone marrow hypoplasia

Normal

Increased

Increased

Hemorrhagic disease of newborn

Normal

Normal

Increased

Hereditary clotting factor deficiencies

Normal

Normal

Normal

Bleeding from local factors, swallowed maternal blood, qualitative platelet abnormalities, factor XIII deficiency (rare)

Healthy neonate

60

Abbreviations: PT, Prothrombin time; APTT, Activated partial thromboplastin time; DIC, Disseminated intravascular coagulopathy; NEC, Necrotizing enterocolitis

vip.persianss.ir

Care of the Newbor

Abnormality

Diagnosis

Long PTT + normal PT + normal platelets + normal bleeding

Factor VIII, IX, XI, XII deficiency

Long PTT + normal PT + normal platelets + long bleeding time

von Willebrand disease

Long PTT + long PT + normal platelets

Vitamin K deficiency

Long PTT + long PT + decreased platelets

DIC or generalized coagulopathy

Normal PTT + long PT

Factor VII deficiency

Normal PTT + normal PT + normal platelets + normal bleeding time + bleeding especially umbilicus

Factor XIII deficiency

Normal PTT + normal PT + low platelets

ITP, aplasia, leukemia, etc.

Many petechiae and normal platelets

HSV, CMV, infections

n

 

Table 2.11.2 Differential diagnosis of clotting disorders

Abbreviations: PT, Prothrombin time; PTT, Partial thromboplastin time; ITP, Idiopathic thrombocytopenic purpura; DIC, Disseminated intravascular coagulopathy; HSV, Herpes simplex virus; CMV, Cytomegalovirus  

Table 2.11.3 Causes of fetal and neonatal thrombocytopenia Time of onset

Possible etiology

Fetal thrombocytopenia

1. 2. 3. 4.

Early onset neonatal thrombocytopenia (< 72 hours)

1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11.

Late onset neonatal thrombocytopenia (> 72 hours)

1. Late onset sepsis 2. NEC 3. Congenital infections (e.g. CMV, Toxoplasma, rubella, HIV)

Placental insufficiency (PET, IUGR, diabetes) Perinatal asphyxia Perinatal infections (e.g. E. coli, Group B streptococci, H. influenzae) DIC Alloimmune thrombocytopenia Autoimmune thrombocytopenia (maternal ITP, SLE) Congenital infections (e.g. CMV, Toxoplasma, rubella, HIV) Thrombosis (e.g. aortic, renal vein) Kasabach-Merritt syndrome Metabolic diseases (e.g. Propionic and methyl malonic acidemia) Congenital inherited syndromes (e.g. TAR, CAMT)

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Alloimmune thrombocytopenia Congenital infections (e.g. CMV, Toxoplasma, rubella, HIV) Aneuploidy (trisomy 13, 18, 21) Autoimmune thrombocytopenia (ITP, SLE, severe Rh hemolytic disease)

Abbreviations: CMV, Cytomegalovirus; HIV, Human immunodeficiency virus; ITP, Idiopathic thrombocytopenic purpura; PET, Positron emission tomography; IUGR, Intrauterine growth restriction; DIC, Disseminated intravascular coagulopathy; TAR, Thrombocytopenia with absent radii; CMAT, Congenital amegakaryocytic thrombocytopenia; NEC, Necrotizing enterocolitis; SLE, Systemic lupus erythematosus

• Bleeding is more common and severe in preterm and LBW infants due to exaggeration of this deficiency state with or without superimposed problems, e.g. sepsis, asphyxia, etc. • The most common cause of bleeding neonate is classical HDN, which can be prevented. Hence all neonates should receive one dose of vitamin K after birth as a prophylactic measures.

B

1. Andrew M, Paes B, Milner R, et al. Development of the coagulation system in the full term infant. Blood. 1987;70:165 172. 2. Andrew M, Paes B, Milner R, et al. Development of the human coagulation system in the healthy premature infant. Blood. 1988;72:1651 7. -



61

-

• Coagulation factors do not cross the placenta. The coagulation mechanism is handicapped in the newborn due to physiological deficiency of vitamin K dependent coagulation factors resulting in prolongation of PT and PTT however, not necessarily associated with clinical bleeding.

ibliography



ey Messages



K

The mother should be advised prednisolone 10–20 mg qid for 10–14 days or IVIG prior to delivery. The baby should be delivered by lower segment cesarean section (LSCS) if fetal scalp platelet count is lesser than 50 × 109/L. Postnatally the neonate should be treated with platelet transfusion, steroid and IVIG.



T

A

Neonatal utoimmune hrombocytopenia

vip.persianss.ir



-







-

-



-



-





T



8. Kuhle S, Mitchell L, Massicotte P, et al. Hemostatic disorders of Newborn. In: Taeusch HW, Ballard RA, Gleason EA (Eds). Avery’s Diseases of the Newborn, 8th edition; 2003. 9. Pramanik AK. The Bleeding Neonate. In: Meherban Singh (Ed). Medical Emergencies in Children, 3rd edition. New Delhi: Sagar Publications; 2000. 10. Roberts I, Murray NA. Neonatal Thrombocytopenia: causes and management. Arch Dis Child Fetal Neonatal Ed. 2003;88:F359 F364.







i



ic

extbook of Ped atr s

3. Anupama Deka Roy. Bleeding Neonate. In: Sharma JN (Ed). Manual of Care of Critical Newborn, 2nd edition. New Delhi: Peepee Publishers and Distributors (P) Ltd; 2011. 4. Arasan DG. Approach to Bleeding Neonate. Indian J Pract Pediatr. 2010;12:401 11. 5. Buchman CR. Coagulation disorders in neonates. Pediatr Clin N Am. 1986;33:203 20. 6. Homans A. Thrombocytopenia in the neonate. Pediatr Clin North Am. 1996;43:737 53. 7. Jadav D, Chandra J. Petechial rash in neonates: Management algorithm. J Neonatol. 2008;22:19 27.

62

vip.persianss.ir

Section

3 Growth and Development













3.1 3.2 3.3 3.4 3.5 3.6 3.7

















Section Editor : KN Agarwal Growth and Development: Basic Concepts: Dilip Mukherjee Growth—Birth to Puberty: KN Agarwal Who Under-5 Growth Standards 2006: Vaman Khadilkar Growth and Sexual Development in Puberty: KN Agarwal Normal Development: DK Agarwal Developmental Delay: MKC Nair Failure to Thrive: Madhulika Kabra

vip.persianss.ir





cs t

ook o Pe ia ri

3.1

Growth and Development: Basic Concepts

ssessment of Growth and Development



Growth can be measured in terms of: • Physical anthropometry (weight, height, circumferences of head, chest, abdomen and pelvis) • Assessment of tissue growth (skin fold thickness and measurement of muscle mass) • Bone age (radiological by appearance and fusion of the various epiphyseal centers) • Dental age (by counting the number of erupted teeth) • Biochemical and histological means. Development can be studied under motor (gross and fine motor), linguistic, adaptive and personal social behavior category.

Length Until 24 or 36 months of age, length in recumbency is measured using an infantometer (see Chapters 1.3 and 3.2). The length is recorded in centimeters up to one decimal point.

Height After the age of 2 years, standing height is recorded by a stadiometer. The details have been provided in the Chapters 1.3 and 3.2. For community survey, portable types of anthropometric rods are also used. For recording stature (height), the subject should remove his/her socks and shoes and stand perfectly straight with arms relaxed by his/her sides and ankles and knees together. Before measurement starts, a gentle pressure may be applied over the spine with one hand while other hand holds the anthropometric rod. The subject’s head is positioned in Frankfort plane [a line passing through the inferior margin of the orbit (orbitale) and upper margin of external auditory meatus (porion)].

A

Physical nthropometry

get expanded due to repeated use, may get rusted and variation of temperature may give false reading. A special type of new weighing scales is recently devised in Japan, which in addition to the weight of an individual, also notes body mass index (BMI) and amount of subcutaneous fat.

Growth denotes an increase in size of an individual due to increase in the number and diameter of the cells, and development denotes functional maturity of the child. Growth and development are not synonymous but they are assessed simultaneously. The growth performance in a child should be coupled with assessment of the development to get an idea of the child’s performance in overall growth. Growth and development begin at conception and end at maturity. They are unique characteristics of children and any obstacle in this process at any stage can possibly result in aberration of growth and/or development. A

Tex

tb

f

d

Dilip Mukherjee

Physical anthropometry should be done in every child from birth till maturity at regular intervals. The methods of assessment are given in chapters 3.2 and 3.4. It needs to be emphasized that the growth measurement needs to be done meticulously with absolute precision and recorded, so as to allow us on subsequent visit, to ascertain whether the child has grown optimally. The measurements should preferably be done by the same person on calibrated checked equipment to avoid personal human errors.

Weight



The weighing scales best suited are those, which are designed on balance arm principle. Accuracy up to 0.1 kg is acceptable. For smaller babies, machines of more accuracy are required as 0.1 kg forms a higher percentage of total body weight. More recently, many electronic weighing scales giving accuracy up to 0.01 kg have been made available. The weighing scales should be checked for accuracy using known weight from time to time. The beam scales are better instruments for all purposes rather than spring 64 weighing scales, i.e. bathroom scales, as the spring may

Sitting Height For recording sitting height, the subject is made to sit on a table or other convenient hard surface so that his/her head lies in Frankfort plane. The back should be straight, thighs horizontal and comfortably positioned. The feet should be supported on the foot board and hands should rest comfortably on the subject’s lap. To ensure that the subject’s back is fully extended, the observer may run his/ her index finger up the spine applying pressure to the lumbar and sacral regions, causing the subject to set up a reflex action. The head board should be lowered and made to touch the head of the subject, and reading should be recorded to the nearest completed unit.

Body Proportions The total body length is divided into two segments. The upper segment (US) is from head to symphysis pubis and lower segment (LS) from symphysis pubis to the toes. The upper to lower segment ratio is 1.7:1 at birth. By 6–7 years, it reaches 1:1. If the ratio is infantile after 1 year of age, it suggests short limb dwarfism due to bone disorders, such as rickets and hypothyroidism.

vip.persianss.ir

Midarm/Head Circumference Ratio

This measurement is done for special purposes and is not used in routine clinical practice. It is measured with a special caliper, skinfold caliper.

Triceps Skinfold Thickness -

The skinfold is picked up over the posterior surface of the triceps muscle, 1 cm above the mark on a vertical line passing upward between bony point identified for taking measurement, maintaining a pressure of 10 g/mm2 on the caliper and freeing the skinfold from the underlying muscle with left hand between thumb, index and middle finger and holding caliper with the other hand. The reading is recorded to the nearest 0.1 mm, maintaining pressure of caliper as before.

Biceps Skinfold Thickness For recording biceps, the child is made to stand erect, facing the observer with arm on side and palm facing forward. The skinfold is picked up over the belly of biceps and 1 cm above the line marked for the upper arm circumference and triceps skinfold on a vertical line joining antecubital fossa to the head of humerus. The caliper is applied at the marked level and reading is recorded to 0.1 mm.

Bone ge or Skeletal

It is based on the relationship between weight and height and is expressed as weight (kg)/height (cm) × 100. Normal value varies from 0.14 to 0.16. In gross malnutrition, it is less than 0.14. It is a quite reliable ratio for assessing malnutrition.

Mid-upper Arm/Height Ratio It is also a very good indicator of nutritional status. A ratio of less than 0.29 indicates gross malnutrition, while the normal value ranges from 0.32 to 0.33.

aturity

Appearance and fusion of various epiphyseal centers follow a definite sequence related to chronologic age from birth to maturity. Radiological examination of left wrist and elbow is usually considered for bone age assessment. X ray of the lower end of femur, and talus is used for the assessment of maturity of newborn babies. The details of appearance and fusion of various centers are given in subsequent sections. -

Quetlet’s Index

wt p





Tissue Growth

A

It is a simple and useful criterion for detection of malnutrition. A ratio 0.280–0.314 indicates early malnutri­ tion, 0.250–0.279 moderate, and less than 0.249 denotes severe malnutrition.

Cube root of body weight (kg) Ponderal index (PI) is similar to BMI and used in defining newborn with intrauterine growth retardation (IUGR).

-

The degree of wasting can be measured by comparing the child’s weight with expected weight for a healthy child of the same height. Combinations of these measurements have been used to distinguish different types of malnutrition. Waterlow suggested that weight for height can be used to distinguish between malnutrition of recent origin, i.e. wasting and malnutrition due to a considerable period of month, i.e. stunting. In chronic malnutrition the child is stunted with the weight for age and height for age being low. In acute malnutrition, height for age is normal but weight for age is low (wasting). In nutritional dwarf (short stature) the weight/height is equal; the child may pass off as a normal child of lower age if the chronological age is not known. These have been discussed in the Chapter 4.3 “Malnutrition.”





Weight for Height

nd



Height (cm)

nt



PI =



As the MAC is relatively constant between 16.5 cm and 17.5 cm in 1–5 years of age, this measurement may be considered as an age independent variable up to 5 years of age. Any child whose MAC is less than 12.5 cm up to 5 years of age, is considered malnourished. Shakir’s tape also measures the MAC. A bangle of 4 cm in diameter, used in field studies is not a reliable method (Bangle test).

M

A

I

Ponderal Index

A

Midarm Circumference

Height (m)2 Body mass index (BMI) is similar to Quetlet’s except that the values are in SI units. Body mass index values can be used to draw standardized percentile curves in children and adolescents. It is especially useful for defining obesity. Body mass index values above 95th percentile for age are usually used to define obesity.

Develo me

ge ndependent nthropometry

Weight (kg)

BMI =

ha

The details of measuring head and midarm circumference (MAC) are given in Chapter 1.3. It should be kept in mind that upper arm circumference can be measured both in flexed and extended positions and also either at the maximum circumference of biceps muscle or midpoint, as the difference between the two is negligible. Chest circumference for boys, prepubertal girls and men can be recorded at the level of nipples during normal breathing. It is recorded to the nearest 0.1 cm.

Body Mass Index

Gro

Body Circumferences

Dental Development Eruption of teeth follows a definite sequence. Eruption of temporary or deciduous teeth begins at about 6 65

vip.persianss.ir

Cross-sectional Study This is a very convenient, easy, less time consuming and economical method to study physical anthropometric growth. For example, healthy children of each age group and gender (minimum being 200 at each point) are measured for their weight, height and other parameters are recorded and an average is found out. These groups of children are studied just once in 20 years to develop national norms. L

inear or ongitudinal Study

In this type of study, the same child is measured from birth to maturity at previously decided regular intervals. It is difficult to study very large number of children in this type of study and hence, the linear studies have comparatively less sample size. The longitudinal study helps us to determine the growth velocity and effect of nutrition, illness and environment on growth.

Concept of Percentiles

-

While making various calculations, the use of terms like mean or average and standard deviation (SD) are well known. While expressing the growth, the term percentile or centile is often used. This may be explained in a simple way, e.g. the height of hundred 1 year old normal children is not exactly the same. They are arranged in such a way that the shortest is number 1 and the tallest is number 100. Rows of children are thus made. The mean of each number is worked out. The child at number 1 is 1 percentile, number 10 is 10th centile, and number 50 is 50th centile and so on. The child -



-

Development refers to qualitative and quantitative changes and acquisition of a variety of competencies for functioning optimally in a social milieu. Further, development is a continuous process from birth to maturity. It depends on maturation and myelination of brain; unless that has occurred, no amount of practice can make the child learn that skill. The process of development is an interaction between the child and his/her required environment. It may be stressed that besides 10% prevalence of development delay, the early identification remains difficult. Although severe disorders can be recognized, in infancy, it is usual to diagnose speech impairment, hyperactivity or emotional disorders before the age of 3 or 4 years, and learning disabilities are rarely recognized before children start schooling. If one can diagnose early stage developmental delay in early stages of growth, the intervention can minimize long term quantum of disability. It takes a long time, great patience and perseverance to assess the development of a child. It should be done with cooperation of the child and parents. The development is assessed by Gesell’s method, Denver Development Screening Test (DDST), Bayley Scale of Infant Development, Brazelton Neonatal Behavioral Assessment Scale (NBAS), Baroda Scale, Trivandrum Developmental Screening Chart (TDSC) and others (Table 3.1.1). The detailed discussion on development assessment is given in Chapters 3.5 and 3.6.

Growth Studies and Percentiles

L

ssessment of Development

A

cs f

d

t

ook o Pe ia ri tb

Tex

months with upper or lower central incisors, followed by lateral incisor. By 1 year of age 4–8 teeth are present. The permanent teeth begin to erupt at 6 years. Details of dental development are provided in subsequent chapters.

 

Table 3.1.1 Developmental screening tests that can be used for children less than 3 years

66

No.

Name of the test

Age range

Domains of development identified

Administration time (in minutes)

I

Developmental observation card

0–1 year

Social smile, head holding, sitting, standing

3

II

Trivandrum developmental screening chart

0–2 years

Personal social, fine motor, language and gross motor

5

III

Baroda development screening test

0–2.5 years

Gross motor, fine motor, cognitive, language

10

IV

Denver developmental screening test

2 weeks to 6 years

Personal social, fine motor, language, gross motor

20

V

Gesell developmental schedules

4 weeks to 6 years

Language, fine and gross motor, cognitive and personal social

Not specified

VI

Receptive expressive emergent language scale

0–3 years

Receptive language and expressive language

20

VII

Vineland adaptive behavior scale

0–19 years

Communication, daily living skills, socialization and motor skills

25

VIII

STYCAR vision test

6 months to 7 years

Integrity of vision

20

IX

STYCAR hearing test

6 months to 7 years

Integrity of hearing

20

X

Checklist for autism in toddlers

18–36 months

Screening for autism

10

vip.persianss.ir

Britain, Tanner’s growth charts are used. In India, Indian Academy of Pediatrics (IAP) has come out with chart based on studies on affluent Indian children. Presently, the World Health Organization (WHO) growth charts are being preferred, especially for children less than 5 years of age.



nd

ha

wt

Gro

on 10th centile on height chart means that 9 children are less in height and 90 children are more in height. The 50th centile is the median value and is also termed the standard value. Accepted range for normal is between 3rd percentile and 97th percentile. The SD charts are based on distribution of data above and below a mean value. The average normal range falls above and below 2 SD expressed as 2 SD. ±1 SD is equal to 84th centile and −1 SD is equal to 16th centile. ±2 SD corresponds to 97th centile and −2 SD corresponds to 3rd centile. The mean ± SD curves are useful for quantifying the degree of retardation exactly.

Develo me

1. Agarwal DK, Agarwal KN, Upadhyay SK, et al. Physical and sexual growth pattern of affluent Indian children from 5 to 18 years of age. Indian Pediatr. 1992;29:1203 83. 2. Agarwal DK, Agarwal KN. Physical growth in Indian affluent children (Birth to 6 years). Indian Pediatr. 1994;31:377 413. 3. Falknar F, Tanner JM. Human Growth, 2nd edition, 3 vol. New York: Plenum; 1986. 4. Khadilkar VV, Khadilkar AV, Choudhury P, et al. IAP growth monitoring guidelines for children from birth to 18 years. Mumbai: Indian Academy of Pediatrics; 2005. 5. Mukherjee D, Nair MKC. Growth and Development. New Delhi: Jaypee Brothers; 2008. 6. Tanner JM. Growth at Adolescence, 2nd edition. Oxford: Blackwell Scientific Publication; 1962. 7. Tanner JM. Physical growth and development. In: Textbook of Pediatrics, Forfar JO, Arneil GC (Eds), 2nd edition. London: Churchill Livingstone; 1978. pp. 249 303. 8. Tanner JM, Whitehouse RH, Cameroon N, et al. Assessment of Skeletal Maturity and Prediction of Adult Height, 2nd edition. London: Academic Press; 1983.

nt









-

-

These charts are developed by long term longitudinal studies. Velocity charts show the rate of change, which could be due to chronic illness, nutrition or growth hormone (GH) deficiency. Different countries may use their own growth charts. In Great





Velocity Growth Charts



Growth chart is the most important tool in assessment of growth of an individual child. A standard chart contains weight for age, height for age and weight for height. The head circumference is included for first 3 years of life. They depict mean, ±SD or percentile values at each age. The available charts are discussed in Chapters 3.2 and 3.3.



Growth Charts



-

p





-





Bibliography

67

vip.persianss.ir

B





Growth— irth to Puberty

D

KN Agarwal

Growth is a continuous process commencing at conception and progressing at a varying pace till its completion about 2 decades later. The process of “growth” is accompanied with increase in body size and/or mass at varying rates. It is multifactorial and complex, still remarkably predictable. Boys and girls grow differently and each child has his or her distinct growth pattern. Growth charts (curves) are used to measure growth. The distance growth curve (Fig. 3.2.1) is a measure of size over time; it records height, weight, and/or skull circumference as a function of age and gets higher with age. The velocity growth curve measures the rate of growth at a given time for a particular body feature (such as height or weight). The height velocity curve is highest in infancy, up to 2 years of age, with more consistent annual growth afterwards and increases again at puberty (Fig. 3.2.1). Any faltering in growth process may indicate disease. Therefore, frequent and accurate growth assessment is of primary importance.

Genetic actors F

efinition

Racial Influence Growth potential of children of different races varies despite of similar environments. Asians tend to be smaller than Europeans while Afro-Americans are taller than White Americans.

Parental Influence Tall parents tend to have taller children. The midparental height reflects the genetic potential for growth for an individual.

Gender

Genetic Disorders Certain genetic disorders can adversely influence growth. These include chromosomal disorders (e.g. Down syndrome and Turner syndrome) and genetic mutations (e.g. mucopolysaccharidosis). F

nvironmental actors

Prenatal Growth



­

The size at birth is primarily influenced by maternal health and uterine environment. Common causes of fetal growth retardation in India are maternal malnutrition and anemia. Disorders leading to placental insufficiency like preg nancy-induced hypertension, multiple pregnancies, chronic systemic disorders and maternal tobacco/alcohol abuse are other important causes of fetal growth retardation. Maternal diabetes, by stimulatory insulin production in the fetus, is an important cause of excessive intrauterine growth leading to a large for date baby.

Growth is influenced by interaction of both genetic and environmental factors. Children generally grow to their genetic height potential with little outside assistance. What parents can do to help their child’s optimal growth and development is to create the best possible environment for their growth to take place.

With same genetic potential and environment, boys tend to be taller and heavier than girls. The difference is obvious from early childhood and gets more pronounced during puberty.

E

actors nfluencing Growth I

F







t

t

Tex book of Pedia rics

3.2

Postnatal Growth

C

• Nutrition: During the first 2 years of life, undernutrition has far reaching consequences; growth deficit that occurs during this period is never fully recoverable. • hronic systemic diseases: Chronic disorders especially congenital heart disease, recurrent pneumonia, persistent diarrhea, recurrent urinary tract infection, tuberculosis, etc. lead to growth failure by causing catabolism and poor nutrition intake. This may become irreversible if there is inadequate disease free period to allow catch up growth.

 

Figure 3.2.1 Distance growth curve. Increase in height with age in boys (birth to 18 years). The broken line is velocity height curve with peak height velocity

68

vip.persianss.ir

Brain growth occurs very rapidly during fetal life and infancy. Although brain cell formation is almost complete before birth, brain maturation continues after birth. The brain of the newborn is not yet fully developed. It contains about 100 billion brain cells that have yet to be connected into functioning networks. But brain development up to age one is more rapid and extensive than was previously  

Table 3.2.1 Growth periods Embryo: mplantation to 8 weeks of gestation Fetus: 9th week of gestation to birth Infant: birth to 1 year of age Toddler: 1–3 years of age Preschool: 3–5 years of age School age: 5–12 years Adolescence: 10–19 years i

•  •  •  •  •  •  • 

t n D p

L

D

S

R R

egulation of Growth

Fetal growth is critical to a person’s eventual height. Before birth, the key measure is the crown-rump length. The fastest growth rate for a human is during embryonic life (rate being 50–60 cm/year). The growth of the embryo and fetus is mainly mediated by maternal nutrition and by growth factors, such as fibroblast and epidermal growth factors, transforming growth factors alpha and beta, insulin and insulin-like growth factors (IGF-I and IGF-II). The GH only begins to play a role in growth in the final weeks before birth. The three components of postnatal growth, that are infancy, childhood and puberty, represent different modes of growth regulation. The growth rate during infancy is rapid but sharply decelerating and is principally dependent on nutrition. The GH and thyroxine have an increasingly important role from 1 year of age. During the first 2 years, the infants establish their own growth trajectory (path); later from about 2 years of age to the onset of puberty, growth occurs in relatively constant annual increments.

linical pplications A

S

B

C

rain ( ead ircumference- rain ize) H

B

The general body growth is rapid during fetal life and first 1–2 years of age. The growth velocity slows later during mid-childhood and accelerates once again during puberty (Fig. 3.2.1). The limbs and arms grow faster than the trunk so that body proportions undergo marked variation as an infant grows into an adolescent.

It grows at different rates around 9–11 years in girls and 11–13 years in boys. The sexual development is complete by 19–20 years of age.



B

General ody

eproductive ( exual) evelopment



The various growth periods are shown in Table 3.2.1.

The growth of lymphoid tissue is the highest during midchildhood when children are often observed to have enlarged tonsils and lymph nodes, maximum being at 8–9 years of age and later decreases in size.

C

S

B

D

Growth Pattern of ifferent ody ystems

ymphoid Tissue Growth

Physical growth begins to slow at around age 1 year. As growth slows, children need fewer calories and parents may notice a decrease in appetite. Two-year-old child can have very erratic eating habits that sometimes make parents anxious. It seems as though some children eat virtually nothing yet continue to grow and thrive. Actually, they eat little 1 day and then make up for it by eating everything in sight the next day. Rapid early growth in low birth weight or healthy fullterm infants (centile crossing) is associated with later fatness, obesity, hypertension, hypercholesterolemia and 69

Pediatricians should remember that the first thing that springs to most parents’ minds when they hear the phrase “physical growth” used in the context of child development is height. Height or how tall a child grows can easily be measured. It is by no means the only facet of physical growth in children. In addition to height, the development of gross motor skills, fine motor skills and coordination are all important indicators of physical growth. • Growth is a fundamental characteristic of childhood. • Despite being influenced by many factors, it remains remarkably predictable. • Normal growth is an indicator of optimum health. • Deviation from the normal pattern is indicative of a pathological process. • Periodic assessment facilitates early detection of growth faltering, which may be the first manifestation of undernutrition/infection/disease.

nt

mportance of Growth ssessment

realized. At birth, the brain of the infant is 25% of the adult size. At the age of 1 year, the brain has grown to 75% of its adult size and to 80% by age three, reaching 90% by age seven. The influence of the early environment on brain development is crucial. Infants exposed to good nutrition, toys and playmates have better brain function at age 12 than those raised in a less stimulating environment. The rapid brain growth is reflected by an increase in head circumference.

A

I

E

H

ormonal influences: After first 6–8 months of life, the GH and thyroxine deficiency, and in addition these hormones during puberty, the sex hormones, have also an important role in induction of the pubertal growth spurt. • motional factors: Emotional deprivation, anxiety and insecurity influence the neurochemical regulation of GH and may affect a child’s growth.

Grow h a d evelo me

•

vip.persianss.ir

L

I

C

A

Growth ssessment in nfancy and hildhood

infants tend to be shorter throughout the life than infants whose size is appropriate for their gestational age.

ength on an nfantometer I

L

A

Tools for nthropometry Infant lying straight with his shoulders and buttocks flat against the measuring surface, eyes looking up, second person holds head to touch the head piece, align the body and extend both legs by one hand on knees, and bring the foot-piece firmly against the heels. Record the length to the nearest 0.1 cm (Fig. 3.2.2) (See Chapters 1.3 and 3.1). H



t

t

Tex book of Pedia rics

insulin resistance; the key risk factors for coronary vascular disease. Buyken et al. showed that breastfeeding reduces these risks. Early growth and the brain in undernutrition and/ or anemia induce structural changes. Studies show that the impact of undernutrition in brain growth induces structural changes (loss of frontal lobe asymmetry); impairs higher mental functions; and there is persistence of soft neurological signs. Maternal anemia (iron-deficiency) affects development of neurotransmitters irreversibly. Puberty is fueled by the secretion of GH and sex steroids. Puberty is the process of physical maturation from child to adult. The timing of this growth spurt is extremely variable. At puberty, a second growth spurt occurs, being earlier in girls by 1 1/3–2 years than in boys, giving rise on the average, to a difference in adult height between men and women of about 14–15 cm.

eight

It is measured standing for a child with minimum clothing without shoes and socks, standing with feet parallel on an even platform, stretching fullest, arms hanging on the sides, and buttocks and heels touching the rod. The head is held erect with lower border of the eye orbit in the same horizontal plane as the external canal of the ear (Frankfort plane). The head piece is lowered to touch the top of the head (Fig. 3.2.3).

ength

eight

It is measured on an anthropometric rod or a stadiometer, if the child can stand (> 2 years of age). In general, length in normal term infants increases about 30% by 5 months of age and more than 50% by 12 months of age. Infants grow 25 cm during the first year; and height at 4 years is about double of the birth length. In boys, half the adult height is attained around 2 years of age; while in girls, height at 19 months is about half the adult height. Some small-for-gestational-age

Figure 3.2.3 Stadiometer for height measure: note the position  

70

Figure 3.2.2 Infantometer for length: help of two measurers is required  

H

It is measured on an infantometer in children too young to stand until 2 years of age.

vip.persianss.ir

Anterior Fontanel

t

­

­

n

­

D p

C

­

nt

This is measured on the left upper arm mid-way between the acromion and olecranon processes. It measures 9.8 cm at birth, 14.5 cm around 1 year with slow increase from 14.8 cm to 16.2 cm between 1 year and 5 years of age. In field surveys, it helps in diagnosis of mal/under nutrition: a value more than 13.5 cm is taken as normal nourished, 12.5–13.4 cm as borderline, 11.5–12.4 cm as mild to moderate undernutrition and less than 11.5 cm as severe undernutrition. C

Anterior fontanel (AF) is a diamond-shaped open gap situated in midline at the junction of the coronal and sagittal

idarm ircumference

hest ircumference C



This is measured over the most prominent part of the occipital and just above the supraorbital ridges, using a flexible, nonstretchable tape. Position the tape just above the eyebrows, above the ears and around the biggest part on the back of the head. Pull the tape snugly to compress the hair and take reading nearest to 0.1 cm (Fig. 3.2.6). The head circumference measurement in infancy serves as a guide to brain growth; it is related to intelligence and cognition. Large infants have larger head circumference.

Posterior Fontanel (PF), placed between the intersection of the occipital and parietal bones, closes within 6–8 weeks of life. An open PF in later life is noted in congenital hypothyroidism. M

C

ead ircumference

Posterior Fontanel

A

At birth, chest circumference is 3 cm less than the head circumference. It equals or exceeds head circumference by 10–12 months of age. In prepubertal children, it can be recorded at the level of nipples.

rm pan S

W H

Infant/child should be naked or in minimal clothing. Ideal is to use sliding beam balance scale or electronic scale (Figs 3.2.4 and 3.2.5). Weighing scale is checked for zero, center the infant on the scale tray and weigh to the nearest 10 g; older child is weighed standing to the nearest 50 g.

sutures (size around 2 × 2 cm) that close by 18 months (range 10–18 months). Small or closed AF is a warning sign, i.e. microcephaly. Large AF may be present in hydrocephalus, hypothyroidism, Down syndrome, achondroplasia, osteo genesis imperfecta and mucopolysaccharidosis.

Grow h a d evelo me

eight

Measure outstretched arms from fingertip to fingertip. In children of European origin, the arm span should approximate the height (intermediate-length arms). Asians have proportionally shorter arms than Europeans, and Africans have significantly longer arms.  

Figure 3.2.4 Electronic weighing scale

Figure 3.2.6 Crossover technique to measure head circumference by fiber glass tape  

 

Figure 3.2.5 Seca scale to measure height and weight

71

vip.persianss.ir

Waist-to-hip ratio (WHR) is measured over the highest palpable points of the iliac crests in the midaxillary lines, by a fiber glass tape. Hip is measured on the maximum extension of the buttocks. • Waist-to-hip ratio index of 0.7 for women and 0.9 for men have been shown to correlate strongly with general health and fertility • Women within the 0.7 range have optimal levels of estrogens and are less susceptible to major diseases such as diabetes, cardiovascular disorders and ovarian cancers • Men with WHR around 0.9, similarly, have been shown to be more healthy and fertile with less prevalence of prostate and testicular cancers.

R

H

W

C

Percentiles describe the frequency distribution of anthropometric parameters like weight, height, skull circumference, BMI, etc. Fiftieth percentile is the average (median) line for the given population. It describes the percent of children expected to be on or below that line, e.g. 50th centile means that 49% of the observations are below and 50% above that observation. A child’s growth parameters may be on the centile line or between two centile lines. Conventionally, for all parameters, 3rd and 97th percentiles are the lowest, and highest 94% of observations. Any child with parameters below or above these limits or those who cross percentiles after 2 years of age needs careful evaluation.

Examples •

If height and weight consistently are on the 60th percentile line until a child is 5-year-old, then the height has dropped to the 30th percentile at age 6, that might indicate that there is a growth problem (catch down-retardation of growth) because the child is not following his or her previous growth pattern. This indicates disease. • Boy with height in 40th percentile and weight in the 85th percentile (he is taller than 40% of the kids of his age, but weighs more than 85% of kids his age). There might be a health problem (overweight/obesity). On the other hand, if he is in the 85th percentile for height and weight, and follows that pattern consistently over time, that usually means that he is a normal child just larger than average.

Z Scores It is calculated as below: Z score =

Observed value − median reference

SD of reference population A value of –2 Z score corresponds to 3rd percentile. Z scores are labeled as 1, 2, 3, –1, –2, and –3. These indicate

R

aist-to- ip atio H

W

S

S

B

Around 50% of body fat is located under the skin. Measurement of triceps and biceps skinfold thickness (SFT) gives estimate of peripheral fat, and subscapular and suprailiac SFT indicates amount of central fat. The Lange’s or Harpenden’s skinfold calipers are used. Measurements are done as follows: • iceps: At the midpoint of their muscle belly, a point generally opposite the nipple • Triceps: Between the tip of olecranon process of ulna (elbow) and the acromion process of the scapula (shoulder), a point is marked on the back of the arm • ubscapular: Below inferior angle of scapula 45° to vertical • uprailiac: Above iliac crest in midaxillary line (approximately 2.5 cm above hip bone).

Percentiles



kinfold Thickness

Growth charts consist of a series of percentile curves. It means graphical representation of growth reference standards and consists of a series of percentile curves that illustrate the distribution of body measurements in the study population (Figs 3.2.7 to 3.2.12).



S

Body mass index (BMI) is the ratio of weight in kilogram to the square of height in meters (wt/ht2). This is a good indicator of variability of energy status. If more than 95th centile, it suggests obesity and less than 5th centile, undernutrition (thin). In adolescents, calculate with the weight and height values in relation to the sexual maturity. The BMI values for Indian children are different than the National Center for Health Statistics (NCHS)—BMI values.

Growth harts



ass ndex I

ody

M

Subtract the LS from the height. The US/LS ratio is calculated by dividing the US by the LS. In children of European origin, this ratio is about 1.7 at birth and decreases to 1 at about age 10, where it remains throughout adulthood. Asians have proportionally shorter legs (therefore, larger US/LS ratio) and Africans have longer legs (therefore lower US/LS ratio).



Upper Segment

Values less than 0.5 exclude central obesity but values more than 0.5 indicate central obesity even in children with normal weight and height. Waist-to-height ratio may predict cardio-metabolic risk in normal weight as well as in overweight/obese children, according to results from the Bogalusa Heart Study, 2011.



Measure from the symphysis pubis to the floor.

aist-to- eight atio



S

L

Lower Segment

B

t

t

Tex book of Pedia rics

Upper and ower egments

72

vip.persianss.ir

n

t



nt

p

Table 3.2.2 provides the summary of the details of physical growth in Indian children related to weight and height gain. Table 3.2.3 provides a summary of the changes in head circumference at different ages in children.

D

C

I

C

C

hoice of Growth urve for ndian hildren

The data on affluent Indian children were collected during 1989–1991 by the Nutrition Foundation of India from birth to 5 years (seven states); only full term with birth weight more than or equal to 2500 g (boys 433 and girls 346) were followed during first year of life at 3, 6, 9 and 12 months of age with minimum of three readings for every infant (cohort I). In cohort II, from 12 months to 5 years of age, 1011 boys and 874 girls were followed on their birthday and 6 monthly with minimum of three measurements for

Grow h a d evelo me

how far points are above or below the mean (Z score 0). A range of ±2 Z scores includes 95.4% of all observations and is the conventionally accepted limits of normality.

73

 

 

Figure 3.2.7 The growth curves (data) for height, weight and skull circumference for girls from birth to 2 years Source: Agarwal et al. Indian Pediatrics; 1994

vip.persianss.ir

Tex book of Pedia rics

Table 3.2.3 Summary of changes in head circumference

each child up to 72 months of age. Children had received exclusive breast milk for 3–4 months of life in cohort I and II (as prevalent in those years). The Indian Council of Medical Research (ICMR) crosssectional data for physical growth and sexual development for 5 years to 17 years in girls and from 5 years to 18 years in boys (9 states, 23 schools, 12893 boys and 10,941 girls), on affluent Indian children were collected during 1989–1991. These two data sets were collected around same time on affluent Indian children – “birth to adolescence.” These data sets on physical growth and sexual development (birth to 18 years of age) continue to serve as the baseline reference data for assessing physical growth and sexual development; assessed by the same measurers at all centers for seeing secular trend in height and percentile of children becoming overweight and obese. These “growth charts” (Figs 3.2.7 to 3.2.12; Tables 3.2.4 to 3.2.13) give height, weight, skull circumference and BMI for age and 74 gender.

 

B

At birth irth to 3 months

35 cm 2 cm/month

3–6 months

1 cm/month

6–9 months

0.5 cm/month

9–12 months

0.25 cm/month

On first birthday

46–47 cm, 35% increase from birth size

At 2 years age

48 cm

At 5 years

50–51 cm

12 years

52 cm

B







t

Anthropometric measures of normal full term newborns: •  Birth weight: 2.5–4.0 kg •  Length: 50 cm (around) •  Head circumference: 34–35 cm Weight gain: Neonates generally lose 5–8% (maximum being 10%) weight during first 2–3 days of life, which is regained by the 10th day. Average daily weight gain during: •  First 3 months: 30 g •  3–6 months: 20 g (birth weight doubles by 5–6 months of age) •  6–9 months: 15 g •  9–12 months: 12 g (birth weight triples by first birthday) •  1–3 years: 8 g (around 3 kg/year). irth weight quadruples by 2 years of age. •  4–6 years: 6 g (around 2 kg/year); this rate of gain continues till the onset of puberty Length/height gain (height velocity): •  Birth to 3 months: 3.5 cm/month •  3–6 months: 2.0 cm/month •  6–9 months: 1.5 cm/month •  9–12 months: 1.2 cm/month •  1–3 years: 1.0 cm/month •  4–6 years: 5 cm/year (at 4 years = 100 cm; double of birth length) Gains in length •  During first year of life: 25 cm •  During second year of life: 12.5 cm •  During third year of life: 7.5–10 cm •  7 cm/year at 3–4 years •  6 cm/year at 5–6 years •  5 cm/year till puberty •  In immediate prepubertal period, growth velocity slows down before the pubertal spurt begins (adrenarche) Abnormal growth: •  Less than 7 cm/year for less than 4 years of age •  Less than 6 cm/year for 4–6 years •  Less than 4.5 cm/year for 6 years–onset of puberty

WHO Multicenter Growth Reference Study The WHO growth data, in India were collected from south Delhi area and pooled in the international data. The WHO Multicenter Growth Reference Study (MGRS) was undertaken between 1997 and 2003 to generate new growth curves for assessing the growth of infants and young children around the world. The MGRS collected primary growth data and related information from approximately 8500 children from widely different ethnic backgrounds and cultural settings (Brazil, Ghana, India, Norway, Oman and the USA). The new growth curves are expected to provide a single international standard that represents the best description of physiological growth for all children from birth to 5 years of age and to establish the breastfed infant as the normative model for growth and development. These growth charts depart from the growth reference model in several ways. Children from six countries provided the data measurements, which were not representative of their country of residence, and were selected on the basis of sociodemographic criteria and child’s nutrition as per WHO guidelines (see Chapter 3.3 for more details).

Comparing Agarwal and Khadilkar Data Sets Comparing Agarwal et al. school data (ICMR) against recently available Khadilkar et al. data set, the changes in height vary from 1% to 2.5% among boys and 1% to 3% in girls. The median final height in boys is higher by 0.7 cm but the 97th percentile is higher by 1.7 cm whereas in girls the median is same but 97th percentile is higher by 2.4 cm. The changes in weight vary from 20% to 29% among boys and 18% to 25% among girls. Therefore, Khadilkar et al. data showed marginal secular trend for height with marked increase in obesity which is in line with the data from the Western countries such as United Kingdom. Their observations are similar to those recently published by Marwaha et al. Khadilkar and Marwaha data sets of school children have important health implications, alarming increase in obesity, which needs immediate action to control or prevent and create nationwide awareness. Redoing the curves in response to increasing weight alone will have the effect of “normalizing” the weight

t

 

Table 3.2.2 Growth pattern in infancy and early childhood: summary of changes in weight and height

vip.persianss.ir

nt

p

D

n

t

Grow h a d evelo me

 

 

Figure 3.2.8 The growth curves (data) for height, weight and skull circumference for girls from birth to 2 years Source: Agarwal et al. Indian Pediatrics; 1994

75

vip.persianss.ir

t

t

Tex book of Pedia rics

 

Figure 3.2.9 The growth curves (data) for height and weight for girls from 2 years to 17 years Source: Agarwal et al. Indian Pediatrics; 1992, 1994 and 2001  

76

vip.persianss.ir

nt

p

D

n

t

Grow h a d evelo me

V d

ti e

n U

G R

 

 

Figure 3.2.10 The growth curves (data) for height and weight for boys from 2 years to 17 years Source: Agarwal et al. Indian Pediatrics; 1992, 1994 and 2001

77

vip.persianss.ir

t

t

Tex book of Pedia rics

V d

ti e

n U

G R

 

 

Figure 3.2.11 The growth curves (data) for body mass index for girls from birth to 17 years Source: Agarwal et al. Indian Pediatrics; 1992, 1994 and 2001

78

vip.persianss.ir

nt

p

D

n

t

Grow h a d evelo me

V d

ti e

n U

G R

 

 

Figure 3.2.12 The growth curves (data) for body mass index for boys from birth to 17 years Source: Agarwal et al. Indian Pediatrics; 1992, 1994 and 2001

79

vip.persianss.ir

t

t

 

Tex book of Pedia rics

Table 3.2.4 Weight (kg) percentiles for girls (birth–6 years of age): affluent Indians Age (months)

Percentiles 3rd

5th

10th

25th

50th

75th

90th

95th

97th

0

2.6

2.6

2.7

2.9

3.1

3.4

3.7

3.8

3.9

3

4.4

4.5

4.8

5.2

5.6

6.0

6.4

6.6

6.7

6

5.6

5.7

5.9

6.4

6.9

7.4

7.9

8.2

8.4

9

6.9

7.0

7.3

7.5

8.0

8.5

8.9

9.2

9.5

12

7.8

8.0

8.2

8.6

9.1

9.7

10.0

10.4

10.6

18

8.7

9.0

9.5

10.0

10.5

11.4

11.8

12.1

12.4

24

9.4

9.6

10.0

10.9

11.6

12.2

13.0

13.5

13.9

30

9.9

10.2

10.6

11.7

12.5

13.0

13.9

14.4

14.8

36

10.5

10.8

11.4

12.4

13.4

14.2

15.0

15.7

16.4

42

11.4

11.8

12.1

13.2

14.2

15.2

16.5

17.2

17.8

48

11.9

12.2

12.9

13.9

15.0

16.5

17.5

18.2

18.9

54

12.9

13.3

14.0

14.8

16.0

17.5

18.7

19.7

20.0

60

13.4

13.9

14.5

15.4

17.0

18.4

19.9

20.0

21.0

66

14.2

14.9

15.6

16.2

17.9

19.2

20.1

21.0

21.4

V d

G R

Table 3.2.5 Height/length (cm) percentiles for girls (birth–66 month of age): affluent Indians  

ti e

Age (months)

Percentiles

3rd

5th

10th

25th

50th

75th

90th

95th

97th

0

47.5

48.0

48.3

49.1

50.0

51.0

51.8

52.3

52.7

3

55.3

56.4

57.5

58.5

59.3

60.9

62.0

63.1

63.8

6

61.0

61.7

62.5

63.7

65.2

66.6

68.3

69.0

69.8

9

65.3

66.2

67.3

68.5

69.6

70.9

72.4

73.1

73.9

12

70.2

70.9

72.0

73.9

74.0

75.4

76.5

77.1

77.8

18

73.5

74.8

75.9

78.5

80.0

81.5

83.4

84.7

85.0

24

77.7

79.0

80.6

82.7

84.0

86.2

88.1

89.4

89.8

30

82.0

82.7

84.7

86.9

89.3

91.3

93.0

94.2

95.0

36

85.3

86.1

87.9

90.7

92.9

95.4

97.5

99.1

99.9

42

88.1

89.9

91.0

93.0

96.7

99.0

101.4

102.7

104.5

48

91.2

92.9

94.9

97.4

99.9

102.4

104.8

106.9

107.9

54

94.5

95.8

97.3

100.5

103.0

105.5

107.0

109.0

110.0

60

96.9

98.4

100.1

103.1

106.0

109.1

111.1

112.5

113.7

66

100.8

102.0

103.6

106.3

109.4

112.2

114.7

116.0

117.0

n U

80

vip.persianss.ir

Grow h a d evelo me

 

Table 3.2.6 Weight (kg) percentiles for boys (birth–66 months of age): affluent Indians

25th

50th

75th

90th

95th

97th

0

2.6

2.6

2.7

3.0

3.1

3.5

3.6

3.8

3.9

3

4.7

4.8

5.0

5.4

5.8

6.2

6.7

6.9

7.0

6

6.0

6.1

6.2

6.5

7.1

7.7

8.2

8.5

8.7

9

7.1

7.3

7.5

8.0

8.4

8.8

9.4

9.8

10.0

12

8.2

8.4

8.7

9.1

9.6

10.0

10.4

10.8

11.1

18

8.7

9.0

9.8

10.1

10.9

11.4

12.0

12.5

12.8

24

9.7

10.0

10.4

11.1

11.9

12.6

13.4

14.1

14.9

30

10.7

11.0

11.4

12.1

12.9

13.7

14.7

15.4

15.9

36

11.4

11.5

12.0

12.8

13.7

14.6

15.9

16.5

17.0

42

11.8

12.2

12.7

13.6

14.5

15.5

16.9

17.8

18.5

48

12.4

12.8

13.4

14.4

15.4

16.4

17.7

18.8

19.6

54

13.1

13.7

14.1

15.0

16.2

17.6

19.0

20.0

20.1

60

13.8

14.5

15.0

16.1

17.2

18.5

20.0

20.5

21.8

66

14.4

15.2

16.0

17.1

18.1

19.7

20.9

21.0

22.0

G R

58.7

V d 60.1

61.5

62.7

63.4

64.3

64.0

65.5

67.0

68.9

70.5

71.8

69.3

70.3

71.6

73.0

73.8

74.2

73.7

74.7

75.9

76.9

78.0

78.6

79.1

80.9

82.0

83.3

84.6

85.2

81.4

83.4

85.7

87.1

88.7

90.1

90.5

85.8

88.1

90.4

92.3

94.0

95.3

95.9

89.9

92.4

94.2

96.4

98.5

99.9

100.7

92.4

95.1

97.9

99.9

102.7

104.1

105.5

94.1

95.4

97.9

100.7

103.3

106.2

108.4

109.4

96.3

98.1

100.6

103.7

106.9

109.9

112.0

113.0

97.9

99.3

101.4

103.9

106.9

109.9

113.4

114.9

116.2

100.8

102.0

104.3

107.6

110.0

113.2

116.8

118.0

119.4

n

10th

D

5th

p

3rd

t

Percentiles

nt

Age (months)

 

Table 3.2.7 Height/length (cm) percentiles for boys (birth– 66 months of age): affluent Indians

ti e

Age (months) 3rd

5th

10th

0

47.6

47.8

48.3

3

56.3

56.7

6

62.0

62.3

9

67.1

67.4

12

71.7

72.2

18

75.3

76.0

24

78.9

79.9

30

84.7

85.0

36

87.7

88.6

42

89.8

91.1

48

92.5

54

95.6

60 66

n U 57.3 63.0 68.1 72.7 76.9

25th 49.1

Percentiles

50th

75th

90th

95th

97th

50.1

50.8

51.9

52.4

52.9

81

vip.persianss.ir

Tex book of Pedia rics

 

Table 3.2.8 Head circumference (cm) percentiles for girls (birth–36 months): affluent Indians

t

t

Age (months)

Percentiles N

3rd

5th

10th

25th

50th

75th

90th

95th

97th

0

269

33.1

33.2

33.6

34.1

34.5

35.2

35.5

36.0

36.4

3

299

37.4

37.3

38.2

39.0

39.6

40.3

41.0

41.3

41.4

6

308

39.7

40.1

40.3

41.8

42.4

42.9

43.4

43.8

44.0

9

302

42.2

42.5

42.8

43.4

43.9

44.5

44.9

45.3

45.5

12

290

43.1

43.5

44.1

44.6

45.1

45.7

46.1

46.4

46.5

18

135

43.9

44.4

44.9

45.7

46.4

47.0

47.8

48.0

48.0

24

179

44.7

45.0

45.5

46.2

47.0

47.7

48.3

48.7

48.9

30

206

44.9

45.4

45.8

46.7

47.8

48.4

49.1

49.7

50.0

36

266

45.3

45.7

46.0

47.0

48.0

49.0

49.9

50.4

50.9

G R

 

Table 3.2.9 Head circumference (cm) percentiles for boys (birth–36 months): affluent Indians Age (months)

Percentiles

46.9

V d 47.7

48.5

49.0

49.5

49.9

47.4

48.2

48.8

49.6

49.9

50.4

47.9

48.7

49.5

50.0

50.9

51.0

3rd

5th

10th

25th

0

33.2

33.5

33.8

34.2

3

38.1

38.2

38.6

39.3

6

40.3

40.7

41.3

9

42.5

42.9

43.2

12

43.7

44.0

44.4

18

44.8

44.9

45.4

24

44.5

45.8

30

46.2

46.4

36

46.1

46.7

ti e

n U 46.3 46.8 47.0

42.1 43.7 44.9 46.1



gains you are seeing at the higher end of the scale since the definition of “normal” weight will shift to the right, potentially putting more children who today are classified as overweight into the normal weight category. At the bottom of the scale, some children who are today classified as normal weight will be classified as underweight. On both the high and low ends of the scale, creating new reference curves with these changes may not be beneficial from a public health perspective. Secondly, Khadilkar et al. data line for 3rd centile flattens after 14 tears if age (sjewubg as cinoared ti Agarwak et al. and Marwaha et al. Thirdly, it is important to take lesson from the “methods including the data source in construction of the Centers for Disease Control and Prevention (CDC) 2000 growth charts”, as weight data from the National Health and Nutrition Examination 82 Survey (NHANES) III (1988–1994) were excluded from the weight for age and BMI-for-age curves because of a secular

50th

75th

90th

95th

97th

34.7

35.3

35.9

36.3

36.5

40.0

40.7

41.4

42.0

42.5

42.7

43.3

43.8

44.2

44.7

44.2

44.8

45.3

45.8

46.2

45.4

45.9

46.5

46.9

47.3

47.0

48.0

48.4

48.7

48.9

trend in body weight that occurred between NHANES II (1976–1980) and NHANES III.

Recommendations Presently, the choice remains using IAP recommended 2007 growth charts based on data (birth to 18 years of age) by Agarwal et al. This was also recommended in growth chart evaluation study by Khadgawat et al. The WHO curves may be used up to 2 years of age to assess growth as practiced in USA. It is important to note that in spite of unprecedented economic growth since 1991, Indian women remain short by 5.5 cm as compared to the average height, in 54 developing countries. The Indian affluent children at 18 years of age are still shorter than the NCHS/WHO data. Thus, we must examine our children in nationally collected growth data sets, as calculated BMI values are also different as compared to the WHO/NCHS values.

vip.persianss.ir

Grow h a d evelo me

 

Table 3.2.10 Weight (kg) percentiles for girls (6–17 years): affluent Indians 97th 23.7 25.4 27.5 29.8 32.3 34.9 37.7 40.5 43.4 46.4 49.3 52.2 55.1 57.9 60.7 63.2 65.7 67.9 70.0 71.8 73.3 74.6 75.6

75th 115.9 118.4 121.3 124.4 127.5 130.7 133.8 136.9 139.8 142.7 145.4 147.9 150.3 152.5 154.4 156.2 157.6 158.8 159.7 160.4 160.4 160.5 160.5

97th 123.3 126.0 129.3 132.8 136.4 139.8 143.1 146.2 149.0 151.7 154.2 156.5 158.5 160.4 162.1 163.5 164.7 165.8 166.5 167.1 167.4 167.6 168.0

G R

V d

ti e

75th 19.2 19.9 20.9 22.2 23.6 25.3 27.2 29.3 31.4 33.7 36.0 38.4 40.7 42.9 45.1 47.1 48.9 50.5 51.8 52.9 53.6 54.0 53.9

n

50th 17.8 18.3 19.0 19.9 20.8 22.0 23.5 25.1 26.9 28.9 30.9 32.9 35.0 37.1 39.1 41.0 42.7 44.3 45.7 46.8 47.7 48.2 48.4

D

25th 16.4 16.9 17.3 18.0 18.7 19.6 20.7 22.1 23.6 25.3 27.1 28.9 30.8 32.6 34.5 36.2 37.8 39.3 40.6 41.7 42.5 43.0 43.3

p

6.0 6.5 7.0 7.5 8.0 8.5 9.0 9.5 10.0 10.5 11.0 11.5 12.0 12.5 13.0 13.5 14.0 14.5 15.0 15.5 16.0 16.5 17.0

5th 15.2 15.5 15.8 16.2 16.4 16.8 17.6 18.5 19.7 21.0 22.4 24.0 25.6 27.2 28.9 30.6 32.1 33.6 35.0 36.2 37.3 38.1 38.7

t

Percentiles 3rd 14.1 14.4 14.8 15.3 15.9 16.4 17.1 18.3 19.5 20.9 22.3 23.7 25.1 26.5 27.9 29.3 30.7 32.1 33.4 34.6 35.7 36.7 37.6

nt

Age (years)

 

Table 3.2.11 Height (cm) percentiles for girls (6– 17 years): affluent Indians Age (years) 6.0 6.5 7.0 7.5 8.0 8.5 9.0 9.5 10.0 10.5 11.0 11.5 12.0 12.5 13.0 13.5 14.0 14.5 15.0 15.5 16.0 16.5 17.0

3rd 102.1 104.5 107.1 109.7 112.3 115.0 117.8 120.6 123.4 126.1 128.8 131.4 133.9 136.3 138.5 140.6 142.4 144.1 145.5 146.6 147.5 148.0 148.3

n U 5th 104.5 107.0 109.4 111.6 113.9 116.2 118.8 121.4 124.1 126.9 129.7 132.4 135.0 137.5 139.8 141.9 143.8 145.4 146.6 147.5 148.0 148.1 148.5

25th 108.8 111.1 113.7 116.4 119.3 122.2 125.1 128.0 130.8 133.7 136.4 139.0 141.5 143.8 145.9 147.8 149.4 150.8 151.8 152.6 152.9 152.9 153.0

Percentiles

50th 112.5 114.9 117.4 120.3 123.2 126.2 129.2 132.3 135.2 138.1 140.9 143.5 146.0 148.3 150.4 152.2 153.8 155.1 156.0 156.6 156.8 156.5 157.0

83

vip.persianss.ir

t

t

 

Tex book of Pedia rics

Table 3.2.12 Weight (kg) percentiles for boys (6–18 years): affluent Indians Age (years)

Percentiles 3rd 15.2 15.7 16.2 16.8 17.5 18.2 19.2 19.9 20.9 21.9 22.9 24.1 25.3 26.7 28.1 29.6 31.2 32.9 34.6 36.5 38.5 40.6 42.8 45.2 47.6

6.0 6.5 7.0 7.5 8.0 8.5 9.0 9.5 10.0 10.5 11.0 11.5 12.0 12.5 13.0 13.5 14.0 14.5 15.0 15.5 16.0 16.5 17.0 17.5 18.0

5th 15.7 16.4 16.9 17.5 18.0 18.6 19.4 20.2 21.2 22.3 23.5 24.9 26.3 27.8 29.3 31.0 32.7 34.5 36.3 38.1 40.0 41.9 43.9 45.8 47.8

25th 18.0 18.6 19.4 20.0 20.7 21.3 22.0 22.9 24.1 25.5 27.1 28.9 30.7 32.7 34.7 36.8 38.8 40.9 42.8 44.7 46.5 48.1 50.0 52.0 54.0

50th 19.0 20.0 21.0 22.0 22.6 23.5 24.4 25.6 27.0 28.7 30.6 32.7 34.8 37.1 39.4 41.8 44.1 46.3 48.5 50.5 52.4 54.0 55.5 57.2 58.6

97th 25.4 27.7 29.7 31.6 33.5 35.5 37.7 40.1 42.7 45.4 48.2 51.1 54.1 57.1 60.0 63.0 65.9 68.7 71.4 73.9 76.3 78.5 80.5 82.2 83.6

75th 118.0 120.7 123.0 125.2 127.4 129.8 132.5 135.3 138.3 141.5 144.7 147.9 151.1 154.2 157.3 160.2 162.9 164.4 167.7 169.6 171.2 172.4 173.1 173.4 173.4

97th 125.9 128.4 130.8 133.2 135.8 138.5 141.4 144.5 147.7 151.0 154.3 157.5 160.8 163.9 166.9 169.7 172.7 174.7 176.8 178.5 179.8 180.7 181.2 181.1 181.6

G R

V d

ti e

75th 20.7 21.9 22.9 23.9 25.0 26.3 27.7 29.4 31.3 33.4 35.6 37.9 40.3 42.7 45.1 47.6 50.0 52.4 54.6 56.8 58.8 60.6 62.3 63.7 64.9

 

Table 3.2.13 Height (cm) percentiles for boys (6–18 years): affluent Indians Age (years)

84

6.0 6.5 7.0 7.5 8.0 8.5 9.0 9.5 10.0 10.5 11.0 11.5 12.0 12.5 13.0 13.5 14.0 14.5 15.0 15.5 16.0 16.5 17.0 17.5 18.0

3rd 103.7 106.1 108.5 110.9 113.3 115.2 118.0 120.3 122.7 125.1 127.5 129.9 132.4 134.9 137.4 140.0 142.6 145.2 148.0 150.8 153.6 156.6 159.6 162.7 161.0

n U 5th 105.5 107.5 109.8 111.3 114.4 116.2 118.5 120.9 123.4 125.9 128.5 131.1 133.8 136.5 139.2 141.8 144.5 147.2 149.8 152.4 154.9 157.4 159.8 163.1 163.5

25th 112.0 113.5 115.9 117.8 119.7 121.9 124.2 126.7 129.4 132.4 135.6 138.0 141.0 143.9 146.8 149.7 152.4 155.0 157.4 159.6 161.6 163.3 165.0 167.5 168.8

Percentiles 50th 114.2 117.3 119.7 121.6 123.6 125.7 128.2 130.8 133.6 136.6 139.6 142.7 145.8 148.9 152.0 154.9 157.6 160.2 162.5 164.6 166.3 167.7 168.7 169.3 169.8

vip.persianss.ir

t n D p nt





















V d

ti e

n U

G R

















B

1. Agarwal DK, Agarwal KN. Physical growth in affluent Indian children (birth- 6 years). Indian Pediatr. 1994;31:377- 413. 2. Agarwal KN, Agarwal DK, Benkappa DG, et al. Growth performance of affluent Indian children (Under-Fives). Nutrition Foundation of India; 1991. 3. Agarwal KN, Agarwal DK, Seth A. The growth infancy to adolescence, 2nd edition. New Delhi: CBS Publishers; 2007. pp. 1-34 and 87-93. 4. Agarwal KN, Agarwal DK, Upadhaya SK, et al. Physical and sexual growth pattern of affluent Indian children from 5-18 years of age. Indian Pediatr. 1992;92:1203-82. 5. Agarwal KN, Saxena A, Bansal AK, et al. Physical growth assessment in adolescence. Indian Pediatr. 2001;38:1217-35. 6. Buyken A. Karaolis-Danckert N, Renner T, et al. Effects of breastfeeding on trajectories of body fat and BMI throughout childhood. Obesity (Silver Spring). 2008;16:389-95. 7. Greulich WW, Pyle SI. Radiographic Atlas of Skeletal Development of the Hand and Wrist, 2nd edition. Stanford, CA: Stanford University Press; 1959.

8. Gupta P. Anthropometry: Assessment of Growth. In: Gupta P (Ed). Clinical Methods in Pediatrics. 2nd edition. New Delhi: CBS Publishers; 2011. pp. 58-110. 9. Khadgawat R, Dabadghao P, Mehrotra RN, et al. Growth charts for evaluation of Indian children. Indian Pediatr. 1998;35:859-65. 10. Khadilkar VV, Khadilkar AV, Cole TJ, et al. Cross sectional growth curves for height, weight, and body mass index for affluent Indian children, 2007. Indian Pediatr. 2009;46:47785. 11. Kuczmarsi RJ, Ogden CL, Guo SS, et al. 2000 CDC Growth charts for the United States: methods and development. Vital Health Stat 11. 2002;246:1-190. 12. Marwaha RK, Tandon N, Singh Y, et al. A study of growth parameters and prevention of overweight and obesity in school children of Delhi. Indian Pediatr. 2006;43:943-52. 13. Marwaha RK, Tandon N, Ganie MA, et al. Nationwide reference data for height, weight and body mass index of Indian School children. Natl Med J India. 2011;24:269-77. 14. WHO Multicenteric Growth Reference Study Group: WHO Child Growth Standards based on length/height, and weight and age. Acta Pediatr Suppl. 2006;450:76-85.

Grow h a d evelo me

ibliography

85

vip.persianss.ir

S





Who Under-5 Growth tandards 2006 Vaman Khadilkar

C

Why New Growth harts? The linear growth patterns of these highly selected, healthy infants were strikingly similar between countries, supporting the view that they represent a standard against which the growth of all children can be assessed, wherever they live and however they are fed. Government of India has given a directive to use WHO growth charts for all children under the age of 5 years and IAP has accepted these standards for children under 5-year-old. C



O

C

C

I

nterpretation of Who harts and ut ffs

Doctors and health care workers find it difficult to interpret various cut offs for diagnosis of underweight, overweight, stunting, wasting, etc. These have therefore been clearly spelt out in the new WHO Multicenter Growth Reference Study and are given in Tables 3.3.1 and 3.3.2. These make it easy for the practicing pediatrician and health care worker to follow as a guideline for management and referral (Tables 3.3.3 to 3.3.8; Figs 3.3.1 to 3.3.5). I

pplicability of Who harts in ndia and round the World C

A



The list of charts available is as follows: • Length/height-for-age • Weight-for-age • Weight-for-length • Weight-for-height • Body mass index-for-age • Head circumference-for-age • Arm circumference-for-age • Subscapular skinfold-for-age • Triceps skinfold-for-age • Motor development milestones WHO growth charts can be downloaded from the site http://www.who.int/childgrowth/standards/en/

V d

ti e

n U

G R

Where to Get Who Growth harts?

A

Growth charts are an essential component of the pediatric toolkit. Their value resides in helping to determine the degree to which physiological needs for growth and development are met during the important childhood period. Beyond their usefulness in assessing children’s nutritional status, many government and United Nation agencies rely on growth charts to measure the general wellbeing of populations, formulate health and related policies, and plan interventions and monitor their effectiveness. The relative magnitude of different measures of nutritional status is affected by the choice of reference charts used. In April 2006, WHO released child growth standards for children up to the age of 5 years, to provide a multiethnic benchmark for breastfed children. The standards are derived from children who were raised in environments that minimized constraints to growth such as poor diets and infection. In addition, their mothers followed healthy practices such as breastfeeding and not smoking during and after pregnancy. Because WHO standards depict physiological human growth under optimal environmental conditions, they provide an improved tool for assessing growth. These charts thus are prescriptive standards and not descriptive references. These standards provide an opportunity to redefine and revitalize actions to promote optimal child growth, foster the adoption of “best practices,” such as incorporating height and BMI to assess the dual burden of under and overnutrition (stunting and overweight), and provide coherence between national and international infant feeding guidelines that recommend breastfeeding as the optimal source of nutrition during infancy. Thus these charts are recommended for assessing the pattern of infant growth and harmonize growth assessment systems within and between the countries. A second feature of the study that makes it attractive as a basis for an internationally applicable standard is that it included children from a diverse set of countries: Brazil, Ghana, India, Norway, Oman and the USA. By selecting privileged, healthy populations, the study reduced the impact of environmental variation. Another key characteristic of the new standards is that they explicitly identify breastfeeding as the biological norm and establish the breastfed child as the normative model for growth and development. The method used to construct the WHO standards generally relied on the Box-Cox power exponential distribution and the final selected models simplified to the LMS model.

t

t

Tex book of Pedia rics

3.3

Different countries have adopted different policies on the use and acceptability of WHO growth charts. In USA, CDC recommends use of WHO charts up to the age of 2 years and CDC charts from 2 years to 18 years. In United Kingdom,

86

vip.persianss.ir

wt

Gro nt

p

D

n

h a d evelo me

V d

ti e

n U

G R

 

Figure 3.3.1 WHO Multicenter Growth Reference Study Group. WHO child growth standards based on length/height, weight and age. Acta Paediatr. 2006;95(suppl 450):76-85

87

vip.persianss.ir

t

t

Tex book of Pedia rics

 

Figure 3.3.2 WHO Multicenter Growth Reference Study Group. WHO child growth standards based on length/height, weight and age. Acta Paediatr. 2006;95(suppl 450):76-85

88

vip.persianss.ir

wt

Gro nt

p

D

n

h a d evelo me

 

Figure 3.3.3 WHO Multicenter Growth Reference Study Group. WHO child growth standards based on length/height, weight and age. Acta Paediatr. 2006;95(suppl 450):76-85

89

vip.persianss.ir

t

t

Tex book of Pedia rics

 

Figure 3.3.4 WHO Multicenter Growth Reference Study Group. WHO child growth standards based on length/height, weight and age. Acta Paediatr. 2006;95(suppl 450):76-85

90

vip.persianss.ir

wt

Gro nt

p

D

n

h a d evelo me

 

Figure 3.3.5 WHO Multicenter Growth Reference Study Group. WHO child growth standards based on length/height, weight and age. Acta Paediatr. 2006;95(suppl 450):76-85

91

vip.persianss.ir

Tex book of Pedia rics t

t

 

Table 3.3.1 Correlation between percentiles and Z scores for WHO charts Z score

Exact percentilesi

Rounded percentilesi

0

50

50

-1

15.9

15

-2

2.3

3

-3

0.1

1

1

84.1

85

2

97.7

97

3

99.9

99

 

Table 3.3.2 Growth parameters and their interpretation for the WHO charts Z score (percentile)

Length/height-for-age

Weight-for-age

Body mass index (BMI)for-age

> 3 (99)

May be abnormal

May be abnormal (use BMI)

Obese

> 2 (97)

Normal

Use BMI

Overweight

> 1 (85)

Normal

Use BMI

Risk of overweight

0 (50)

Normal

Use BMI

Normal

< −1 (15)

Normal

Normal

Normal

< −2 (3)

Stunted

Underweight

Wasted

< −3 (1)

Severely stunted

Severely underweight

Severely wasted

 

Table 3.3.3 Weight (kg) by age of boys and girls aged 0–2 years (WHO) Percentiles for boys

Percentiles for girls

Age in months

3rd

5th

25th

50th

75th

95th

97th

3rd

5th

25th

50th

75th

95th

97th

0

2.5

2.6

3.0

3.3

3.7

4.2

4.3

2.4

2.5

2.9

3.2

3.6

4.0

4.2

3

5.1

5.2

5.9

6.4

6.9

7.7

7.9

4.6

4.7

5.4

5.8

6.4

7.2

7.4

6

6.4

6.6

7.4

7.9

8.5

9.5

9.7

5.8

6.0

6.7

7.3

7.9

8.9

9.2

9

7.2

7.4

8.3

8.9

9.6

10.6

10.9

6.6

6.8

7.6

8.2

8.9

10.1

10.4

12

7.8

8.1

9.0

9.6

10.4

11.5

11.8

7.1

7.3

8.2

8.9

9.7

11.0

11.3

15

8.4

8.6

9.6

10.3

11.1

12.3

12.7

7.7

7.9

8.8

9.6

10.4

11.8

12.2

18

8.9

9.1

10.1

10.9

11.8

13.1

13.5

8.2

8.4

9.4

10.2

11.1

12.6

13.0

21

9.3

9.6

10.7

11.5

12.5

13.9

14.3

8.7

8.9

10.0

10.9

11.8

13.4

13.8

24

9.8

10.1

11.3

12.2

13.1

14.7

15.1

9.2

9.4

10.6

11.5

12.5

14.2

14.6

 

Table 3.3.4 Weight (kg) by age percentiles of boys and girls aged 2–5 years (WHO) Age

92

Percentiles for boys

Percentiles for girls

Year

Month 3rd

5th

25th

50th

75th

95th

97th

3rd

5th

25th

50th

75th

95th

97th

2

0

10.1

11.3

12.2

13.1

14.7

15.1

9.2

9.4

10.6

11.5

12.5

14.2

14.6

9.8

2

6

10.7

11.0

12.3

13.3

14.4

16.2

16.6

10.1

10.4

11.7

12.7

13.8

15.7

16.2

3

0

11.4

11.8

13.2

14.3

15.6

17.5

18.0

11.0

11.3

12.7

13.9

15.1

17.3

17.8

3

6

12.2

12.5

14.1

15.3

16.7

18.9

19.4

11.8

12.1

13.7

15.0

16.4

18.8

19.5

4

0

12.9

13.3

15.0

16.3

17.8

20.2

20.9

12.5

12.9

14.7

16.1

17.7

20.4

21.1

4

6

13.6

14.0

15.9

17.3

19.0

21.6

22.3

13.2

13.7

15.6

17.2

18.9

22.0

22.8

5

0

14.3

14.7

16.7

18.3

20.1

23.0

23.8

14.0

14.4

16.5

18.2

20.2

23.5

24.4

vip.persianss.ir

Gro

 

Table 3.3.5 Weight (kg) by age of boys and girls aged 5–10 years (WHO) 3rd

5th

15th

25th

50th

75th

85th

95th

wt

Percentiles Age (years)

97th

h a d evelo me

14.7

16.7

18.3

20.1

23.0

23.8

16.6

17.9

18.8

20.5

22.5

23.6

25.8

26.7

7

17.9

18.4

19.9

20.9

22.9

25.2

26.5

29.1

30.1

8

19.8

20.4

22.0

23.1

25.4

28.1

29.7

32.7

34.0

9

21.6

22.3

24.2

25.4

28.1

31.3

33.2

36.9

38.6

10

23.6

24.4

26.6

28.0

31.2

34.9

37.3

41.9

43.9

5

14.0

14.4

16.5

18.2

20.2

23.5

24.4

6

15.5

16.0

18.3

20.2

22.4

26.2

27.3

D

14.3 16.1

p

5 6

n

Boys

17.4

23.7

7

17.0

17.6

19.2

20.2

22.4

24.9

26.5

29.5

30.8

8

18.9

19.5

21.3

22.5

25.0

28.0

29.8

33.4

34.9

9

21.1

21.8

23.9

25.3

28.2

31.7

33.9

38.1

40.0

10

23.7

24.5

26.9

28.5

31.9

35.9

38.5

43.5

45.7

nt

Girls

 

Table 3.3.6 Length (cm) by age of boys and girls aged 0–2 years (WHO) Age (months)

3rd

5th

25th

Percentiles for boys 50th

75th

95th

97th

3rd

5th

25th

Percentiles for girls 50th

75th

95th

97th

0

46.3

46.8

48.6

49.9

51.2

53.0

53.4

45.6

46.1

47.9

49.1

50.4

52.2

52.7

3

57.6

58.1

60.1

61.4

62.8

64.8

65.3

55.8

56.3

58.4

59.8

61.2

63.3

63.8

6

63.6

64.1

66.2

67.6

69.1

71.1

71.6

61.5

62.0

64.2

65.7

67.3

69.5

70.0

9

67.7

68.3

70.5

72.0

73.5

75.7

76.2

65.6

66.2

68.5

70.1

71.8

74.1

74.7

12

71.3

71.8

74.1

75.7

77.4

79.7

80.2

69.2

69.8

72.3

74.0

75.8

78.3

78.9

15

74.4

75.0

77.4

79.1

80.9

83.3

83.9

72.4

73.0

75.7

77.5

79.4

82.0

82.7

18

77.2

77.8

80.4

82.3

84.1

86.7

87.3

75.2

75.9

78.7

80.7

82.7

85.5

86.2

21

79.7

80.4

83.2

85.1

87.1

89.9

90.5

77.9

78.6

81.6

83.7

85.7

88.7

89.4

24

82.1

82.8

85.8

87.8

89.9

92.8

93.6

80.3

81.1

84.2

86.4

88.6

91.7

92.5

 

Table 3.3.7 Height-for-age percentiles for boys and girls 2–5 years of age (WHO)

y

Age 2

ear

Percentiles for boys

Percentiles for girls

Month

3rd

5th

25th

50th

75th

95th

97th

3rd

5th

25th

50th

75th

95th

97th

0

81.4

82.1

85.1

87.1

89.2

92.1

92.9

79.6

80.4

83.5

85.7

87.9

91.0

91.8

2

6

85.5

86.3

89.6

91.9

94.2

97.5

98.3

84.0

84.9

88.3

90.7

93.1

96.5

97.3

3

0

89.1

90.0

93.6

96.1

98.6

102.2

103.1

87.9

88.8

92.5

95.1

97.6

101.3

102.2

3

6

92.4

93.3

97.2

99.9

102.5

106.4

107.3

91.4

92.4

96.3

99.0

101.8

105.7

106.7

4

0

95.4

96.4

100.5

103.3

106.2

110.2

111.2

94.6

95.6

99.8

102.7

105.6

109.8

110.8

4

6

98.4

99.4

103.7

106.7

109.6

113.9

115.0

97.6

98.7

103.1

106.2

109.2

113.6

114.7

5

0

101.2

102.3

106.8

110.0

113.1

117.6

118.7

100.5

101.6

106.2

109.4

112.6

117.2

118.4

WHO growth charts are used for children up to 4 years of age and thereafter British charts are used. The reason to adopt WHO growth charts for use in young children is because it establishes the growth of breastfed infants as the norm for

growth; the WHO standards provide a better description of physiological growth in infancy and WHO standards are based on a high-quality study designed explicitly for 93 creating growth charts.

vip.persianss.ir

Tex book of Pedia rics

 

Table 3.3.8 Height (cm) by age of boys and girls aged 5–10 years (WHO)

t

Height-for-age percentiles Age (year)

3rd

5th

5

101.2

102.3

6

106.7

107.8

7

111.8

113.0

15th

25th

50th

75th

106.8

110.0

113.1

110.8

112.6

116.0

119.3

116.3

118.2

121.7

125.3

85th

95th

97th

117.6

118.7

121.1

124.1

125.2

127.2

130.4

131.7

t

Boys

8

116.6

118.0

121.4

123.5

127.3

131.1

133.1

136.6

137.9

9

121.3

122.7

126.3

128.5

132.6

136.6

138.8

142.5

143.9

10

125.8

127.3

131.2

133.5

137.8

142.1

144.4

148.3

149.8

5

100.5

101.6

106.2

109.4

112.6

117.2

118.4

6

105.5

106.7

111.7

115.1

118.6

123.5

124.8

Girls 109.8

120.4

7

110.5

111.8

115.1

117.1

120.8

124.5

126.5

129.8

131.1

8

115.7

117.0

120.5

122.6

126.6

130.5

132.6

136.1

137.5

9

121.0

122.4

126.2

128.4

132.5

136.6

138.8

142.5

144.0

10

126.6

128.1

132.0

134.3

138.6

143.0

145.3

149.2

150.7



B







1. de Onis M. Growth curves for school age children and adolescents. Indian Pediatr. 2009;46(6):464-5. 2. Khadilkar VV, Khadilkar AV, Chiplonkar SA. Growth performance of affluent Indian preschool children: a comparison with the new WHO growth standard. Indian Pediatr. 2010;47(10):869-72. 3. WHO Multicentre Growth Reference Study Group. WHO Child Growth Standards based on length/height,weight and age. Acta Paediatr Suppl. 2006;450:76-85. 4. World Health organization. Growth charts. Available from http://www.cdc.gov/growthcharts/who_charts.htm. [Accessed 30 September 2011]. 5. World Health Organization. Training Course on Child Growth Assessment. Geneva, WHO; 2008.



ibliography





In India using WHO growth charts for children under the age of 5 years is likely to overdiagnose stunting or underweight or both. In a recent multicentric study done on 1,493 affluent Indian children on all zones of India, published by the author, the mean Z scores for height, weight, BMI and weight-for-height [−0.75 (1.1), −0.59 (1.1), −0.19 (1.22) and −0.26 (1.18), respectively] were much below the WHO 2006 standards. The overall incidence of stunting was 13.6% and underweight was 8.5% amongst affluent Indian children under the age of 5 years. The same is likely to be higher in rural areas and in underprivileged urban areas of India, although, at the present time no such data is available. It may therefore be necessary to use a lower cut off of WHO standards (i.e. using the 1st rather than 3rd percentile) for referral to specialized centers in developing countries such as India.

94

vip.persianss.ir

P

D

Growth and Sexual evelopment in uberty





Growth and evelopment

3.4

KN Agarwal

•

­



It occurs between 8 years and 14 years of age in girls and between 9 years and 15 years of age in boys • Girls: Breast enlargement, occasionally initially unila teral, is the first obvious sign of puberty and occurs between 10 years and 11 years of age • Boys: Testicular volume increases from 2.0 mL to more than 4.0 mL or testes length from 2.0 cm to 3.2 cm between 12 years and 13 years of age; 1 year later penile and scrotal enlargements occur.

Growth Spurt • • •

Period extends for 4 years in girls and 6 years in boys to cross “sexual maturity stages” 2–5 Height gain is 27–29 cm in boys and 24–26 cm in girls. Weight gain in both is around 25–30 kg.

D

P

E P

Initiation of uberty

D





For 2 years before puberty, there is a rise in levels of adrenal androgens (adrenarche) that can sometimes result in the early appearance of pubic hair and spots. The initial event of puberty is an increase in pulsatile release of gonadotropin hormone releasing hormone (GnRH). Therefore, the initiation of the pubertal process itself requires both changes in trans-synaptic communication and the activation of glia-to-neuron signaling pathways. While neurons that utilize excitatory and inhibitory amino acids as transmitters represent major players in the trans-synaptic control of puberty, glial cells utilize a combination of trophic factors and small cell-signaling molecules to regulate neuronal function and, thus, promote sexual development. A coordinated increase in glutamatergic transmission accompanied by a decrease in inhibitory GABAergic tone appears to initiate the trans-synaptic cascade of events leading to the pubertal increase in GnRH release. In response to GnRH stimulation, pituitary releases luteinizing hormone (LH) and follicle-stimulating hormone (FSH). In males, LH stimulation is responsible for testosterone production, while FSH causes maturation of sperms. In females, both LH and FSH are required for production of estradiol while FSH is also required for ovulation.

ifferences between Boys and Girls

For boys, testosterone is the principal sex hormone. It induces the characterization known as virilization. A substantial product of testosterone metabolism in males is estradiol, though levels rise later and more slowly than in girls. The male “growth spurt” also begins later, accelerates more slowly and lasts longer before the epiphysis fuse. Although boys are on average similar in height or 2 cm shorter than girls before puberty begins, adult men are on average about 13 cm taller than women. The growth spurt occurs later than in girls because testosterone is a poor stimulator to GH responsiveness than estradiol in girls. Testosterone is required in relatively higher concentration to produce the same growth effect. The hormone that dominates female development is an estrogen called estradiol, which rises earlier and reaches higher levels in women than in men. It promotes growth of breasts and uterus, and is responsible for the pubertal growth spurt, epiphysis maturation and closure. Girls attain reproductive maturity about 4 years after the first physical changes of puberty appear. In contrast, boys accelerate more slowly but continue to grow for about 6 years after the first visible pubertal change.



Puberty is the growth process associated with appearance of both primary and secondary sexual characteristics in children. The changes encompass aspects of sexuality and somatic growth, together with cognitive development. Pubescent children are those in whom secondary sexual characters and early genital changes are appearing. The WHO defines adolescents as individuals in the age group of 10–19 years. The various physiological: • Adolescent growth spurt • Change in body composition (muscle/fat) • Skeletal maturity. The time of onset and the rate of sexual development have individual variability but the sequence of events remains the same. From a biological perspective, puberty is the stage of physical maturation in which, an individual becomes physiologically capable of sexual reproduction. The biological changes that occur during puberty include several neurosecretory factors and/or hormones, all of which modulate somatic growth, the development of the sex glands and their endocrine as well as exocrine secretions. The resultant increase in sex steroid production will ensure the appearance and maintenance of sexual characteristics and the capacity for reproduction. It is essentially the activation of the hypothalamic-pituitary-gonadal axis that induces and enhances the progressive ovarian and testicular sex hormone secretion that are responsible for the profound biological, morphological and psychological changes to which the adolescent is subjected.

ndocrinal Control of uberty



D

efinition

95

vip.persianss.ir

P

N

First sign of ovarian estradiol secretion is breast development “thelarche” [breast budding, B-2 or sexual maturity rating (SMR-2)] with “growth in height” (Fig. 3.4.1 and Table 3.4.1). Estradiol being a good stimulator of “growth hormone” doubles the growth velocity. The maximum height velocity [peak height velocity (PHV) = 9–10 cm/year] coincides with B-3. Thus growth spurt occurs early in female puberty, following B-2 by 1 year. Height velocity reduces to 4 cm/ year at menarche. The growth in the post enarche period is limited as girls can gain 5–6 cm in linear growth only. Puberty is associated with change in body shape like hip growth, increase in body fat from 16% to 28% and reduction in lean body mass from 80% to 72%. • Menarche follows PHV by 14–18 months or usually occurs about 2–3 years after the start of breast development (thelarche). The age of menarche is around 12–13 years (12.6 years in Indian girls). • Estradiol is the main hormone in females influencing the pubertal development, i.e. breast and genitals; and promotes uterine maturation and fat deposition in typical female contours, while androgens from adrenals and ovaries are responsible for pubic and axillary hair and the typical body odor and acne.

The first sign of puberty is testicular enlargement, which usually occurs between ages of 12 years and 13 years. The prepubertal testis is about 2 mL in volume with puberty taken to begin when a volume of around 4 mL is attained.

B-2

 



Rapid pubertal growth occurs once testes are more than 6 mL. Actually testicular growth starts as early as 10 years of age, associated with enlargement of seminiferous tubules, epididymis, seminal vesicles and prostate. Prader orchidometer (Fig. 3.4.2) is used to measure testicular volume. Penile and scrotal enlargements occur typically about a year after testicular enlargement is noticed. Pubic hair typically appears at a similar time (Fig. 3.4.3 and Table 3.4.2). • A greater and later growth spurt occurs in boys and ultimately achieves an average 12.6 cm with greater height in adult men. The growth spurt in boys is on average 2 years later than girls in SMR stages 3 and 4.

G-2

G-3

G-4

G-5

B-3

 

Figure 3.4.1 Development of breast (stages B2–B5) during puberty in girls

Figure 3.4.3 Genital development in boys in sexual maturity stages (G 2-G 5)  

B-5

B-4

96

Figure 3.4.2 Prader orchidometer



ormal uberty in Boys P

N



ics b

P

i

ext ook of ed atr T

ormal uberty in Girls

vip.persianss.ir

Breasts

Pubic hair (mean age = 13.6 years)

Growth

Other features

Undernourished rural girl

Stage 1 (B-1)

Prepubertal: elevation of papilla only

Prepubertal villus hair only

Basal level: 5–6 cm/ year

Adrenarche: ovaries grow and enlarge

Height was lower by 15.3 cm versus affluent girl

Stage 2 (B-2)

Breast bud appears under an enlarged areola. Milk ducts begin to grow (mean age 10.2 years)

Sparse hair along labia (in 22%)

Accelerated growth: about 7–8 cm/year

Breast development Clitoral enlargement delayed by 2.2 years with labial pigmentation, uterine enlargement, increase in vaginal length, menarche attained in 10%

Stage 3 (B-3)

Breast tissue grows beyond areola but without contour separation. The areola begins to darken in color. The milk ducts give rise to milk glands that also begin to grow (mean age 11.6 years)

Hair coarser and pigmented: spreads across pubes (becoming thicker, curly and darker in 92%)

Peak velocity: about 8 cm/year

Axillary hair present in majority Acne in majority Menarche attained in 20% (total 30%)

Peak height velocity not observed Height gain similar 24–2 cm; early life deficit not corrected

Stage 4 (B-4)

Projection of areola: papilla forms a secondary mound (mean age 13.6 years)

Deceleration: less Adult pattern but than 7 cm/year without spread to medial thigh, growing faster and already forming sexual pubic triangle (in 98.8%)

Menarche: mean age = 12.6 year. Regular periods (around 14 years) Menarche attained in total = 90%

Menarche delayed by 0.82 years

Stage 5 (B-5)

Adult breast contour with projection of nipple only (mean age 14.5 years)

Adult triangle is already developed. Hair is thick and often curly with spread to medial thigh but not up linea alba (100%)

Adult genitalia Menarche in all girls

Total period of growth in puberty shortened

Cessation of growth at around 16 years

D

 

Tanner stage

Growth and evelopment

Table 3.4.1 Stages of normal puberty (Tanner stages) in girls for breast, pubic hair and in relation to the peak height velocity (values in parenthesis are for Indian girls)

Source: Agarwal et al.

Body takes on a more muscular and angular shape because of testosterone. This generally begins around age 12.5 years when testosterone causes muscle mass to increase. The greatest effect can usually be seen in the upper chest and shoulder muscles. Testosterone also causes bones to lengthen, giving young men a heavier bone structure and longer arms and legs. At the end of puberty, adult men have heavier bones and nearly twice as much skeletal muscles. Some of the bone growths (e.g. shoulder width and jaw) are

Body dor and cne A

V

M

uscle Growth

disproportionately greater resulting in noticeably different male and female skeletal shapes. The average adult male has about 150% of the lean body mass of an average female, and about 50% of the body fat. Muscle growth can continue even after boys are biologically adult. The peak of the so-called “strength spurt”, the rate of muscle growth, is attained about 1 year after a male experiences his peak growth rate. O

F

ace: 25% of the total height of the mandible ramus occurs between 12 years and 20 years of age. • yes: Growth in axial diameters results in a tendency to “myopia” in adolescence. • oice: Growth of larynx, pharynx and lungs leads to typical voice. E

•

Rising levels of androgens can change the fatty acid composition of perspiration resulting in a more “adult” body odor. As in girls, another androgen effect is increased secretion of oil (sebum) from the skin and the resultant 97 variable amounts of acne. Acne cannot be prevented or diminished easily, but it fully diminishes at the end of puberty.

vip.persianss.ir

ics

Tanner stage Genitalia

Pubic hair

Growth

Other

Undernourished rural boy

Stage 1 G-1

Prepubertal: testes less than 2.5 cm or less than 2-0 mL

Villus hair only

Basal height velocity 5–6 cm/year

Adrenarche

Height at 13 years was short by 13.6 cm versus affluent Indian boy

Stage 2 G-2

Thinning and reddening of scrotal skin (mean age 11.3 years). Testes 2.5–3.2 cm or 4 mL

Sparse growth at base of penis (60%)

As above

Total body fat 16–18%

Stage 3 G-3

Growth of penis (mean age 12.8 years). Testes 3.3–4.0 cm or 6–8 mL

Thicker hair: spreads to mons pubis (97%)

Accelerated growth: 7–8 cm/year

Gynecomastia Voice break Increase in muscle mass

Genital development delayed by 1.54 years

Stage 4 G-4

Growth of penis and glans with darkening of scrotum (mean age 14.1 years) Testes 4.1–4.5 cm or 10–12 mL

Adult but no spread Peak velocity about to medial thigh 10.0 cm/year (99%)

Axillary hair Voice change Acne

Delayed appearance: axillary hair by 0.65 year; pubic hair by 0.82 year No peak height velocity noted

Stage 5 G-5

Adult genitalia (mean age Adult with spread to medial thigh but 16.4 years) Testes greater than 4.5 cm not linea alba or 12 mL (adult volume 18 mL)

Facial hair (mean age 14.8 years) Muscle mass increases further and beyond stage 5

Total weight gain only 38% as compared to affluent Indian boy

Deceleration and cessation (about 17 years)

recocious uberty P

P

No testicular enlargement by age of 14 years Pubic hair absent by age of 15 years More than 5 years between the start and completion of growth of the genitalia.



Precocious puberty is defined as the onset of secondary sexual characteristics before the age of 8 years in girls (although some sources lower this to 7 years) and 9 years in boys. It is five times more common in girls. Precocious puberty is usually a benign central process in girls but in boys a pathological peripheral cause should be excluded (found in 50%). Benign variants may manifest as breast development in girls aged less than 3 years, which spontaneously regresses, and pubic hair in both boys and girls aged less than 7 years due to adrenal androgen secretion in middle childhood (polycystic ovary syndrome—recommend follow-up). In central precocious puberty, the puberty process starts too soon. Although they begin earlier than they should, the pattern and timing of the steps in the process are otherwise normal. For the majority of children with this condition, there is no underlying medical problem and no identifiable reason for the early puberty.

• 98 • •

No breast development by 13 years No menarche by 3 years after breast development Menarche not attained by 16 years.

E

Changes under Sex ormone ffects H

Girls

P

P

D

P

ointers for elayed uberty

A

Growth attern and chievement during uberty P

•

When a boy or girl has passed the usual age of onset of puberty by 2–3 years with no physical or hormonal signs Puberty may be delayed for several years and can still occur normally—constitutional delay, a variation of healthy physical development. Delay of puberty may also occur due to undernutrition (malnutrition, cystic fibrosis and anorexia nervosa), many forms of systemic diseases (thalassemia and chronic renal failure) or defects of the reproductive system (hypogonadism and polycystic ovarian disease) or the body’s responsiveness to sex hormones. Constitutional growth delay is the most common cause in boys (> 50%). The most common cause in girls is Turner syndrome (> 80% girls have pathological cause of delayed puberty).

• • •



•

ate uberty P

L

The genetic factors account for half of the variation of pubertal timing; association of timing is strongest between mothers and daughters. The environment factors are clearly important as well; puberty occurs later in children raised at higher altitudes and in undernourished children (Tables 3.4.1 and 3.4.2). It is advanced in obese children.

•

Boys

P

actors ffecting uberty A

F

T

b

P

i

ext ook of ed atr

 

Table 3.4.2 Genital development and growth in boys during puberty (the values in parenthesis are for Indian boys)

Growth begins in distal parts like feet and hands, which also stop growing first. It is followed by growth of arms, legs, trunk and chest. The growth of trunk changes US/LS ratio

vip.persianss.ir



• •

D

P

W

P

A

ssential easurements to ssess Sexual evelopment and Growth in uberty M

At 10.5 years, boys and girls have similar height 11–12.5 years girls are taller by 2.0 cm 14 years boys are taller by 5 cm 16.0 years boys are taller by 12.5 cm The height velocity is given in relation to sexual maturity in Tables 3.4.1 and 3.4.2.

D

• • • • •

E

D

H

eight and eight Gains in uberty ( uration 4–6 Years)

Total weight gain is around 25–30 kg during puberty. The peak weight velocity follows peak height velocity. Bone growth: 50% completed during first month of life to puberty onset, 30% in puberty and 20% in late adolescence to adult. Girls mature earlier than boys, grow for a shorter time and ultimately have shorter overall bone lengths by about 7%.

Growth and evelopment

(U/S ratio), becomes 1.1 at 10–11 years, 0.98–1 at 13–14 years and 1–1.1 at completion of puberty. Pelvic inlet is wider in girls (wider hips) with more growth of acetabula. In contrast, boys have greater stature and broader shoulders.

Assess sexual maturity as per Tanner classification (Tables 3.4.1 and 3.4.2, Figs 3.4.1 and 3.4.2). The variability in time of onset and progression of puberty, which relates to somatic

Breast Development Stages Source: Agarwal KN et al. Physical growth assessment in adolescence. Indian Pediatrics. 2001;38:1217-35

99

vip.persianss.ir

ics b

P

i

ext ook of ed atr T

Genital Development Stages Source: Agarwal KN et. al. Physical growth assessment in adolescence. Indian Pediatrics. 2001;38:1217-35













1. Agarwal KN, Agarwal DK, Seth A. The growth infancy to adolescence, 2nd edition. New Delhi: CBS Publishers; 2007. pp. 35-49. 2. Agarwal KN, Agarwal DK, Upadhaya SK, et al. Physical and sexual growth pattern of affluent Indian children from 5-18 years of age. Indian Pediatr. 1992;29:1203-82. 3. Agarwal KN, Saxena A, Bansal AK, et al. Physical growth assessment in adolescence. Indian Pediatr. 2001;38:1217-35. 4. Duke PM, Litt IF, Gross RT. Adolescents’ self-assessment of sexual maturation. Pediatrics. 1980;66:918-20. 5. Tanner JM. Growth in Adolescence, 2nd edition. Oxford: Blackwell Scientific Publication; Oxford.



Bibliography



growth rather than chronological age, requires assessment of stage of sexual maturation. The necessity to undress the teenager has seriously curtailed the assessment by experts, the best approach is to give Tanner’s stages as diagram to children and they make self-assessment. • The height, weight, BMI and SFT values for age in relation to “sexual maturity” are described (see WHO Growth Curves 1 and 2). The details of measurements are given in the Chapter 3.2. • Waist/hip ratio less than 0.8 women and less than 0.9 men, indicates good health, values over and above suggest overweight/obesity). 100 • Waist-to-height ratio less than 0.5, no central obesity versus more than or equal to 0.5, central obesity present.

vip.persianss.ir

D

ormal evelopment

N



Growth and evelopment

3.5

DK Agarwal Prefrontal cortex is not myelinated until close to adolescence.

D

•

P

D

evelopmental rofile

• • • • • • • •

Early brain stem and cord—birth: light reflex, startle reflex, Babinski reflex, reflex movement, reflex birth cry and grasp reflex Visual, auditory, tactile, mobility, language and manual competences Brain stem and early subcortical areas—2.5 months Midbrain and subcortical areas—7 months Initial cortex—12 months Early cortex—18 months Primitive cortex—36 months Sophisticated cortex—72 months



Development refers to qualitative and quantitative changes and acquisition of a variety of competencies for functioning optimally in a social milieu. Further, development is a continuous process from birth to maturity. It depends on maturation and myelination of brain. Unless that has occurred, no amount of practice can make the child learn that skill. It may be stressed that besides 10% prevalence of developmental delay, the early identification of such problems remains difficult. Although severe disorders can be recognized in infancy, it is unusual to diagnose speech impairment, hyperactivity or emotional disorders before the age of 3 or 4 years, and learning disabilities are rarely recognized before children start their schooling. If one can diagnose developmental delay in early stages of growth, the intervention can reduce long-term sequel. Developmental delay is said to exist, if the child does not reach developmental milestones at the expected age, i.e. broad variation among normal children. Although the delay may occur from a biological factor such as chromosomal disorder or an environmental factor such as maternal depression, the primary model for pathogenesis of developmental delay is a transactional one, with the process of development viewed as an interaction between the child and environment, in which each can have profound effect on other.

P

rocess of Brain Growth

•

A



The developmental history and physical findings should be compared with the achievements listed for normal children. For preterm, one must take their corrected age into account. If one finds any “warning sign”, take note of the points in history and physical examination and define the type of impairment, disability or any handicap. The children may be observed for their activities as follows:

reschool ge A

P

•



Play, climbing stairs, speech, hand (for adaptive behavior) and feeding • Posture, walking, play and manipulation with toys (tests for vision) • erformance: understanding, matching color, concent ration and visual acuity • Comprehension of language: To point to body parts, objects in books, to pick named toys and accept commands appropriate to age.

• •

Sensory and motor areas within first month to first year of life—training and practice are effective only after myelination Maximum myelination occurs by 6 years of life

­

yelination

P

rocess of

M

P



–

D

ssessment of ormal evelopment

–

E

• •

In the ectoderm, notochord develops to form a neural groove—neural tube (cavity with overlying neural crest) form in 18–24 days. rror: results in spina bifida, anencephaly, etc. Few weeks after conception to cellular level through adolescence, brain continues to grow and myelinate – Cells inside the tube form central nervous system (CNS) – Cells outside and the ectoderm form autonomic nervous system (ANS).

Gross motor Fine motor Language Cognitive Self-help Social. The developmental pattern from birth to first birthday is given in Figure 3.5.1. Normal development is given in Table 3.5.1 for the developmental abilities at various ages. N

Brain growth is important to receive stimuli and take body functions. The process of brain growth and acquisition of developmental processes is summarized as:

• • • • • •

A

D

Brain Growth and evelopment

Skill chievements

101

vip.persianss.ir

cs b

P

ext ook of ediatri T

 

Figure 3.5.1 Growth and development in first 2 years of life

102

vip.persianss.ir

Growth and evelopment

 

Table 3.5.1 Developmental abilities and warning signs at various ages AREAS Personal social

Language hearings and speech

Adaptive

Gross motor

Warning signs

Stills to mother's voice, startles at sudden noice

Follows face 90° stares intently

Primitive reflexes +head in line with trunk when lifted

* None is elicited *Abnormal Moro's *Persistent squint

6 month. Responds to own name. Speaks ma, da. 9 month. Mama, dada (Double syllable) Understands 'No'

6 to 7 months. Change grasp palmar to index. Transfers objects hand to mouth. 9 months Pincer grasp foot regard. Fixes pellet of paper, follows fallen object

6 months bears some wt on legs, rolling over, in prone head up. wt.of hands 7 months Crawls and pulls to stand

*Slow social responses *Absence of babble *Persistence of hand Pats regard. *Abnormal voluntary hand grasp. *Persistent primitive reflexes

Understands some words, uses 'mama, dada' with meaning 'No'

Throws objects, watches them fall, picks up crumbs from floor. Pincer grasp, shakes head, Bangs two bricks together

Shuffling gait like a bear. Cruises round holding on to furniture. Walks one hand held, pivots when sitting

*No tunefull babble *Hold objects close to eyes. *Immature gait *No sitting

Points to 3 body parts. Obeys single commands. Says 6 words. Jargons echoes, speech

Walks well, Carries toys. Neat pincer picking of threads pins. Scribbles using Climb stairs. Climbs into chair fisted grasp. Turns 2 or more pages at a time. Builds tower of 3–4 (2.5 cm) cubes

Phrases of 2–3 words, gives name, 50 words+, naming games, Has inner language

Turns one page at a time, Imitates a straight line in both vertical and horizontal and a circle, unscrews lids, makes tower of 6–8 cubes

Pushes tricycle with feet, walks downstairs 2 feet per tread. Runs, kicks ball jumps on the spot

*No speech *Unsteady on feet

Mature pen grasp, copies + and 0 Correctly matches 2 or more colors. Threads large beads. Makes tower of 9

Stands on one leg for a few seconds. Peddles tricycle, stairs adult style for ascent, jumps of bottom step

*No phrases * Persistent day-time wetting/soiling *Clumsy

Matches 4 colors, Copies cross, square and by 5 a triangle. Draws a recognizable man

4 years climbs trees and ladder, enjoys ball games. 5 years hops, skip jump off 3 steps, catches a ball

*Socially isolated. *Unintelligible or ungrammatic speech. *Unable to tell name or address

Smiles, coos responsively

D

6 weeks old

6 to 9 months old 6 month enjoys bath, playing boo and chew on items. 9 months show objects to mother, mirror image

12 months old Comes when called, finds hidden objects, waves byebye, gives toys on request

18 months old Cup: Lifts-drinks-and puts down. Self spoon feeding. Pulls at dirty nappy. Does dusting, sweeping

*Drools no words *Absent pincer grasp *Does not walk

2 to 2½ years Plays alone, tantrums, demanding. Day by day, puts on shoes, socks and pants. Turns door handles. Uses spoon and fork 3 to 3½ years Gives full name, sex. Counts Goes toilet unassisted, to 10, 3–5 word sentences dresses/undresses with minimum assistance. Knows some nursery rhymes, handles knife and fork, plays with peers 4 to 5 years Wipes own bottom. Eats using knife and fork, dresses-except for tie and laces, imaginative play, plays in groups, shares toys, obeys rules

Gives address/age/ telephone no., Counts up to 10 by 4 years, 20 by 5 years knows 3 coins, grammatical speech asks meaning of abstract words

A

School ge

•

Test for reading, arithmetic functions like +, –, ×, ÷, writing name, age, address, drawing a picture; to test application, concentration and organizational skills Test deafness and physical examination

• •

Vision by 3–5 years age of 6/6 (adult) capability Intelligence assessment.

Various tests which can be used for developmental screening are already detailed in sections 3.1 and 3.6.

•

103

vip.persianss.ir

D

D



evelopmental elay

E

104

tiopathogenesis

Child development is a dynamic process optimally utilizing the genetic potential of the baby, within the context of the available environment, enabling achievement of full potential. Severe forms of disability are less common and are often due to congenital, genetic, metabolic causes or intrauterine infections and need specific preventive strategies. Experience of a developmental evaluation clinic has shown that nearly 50% of babies referred for developmental evaluation had developmental delay without a specific clinical diagnosis. Delayed cry at birth, increasing age of the child, presence of feeding problems, assisted



E E B

Advances in perinatal care have improved the survival chances of low birth weight babies, adding to the burden of developmental delay. It has been shown that 40% reduction in poor performance could be achieved among neonatal nursery graduates by CDC model early stimulation. While “high-risk” newborns require periodic screening, ideally need to be determined locally. It must also be remembered that many babies not considered “high-risk” may also manifest developmental problems as they grow. These babies would obviously not be seen during “high-risk” focused follow-up screening.

evelopmental ssessment A

Statistics from different sources indicate that in India, 3.8% of the population has some form of disability, and the same was found to be more common among children of the lowest-socioeconomic class families when compared with the next-to-lowest class families. Out of the 2.5% prevalence of developmental delay/disability among under 5 years children in an integrated child development services (ICDS) block, majority had speech and language problems followed by orthopedic deformities, cerebral palsy, vision and hearing problems and mental retardation. The observed 2.5% prevalence of developmental delay in less than 2-yearold children deprived of urban settlements, the presence of risk factors for developmental delay-like low birth weight, birth asphyxia, coupled with poor environment of home and alternate child care services, highlights the need for simple cost-effective community model for promoting early child development.

Developmental screening of all babies particularly graduates of neonatal intensive care unit (ICU), including vision and hearing domain is important in identification of potentially handicapping conditions that may be prevented or ameliorated if addressed early. A screening test is only meant to identify children who might have a delay and who are in need of further developmental evaluation.

irth to ne Year O

pidemiology



Developmental delay exists when a child does not reach developmental milestones at the expected age (with the adequate provision for the broad variation among normal children). Developmental delays may occur in any or all of the major areas of child development: gross motor, fine motor, language and social. Identification of developmental delay is useful for introducing early intervention programs, with the objective of reducing childhood disability. Developmental prediction is not always possible, as we may go wrong often. By doing developmental assessment, we can only opine on present status in relation to age and average performance of other children of same age. We cannot accurately predict future intelligence, delayed maturation effects and longterm effects of emotional deprivation.

delivery and birth injury were found to be associated with increasingly abnormal developmental test. A risk factor is something that increases the likelihood of getting a disease or condition. The risk factors can be classified as follows: • stablished risk: These include medical disorders that can lead to developmental delay. It includes Down syndrome, hydrocephalus, cerebral palsy, hearing impairment, visual impairment and other congenital anomalies. • nvironmental risk: This includes limited environmental factors, which put a child at risk for developmental delay. The various factors are: a very young mother, extreme poverty, low socioeconomic status, single parent, etc. • iological risk: These include factors-like prematurity, low birth weight, neonatal hypothermia, asphyxia, hypoglycemia, hyperbilirubinemia and convulsions. These are factors which operate in the prenatal, natal and postnatal periods. • No apparent risk: Developmental delays also occur in infants without any apparent risks. In not more than 10% of cerebral palsy cases perinatal asphyxia could be attributed as the true cause.

D

efinition

B

D

MKC Nair

E

t

t

Tex book of Pedia rics

3.6

Developmental Observation Card The developmental observation card (DOC) is a simple developmental card that can be used by parents to identify delay.

vip.persianss.ir

ompleted 2 months: Social smile—baby smiling back in response to your smile • ompleted 4 months: Holds head steady—keeping head steady when baby is held upright • ompleted 8 months: Sits alone—baby is able to sit alone with back straight, no support • ompleted 12 months: Stands alone—baby is able to stand bearing weight on both legs with minimal support.

D

C

n

C

t

C

C

Grow h a d evelo me

•

IV

III

II

nt

I

Head holding grading (assessed at completed 4 months): • Grade 0: No head holding at all • Grade : Head erect and steady momentarily (Fig. 3.6.1) • Grade : Dorsal suspension—lifts head along with body (Fig. 3.6.2) • Grade : Prone position—elevates on arms, lifting chest (Fig. 3.6.3) • Grade : Holds head steady while mother moves around (Fig. 3.6.4) • Grade : Head balanced at all times (Fig. 3.6.5). Sitting grading (assessed at completed 8 months): • Grade 0: No sitting at all • Grade : Sits momentarily (Fig. 3.6.6) • Grade : Sits 30 seconds or more leaning forward (Fig. 3.6.7) • Grade : Sits with the child’s back straight (Fig. 3.6.8) • Grade : While sitting, can turn around and manipulate a toy (Fig. 3.6.9) • Grade : Raises self to sitting position (Fig. 3.6.10). Standing grading (assessed at completed 12 months): • Grade 0: Not standing well • Grade : Stands holding on to furniture momentarily (Fig. 3.6.11) • Grade : Take few steps with both hands supported (Fig. 3.6.12) • Grade : Can stand alone with legs apart (Fig. 3.6.13) • Grade : Come to standing position by throwing weight on arms (Fig. 3.6.14) • Grade : Without support takes few steps (Fig. 3.6.15). (Interpretation of CDC grading—grades III, IV, and V are normal for that age)

p

CDC Grading for Motor Milestones

 

Figure 3.6.2 Head holding grade II

B



V

IV

III

II

I

V

IV

III

II

I

V

 

Figure 3.6.1 Head holding grade I

irth to Two Years



Trivandrum Developmental Screening Chart (Fig 3.6.16 and Table 3.6.1) This is a simple developmental screening test for babies below 2 years that can be used in large scale community developmental screening programs by anganwadi workers and other health workers. The left end of each horizontal dark line represents the age at which 3% of children passed the item and the right end represents the age at which 97% of the children passed the item. A vertical line is drawn or a pencil is kept vertically, at the level of the chronological age of the child being tested. If the child fails to achieve any item

 

Figure 3.6.3 Head holding grade III

that falls short on the left side of the vertical line, the child is considered to have a developmental delay. Any obvious abnormality or asymmetry is also considered abnormal.

105

vip.persianss.ir

t

t

Tex book of Pedia rics

Figure 3.6.5 Head holding grade V

Figure 3.6.8 Sitting grade III

Figure 3.6.6 Sitting grade I

Figure 3.6.9 Sitting grade IV

   

 

 

106

 

Figure 3.6.7 Sitting grade II

 

Figure 3.6.4 Head holding grade IV

vip.persianss.ir

nt

p

D

n

t

Grow h a d evelo me

 

Figure 3.6.10 Sitting grade V

 

Figure 3.6.13 Standing grade III

 

Figure 3.6.11 Standing grade I

 

Figure 3.6.14 Standing grade IV

F

Two to our Years Developmental Assessment Tool for Anganwadis

­

­

­

Developmental assessment tool for anganwadis (DATA) is a short, psychometrically strong, norm-referenced develop mental scale with partial criterion referencing to identify toddlers at anganwadi who are at risk for developing developmental delays, and differentiate those who already have developed delays at 2.5 years for appropriate interventions. In addition, it is recommended that regular developmental assessments be conducted on the bene ficiaries of anganwadis every year at three more key ages of 3.5, 4.5 and 5.5 years to institute early intervention when 107 required.

 

Figure 3.6.12 Standing grade II

vip.persianss.ir

Table 3.6.1 Test items used in Trivandrum Developmental Screening Chart (Fig. 3.6.16) S. no. Test items

 

 

 

Assessment of a preschool child in a clinic setting by a developmental pediatrician or psychologist using DDST-II would help identify children with developmental delay. But in a borderline case what is more important is to understand the level of skill development in the child as compared to

 

S

F

Nursery Evaluation Scale Trivandrum—Abridged (Table 3.6.2)

other children of the same age (percentile position), so that appropriate item based interventions can be offered.

 

 

 

 

 

 

t

t

Tex book of Pedia rics

our to ix Years

 

Figure 3.6.15 Standing grade V

97% pass

Social smile

0.1

2.7

2.

Eyes follow pen/pencil

1.1

3.9

3.

Holds head steady

1.1

3.8

4.

Rolls from back to stomach

2.7

10.0

5.

Turns head to sound of bell/rattle

3.0

5.8

6.

Transfer object hand to hand

4.1

7.0

7.

Raises self to sitting position

5.8

11.0

8.

Standing up by furniture

6.3

11.0

9.

Fine prehension pellet

6.7

10.9

10.

Pat a cake

6.7

12.7

11.

Walk with help

7.7

13.0

12.

Throws ball

9.5

16.7

13.

Walk alone

9.9

17.4

14.

Says two words

11.2

19.1

15.

Walk backwards

11.2

19.5

16.

Walk upstairs with help

12.2

24.2

17.

Points to parts of doll

15.3

24.3

Figure 3.6.16 Trivandrum Developmental Screening Chart (TDSC) (Based on BSID Baroda norms) MKC Nair, Babu George, Elsie Philip. Indian Pediatr 1991;28:869-72  

108

3% pass

1.

vip.persianss.ir

3rd, 50th and 97th percentile age placements in months 3rd percentile

50th percentile

97th percentile

61 48 64 53

68 57 69 64

72 66 72 68

50 53 63 48 50 56

56 64 66 53 57 61

59 68 72 56 65 66

55 54 53 48 46 50 57 61

64 66 63 54 52 56 65 68

66 71 69 59 65 60 70 72

56 42

63 45

68 48

49 53

52 60

57 66

51 52 51 58

59 60 57 68

63 65 62 72

n

Heel to toe walk 8 steps Stands on one foot 10 seconds Walk on 20 cm elevated balanced beam Catches a soft ball with one hand

D







1. 2. 3. 4.











Gross motor development

p

Paints shape Tears simple shape Copies diamond shape Prints A E F Threads 10 beads in 1 minute Draw picture with three parts (excluding draw a man)

nt















Fine motor development 5. 6. 7. 8. 9. 10.

t

 

Items

Grow h a d evelo me

Table 3.6.2 Nursery Evaluation Scale Trivandrum (NEST)—Abridged















11. 12. 13. 14. 15. 16. 17. 18.



















Cognitive development Picks specific number of objects (5) Builds pyramid of 10 blocks Arranges objects according to width and length Puts numbers 1 to 10 in a sequence Names positions of objects Completes a simple maze Names days of a week in order Reads 10 printed words



19. Buttons and unbuttons dress 20. Washes hands and mouth when directed





Personal social development



21. Names function associated with three body parts 22. Tells materials out of which objects are made of





Expressive language development

Points to hard/soft/rough/smooth Points to middle Points to absurdities in a picture Puts together five parts picture

Nursery Evaluation Scale Trivandrum (NEST)—Abridged is such a simple tool that consists of skill-based items in the areas of gross motor, fine motor, cognitive, personal social, expressive language and receptive language, to give an overall assessment of the child.

Denver Developmental Screening Test II This instrument was designed to be a quick and simple screening tool to be used in clinical settings by persons with little training in developmental assessment. The test comprises of 125 items, divided into four categories; (1) gross motor, (2) fine motor/adaptive, (3) language and (4) personal social. The items are arranged in chronological order according to the ages at which most children pass them. The test items are represented on the form by a bar that spans the age at which 25%, 50%, 75% and 90% of the standardization sample passed that item. The child’s age is drawn as a vertical line on the chart and the examiner administers the items bisected by the line. The child’s performance is rated “pass”, “caution”, or

“delay” depending on where the age line is drawn across the bar. The total number of delays or cautions determines the rating of DDST as normal, questionable, or abnormal.

Developmental Assessment Scale for Indian Infants This is the gold standard test used for developmental evaluation, developed by Pramila Phatak, and is based on Bayley Scales of Infant Development (BSID). Developmental Assessment Scale for Indian Infants (DASII) consists of two scales, viz. mental scale and motor scale. The results of administration of mental scale are expressed as a standard score, the mental development index (MDI) and that of the motor scale as the psychomotor development index (PDI).

Neurological valuation E







23. 24. 25. 26.











Receptive language development

Although it is easy to diagnose a given case of cerebral palsy, when it comes to follow-up of high-risk babies, a 109 system of neurological examination that allows detection

vip.persianss.ir

Figure 3.6.18 Heel to ear angle  

110

A

F

A

D

The examiner holds the infant’s leg straight and flexes the foot toward the leg. This is accomplished by applying pressure with the thumb to the sole of the foot. The dorsiflexion angle is formed by the dorsum of the foot and the anterior aspect of the leg (Fig. 3.6.20).

carf ign

The infant is held in a semi-reclining position, supported by the examiner’s palm. At the same time, the examiner takes the infant’s hand and pulls the arm as far as possible, across the chest towards the opposite shoulder. Four

Figure 3.6.19 Popliteal angle  

 

Figure 3.6.17 Adductor angle

orsiflexion ngle of the oot

Figure 3.6.20 Dorsiflexion angle  

With the infant lying supine, the legs are held together and pressed as far as possible, towards the ear. The pelvis must not be lifted from the table. The angle is represented by the arc extending from the infant’s heel to the table. Increased resistance on one side is an indication of asymmetry, but it might be difficult to apply equal pressure to both sides (Fig. 3.6.18).

The thighs are flexed laterally at the hip along both sides of the abdomen. While holding the infant in this position, the examiner presses the lower leg as far as possible towards the thigh. The popliteal angle, which is formed by the calf and the thigh, is estimated in both legs simultaneously. In contrast to the maneuvers described above, it is easier to apply equal pressure to both sides when examining the popliteal angle. Therefore, the estimation of asymmetry is more objective. Significant asymmetry is indicated by a difference of 10–20° between the right and left angles (Fig. 3.6.19).

S

E

eel to ar

Popliteal ngle

S

A

A

dductor ngle

With the infant lying supine, the legs are extended and gently pulled as far apart as possible. The angle formed by the legs at this point is called the adductor angle. Asymmetry between the right and the left leg should be noted (Fig. 3.6.17). H

t

t

Tex book of Pedia rics

and recording of subtle neurological abnormalities that may disappear at the end of first year (called transient abnormalities) or persist even beyond that (persistent abnormalities) is needed. Amiel-Tison has provided us with a comprehensive system of neurological evaluation for the first five years of life that gives us a framework for instituting physical therapy program. In the Amiel-Tison method of neurological evaluation presence of hypotonia is identified by measuring the following angles (Table 3.6.1).

vip.persianss.ir

M

M

B

otor

ilestones

Grow h a d evelo me

Therapy ased on

Head Holding/Neck Control

•

•

 

Figure 3.6.21 Scarf sign

t n D

 

Table 3.6.3 Neurological assessment

p

•

Stimulating the child to hold the head by carrying the child in an upright position by supporting the infant’s head as and when possible. While playing and talking with the child, lift the child by supporting his upper arm and chest, thereby stimulating him to lift and hold his head. The child must be made to lie on his stomach and is guided on his elbow (a roll or round pillow can be used if necessary). Encourage the child to lift and hold his head by showing a colorful toy. Stimulate the child in prone position guiding on his hand on the surface, encourage the child to lift and hold his head and then rotate laterally showing a colorful toy.

nt

•

Sitting

Angles in degrees Angle

1–3 months

4–6 months

7–9 months

10–12 months

Adductor

40–80

70–110

100–150

130–150

Heel to ear

80–100

90–130

120–150

140–170

Popliteal

80–100

90–130

120–150

140–170

Dorsiflexion 45

45

45

45

Scar sign

Elbow cross midline

Elbow reaches axilla

Elbow beyond axilla

Elbow not cross midline

• • • •

D

positions are possible in describing the position of the elbow in relationship to the umbilicus (Fig. 3.6.21 and Table 3.6.3).

evelopmental Therapy

•

E

B

Therapy ased on Passive xercises

Encourage the child to sit by putting him in an arm chair in a sitting position supporting him with pillows, as and when possible. While playing and talking with the child, encourage the child in sitting position with a wide base (thighs apart), supporting at the pelvis with a downward force. During play, the child can be encouraged in side-sitting position on both sides by supporting himself on the hand to the side which he is sitting. Guide the child to support on his hand and knees (four point kneeling/quadruped position) during play. A roll or pillow can be used if necessary. Then slowly guide him to sit on one of his sides supported by the same hand. Help the child to maintain this position for a while. Then guide him again on to his hands and knees and then gradually to side sitting on the other side. Baby walker can also be used to stimulate and improve sitting.

Out of the items assessed in the Amiel-Tison method, the angles give an important clue for the therapy and stimulation part of intervention. A limitation in angles indicates hypertonia and wide angle indicates hypotonia in most cases. In such instances, stimulation becomes effective only after normalizing the muscle tone. The purpose of passive therapy is essentially to reduce these deformities by constant effort of the mother in a playful manner. Hometherapy based on Amiel-Tison passive angles is a simple concept which does not aim to hasten developmental milestones, but aims to prevent: • Mental subnormality by better mother-infant interaction • Joint stiffening by repeated passive movements • Contractures by repetitive passive movements • Muscle wasting and fibrosis • Helplessness in parents.

Standing •

• •

Guide the child on to his both knees during play. Finally support him at the pelvis. If necessary, give support to the upper part of his body. Gradually the support can be withdrawn and the child can be made to support himself by holding on to a low stool. This position can be maintained by directing the child’s attention to any play activity. From lying on the back position (supine position), stimulate the child to sit and gradually to the standing position during play time. Firstly, guide the child to his both knees supporting himself on a low stool with both hands. While directing his attention to a colorful toy through play, slowly help him to raise one leg so as to make him stand on one foot (leg straight), the other on the knee (half standing

111

vip.persianss.ir





1. Illingworth RS. The Development of the Infant and Young Child: Normal and Abnormal, 9th edition. Haryana: Reed Elsevier Private Limited; 2010.













2. Malhi P. Screening young children for delayed development. Indian Pediatr.1999;36:569-77. 3. Nair MKC, Babu G, Sabarinathan. CDC grading for head holding sitting and standing. Indian J Pract Pediatr. 1995;3(1):53-4. 4. Nair MKC, George B. Trivandrum Developmental Screening Chart (TDSC). Indian Pediatr. 1991;28:869-72. 5. Nair MKC, Jain N, Murkhi S, et al. The High Risk Newborn. New Delhi: Jaypee Brothers Medical Publishers (P) Ltd; 2007. 6. Nair MKC. Early stimulation CDC Trivandrum model. Indian J Pediatr. 1992;59:663-7. 7. Nair MKC. Editorial: simplified developmental assessment. Indian Pediatr. 1991;28:837-40. 8. Indian Pediatrics. (2009). Supplement Issue on Child Development. [Online] Indian Pediatrics website. Available from http://www.indianpediatrics.net/suppl2009/supple. htm [Accessed September 2012].



ibliography

B

t





t

Tex book of Pedia rics

•

position) help the child to maintain this position while playing and talking with him. This position can be repeated on other side. Meanwhile depending on the child’s ability, stimulate him to pull to standing position by himself, supporting on the stool. Encourage the child in standing position as and when possible, first with support then gradually withdrawing the support as per the child’s ability. A baby walker will also severe the purpose of developing standing and walking skills.

112

vip.persianss.ir

T

Failure to hrive





Growth and evelopment

3.7

Madhulika Kabra

tiology

The standard classification of dividing the causes of FTT as organic and non-organic is probably not very appropriate. Whether the condition is primarily organic or non-organic in origin, all children who fail to thrive suffer the physical

D

 

Table 3.7.1 Definition of failure to thrive Attained growth •  Weight < 3rd percentile on standard growth chart •  Weight for height < 5th percentile on standard growth chart •  Weight 20% or more below ideal weight for height Rate of growth •  •  •  • 

Less than 20 g/day from birth to 3 months of age Less than 15 g/day from 3 months to 6 months of age Falloff from previously established growth curve Downward crossing of > 2 major percentiles



and psychological consequences of malnutrition and are at a significant risk for long-term physical and psychodevelopmental sequelae. Table 3.7.2 summarizes the causes of FTT. Organic diseases are responsible for less than 20% of cases with FTT. It should be recognized that environmental  

Table 3.7.2 Causes of inadequate weight gain Inadequate intake and inappropriate feeding practices •  •  •  •  •  •  •  • 

Non-availability of food Misperceptions about diet and feeding practices Errors in formula reconstitution Dysfunctional parent-child interaction, child abuse and neglect Behavioral feeding problem Mechanical problems with sucking, swallowing and feeding Primary neurological diseases Chronic systemic disease resulting in anorexia, food refusal and neurological problems 

E



Failure to thrive (FTT) is a common and often perplexing concern for parents and pediatricians. Pathophysiologically, FTT is a state of caloric insufficiency without an apparent etiology. It is a descriptive term rather than a diagnosis and is used for children whose attained weight or rate of weight gain is significantly below their age, gender and ethnicity matched controls. Though primarily weight is affected, linear growth and head circumference may also get affected if the insult is prolonged and severe. By definition, FTT is sustained weight loss, failure to gain weight or a persistent fall in weight from the child’s normal centile (Fig. 3.7.1). This definition excludes transient weight loss associated with acute illness. Persistence of FTT may not only lead to long term complications related to physical growth but also development and behavior. There is lack of consensus about anthropometric criteria for FTT. Commonly used criteria are shown in Table 3.7.1. Important points to remember are: • Label of FTT should not be given, based on a single observation, i.e. failure to gain weight or weight loss should be observed over a period of time. • Usually children less than 3 years or maximum up to 5 years are included in this definition. • Small size alone is not an adequate criterion for confirming FTT, as constitutional and genetic factors may result in short stature.

Reduced absorption or digestion •  Pancreatic insufficiency—cystic fibrosis •  Damage to villus surface—celiac disease Excessive loss •  •  •  •  •  •  • 

Persistent vomiting Gastroesophageal reflux disease Gastrointestinal obstruction Increased intracranial pressure Renal losses—renal tubular acidosis Diabetes mellitus Inborn errors of metabolism

Increased caloric requirements •  •  •  •  • 

 

Figure 3.7.1 Failure to thrive: A: weight loss, B: static weight, C: Fall-off in weight gain

Congenital heart disease Chronic respiratory disease Neoplasm Hyperthyroidism Chronic or recurrent infection

Altered growth potential or regulation •  Chromosomal abnormalities •  Endocrinopathies

113

vip.persianss.ir

H

P

ntenatal, atal and erinatal istory N

A

Ideally, both parents should be present during the interview and parent-child and parent-parent interaction must be critically assessed.

Apart from the details of pregnancy, delivery and perinatal details, some points need special mention. • Was the child born of unplanned pregnancy? Did the parents consider medical termination of pregnancy? Children born of unplanned pregnancy tend to be emotionally deprived. • Was it a preterm delivery? If growth parameters are not corrected for gestational age, these children may be erroneously labeled as FTT. • Intrauterine growth retardation is another risk factor for FTT. Symmetrical IUGR children have a worse prognosis in this regard. • History suggestive of exposure to intrauterine infections. D

Growth ata

ietary istory H

D

Evaluation of growth pattern is the most important aspect of evaluation. Review child’s present and past growth parameters. This is only possible if parents have maintained a growth chart or previous growth status is known. In situations where previous record is not available, it is advisable to follow the child for weight gain.

M

E

etailed valuation of evelopmental ilestones hysical xamination E

P

These are provided in earlier chapters.

Physical examination should be thorough and complete. Detailed anthropometry should include length/height, weight, head circumference, upper/lower segment ratio, skinfold thickness and mid-arm circumference. • Routine and thorough general and systemic examination is a must, as it gives clues to do specific investigations. • Detailed neuro-developmental assessment should be performed. • Specific behavior patterns should be looked for. These may give a positive clue for non-organic FTT. These include unusual watchfulness, decreased vocalization, lack of cuddliness, head banging, rocking movements and rumination. • Signs of abuse and neglect. • Signs of vitamin and nutrient deficiencies should be looked for.

aboratory nvestigations

In most instances, a detailed general and systemic examination will help in ruling out an organic cause for FTT. Detailed laboratory investigations are indicated only if the history and physical examination suggest that an organic cause is responsible for FTT and to localize the systems involved. A battery of routine investigations should be avoided because they are unproductive in most instances, expensive, may be misleading and diverting attention. The following investigations are considered adequate for initial evaluation: • Complete blood count with erythrocyte sedimentation rate (ESR) • Urine and stool examination • Urine culture and sensitivity • Tuberculin test • Blood urea and serum creatinine • Specific tests: workup for inborn errors of metabolism, karyotyping, sweat test, celiac serology, endoscopy, etc. Radiological investigations are not routinely indicated, unless the child needs evaluation for tuberculosis or physical abuse. Determination of bone age may be required in some cases. More invasive diagnostic procedures are called for, when a specific diagnosis is suspected.

ocial and Family istory H

S



A detailed dietary history, both past and present should be elicited to evaluate caloric and protein intake.

D

D

istory

Lack of “support systems”: relatives and friends Financial constraints Psychiatric problems or drug abuse in family Marital problems and parental discord Serious illness or death in family.

I

H

History and physical examination are the mainstay for diagnosis of FTT. It should be emphasized that extensive laboratory investigations have no role in the diagnosis unless assessment suggests a probable organic cause and localizes the pathology to a particular system. Before labeling an infant as FTT, one should exclude normal variants of growth. These include infant with small parents, constitutional delay and prematurely born babies.

L

D

iagnosis

• • • • •



ediatrics T

extbook o

f

P

deprivation can coexist with and complicate organic FTT. For example, a child with cerebral palsy or multiple congenital malformations is likely to be environmentally deprived due to lack of care. Organic causes of FTT are most commonly gastrointestinal or neurologic.

A detailed social and family history provides useful clues for diagnosis of non-organic failure to thrive. Following factors need special evaluation:

114

vip.persianss.ir

D

 

Growth and evelopment

Flow chart 3.7.1 Schematic diagram showing evaluation of FTT

D



The major goals of management are nutritional rehabilitation, treating an organic cause if detected and addressing psychosocial and developmental issues involved. Individual management of these has been discussed in detail in subsequent chapters. The first decision that one has to take is, whether the child requires hospitalization or not. Indications for hospitalization are as follows: • Weight for height less than 70% of the median • Detailed evaluation for a suspected organic disorder • Suspected abuse or neglect • Non-response to outpatient management.

iet

An experienced dietician should always be involved in planning and supervising diet. Unless there is a strong suspicion of an organic cause, one should proceed directly for a two weeks trial feeding. Daily monitoring during this period is extremely important. Nutrition monitoring record includes daily weight and total calories consumed during last 24 hours against expected. Every effort should be made to feed the child orally. If oral feeding is inadequate, tube feeding may be tried for short periods.



Help of a child psychologist may be sought if indicated. Organization of a program of intensive environmental stimulation and affection is also needed. Every attempt should be made to see that parents are actively involved in the management. At the end of 2 weeks of trial feeding, the child is reassessed. A good intake during the feeding trial, and a good response in terms of weight gain, suggest that the primary problem was nutritional deprivation usually associated with emotional deprivation. Further management of a child who fails to respond to the feeding trial, is shown in Flow chart 3.7.1. It is perfectly justified to undertake detailed investigations in the child with FTT who fails to respond to a two weeks trial feeding. However, by this time, the physician usually has an idea of the diagnostic possibilities, and he/she can tailor the investigations accordingly.

anagement

P

M

Abbreviations: GI, Gastrointestinal; GER, Gastroesophageal reflux; RTA, Renal tubular acidosis; CRF, Chronic renal failure; CNS , Central nervous system

rognosis

Most children with FTT have good growth recovery in all domain provided optimal care is taken for nutritional supplementation and emotional support. The overall prognosis for intellectual and behavioral recovery is variable and less certain.

115

vip.persianss.ir





















1. Brgman P, Graham J. An approach to failure to thrive. Aus Fam Phy. 2005;34:725-9. 2. Hank DA, Jeisel SH. Failure to thrive. Pediatr Clin North Am. 1998;35:1187-205. 3. Maggioni A, Lifshitz F. Nutritional management of failure to thrive. Pediatr Clin North Am. 1995;42:791-810.

4. Marcovitch H. Failure to thrive. Br Med J. 1994;108:35. 5. Overby KJ. Failure to thrive. In: Rudolph AM (Ed), Rudolph’s Pediatrics, 20th edition. Stamford, Connecticut: Appleton and Lange; 1996.pp.3-9. 6. Suri M, Kabra M, Aggarwal A, et al. Failure to thrive. Indian J Pediatr. 1994;8. 7. The American Board of Pediatrics. Program for renewal of certification in pediatrics. Failure of Thrive. Supplement to Pediatrics in Review 1993.

B

ediatrics P f

extbook o T

ibliography

DISCLAIMER: The views expressed by the individual authors about the Growth Curves are their own and do not reflect the official position/stand of the Editors or Indian Academy of Pediatrics.

116

vip.persianss.ir

Section

4 Nutrition

P

Breastfeeding: Jp Dadhich Infant and Young Child Feeding: RK Anand Malnutrition: Meenakshi Mehta Water Soluble Vitamins: Dheeraj Shah Fat Soluble Vitamins: anna Choudhury Trace Elements: Ke lizabeth E



4.1 4.2 4.3 4.4 4.5 4.6















Section Editor : Dheeraj Shah

vip.persianss.ir

ics

Breastfeeding





t

ook o P d a r

4.1

R

efinitions elated to Breastfeeding

Child health and nutrition programs all across the world (including India) conform to these guidelines based on the global recommendations. These recommendations are based on the available scientific evidence, some of which are defined as follows:

Initiation of Breastfeeding Immediately after Birth, Preferably within ne Hour Early initiation has been documented to improve neonatal survival, and protective against the infection specific mortality among newborn infants. Early initiation of breastfeeding helps to develop a bond between a mother and her baby. Early initiation is extremely important to establish successful and sustained lactation. It stimulates contractions and expulsion of placenta. The practice of delaying breastfeeding after birth and giving something else, i.e. prelacteal feeds expose the infant to infections and also lead to problems in establishing a successful lactation. Scientific evidence suggests that early is the initiation of breastfeeding, more are the chances of survival of neonate. After cesarean section, some delay in initiation of breastfeeding may be unavoidable due to the condition of the mother or infant. After cesarean section with spinal anesthesia, breastfeeding can often be initiated immediately. With general anesthesia, breastfeeding can be initiated within a few hours as soon as the mother regains consciousness.

Various definitions used in context of breastfeeding are summarized in the Table 4.1.1.

ecommendations for Breastfeeding

O

Optimal infant and young child feeding (IYCF) practices are critical for child nutrition and survival. Breastfeeding is a vital component of IYCF. The “Global Strategy for Infant and Young Child Feeding” states that “breastfeeding is an unequalled way of providing ideal food for the healthy growth and development of infants; it is also an integral part of the reproductive process with important implications for the health of mothers. As a global public health recommendation, infants should be exclusively breastfed for the first 6 months of life to achieve optimal growth, development and health. Thereafter, to meet their evolving nutritional requirements, infants should receive nutritionally adequate and safe complementary foods while breastfeeding continues for up to 2 years of age or beyond”. Presently, when global efforts are on to achieve better nutritional status and survival for children enshrined in the Millennium Development Goals (MDGs), breastfeeding emerges as a very effective intervention to achieve these targets.

R

R

”The provision has been made for infants to be fed upon their mother’s milk. They find their food and their mother at the same time. It is complete nourishment for them, body and soul. It is their first introduction to the great truth that man’s true relationship with the world is that of personal love and not that of the mechanical law of causation”. abindranath Tagore

D

e

T x

tb

f

e

P

i

J Dadhich

Table 4.1.1 Definitions related to infant feeding

Exclusive Breastfeeding for the First Six Months Exclusive breastfeeding is recommended as breast milk contains all the necessary nutrients which are sufficient to sustain appropriate growth and development of a healthy term infant for the first 6 months of life. There is sufficient evidence that a significant number of under-five-month deaths in resource poor countries could be prevented through achievement of 90% coverage with exclusive breastfeeding for 6 months. Any supplementation during the first 6 months will expose infant to infections and also decrease breast milk output.

 















•  Exclusive breastfeeding: Giving a baby no other food or drink, including water, in addition to breastfeeding with the exception of syrup/drops of vitamins, minerals and medicines (expressed breast milk is also permitted) •  Predominant breastfeeding: Giving small amounts of water or water-based drinks such as tea in addition to breastfeeding •  Partial breastfeeding: Giving a baby some breastfeeds and some artificial feeds, either milk or cereal, or other food •  Bottle feeding: Feeding a baby from a bottle, whatever is in the bottle, including expressed breast milk •  Cup feeding: Feeding a baby from cup (katori, pallad, etc.) whatever is in the cup including breast milk •  Artificial feeding: Feeding a baby on any kind of artificial milk such as animal milk, tinned milk, etc. and not breastfeeding at all •  Complementary feeding: Giving other foods and liquids in addition to breast milk or nonhuman milk • Replacement feeding: Process of feeding a child who is receiving no breast milk with a diet that provides all nutrients the infants need until the age at which they can be fully fed on family foods

A

A

A

ppropriate and dequate Complementary Feeding after Six Months of ge while Continuing Breastfeeding

118

Source: Adapted from Infant and young child feeding counseling—a training course, the “4 in 1 course” (integrated course on breastfeeding, complementary feeding and infant feeding and HIV). Breastfeeding Promotion Network of India (BPNI), Delhi.

Additional foods are needed at this stage to complement the breast milk to sustain the growth and development of the infant. Along with the breastfeeding, children age 6–24 months

vip.persianss.ir

t iti

The breast milk contains all the macronutrients (carbo hydrates, proteins and fats), micronutrients, like vitamins and minerals, and adequate water to meet the requirements of a healthy term infant for the first 6 months of life. Apart from the nutrients, breast milk provides a variety of bioactive factors which protect the infant against infection, and also modulate the composition of the indigenous intestinal microbiota. Breast milk also contains some factors to help in digestion and absorption of nutrients.

Status of Breastfeeding Practices in India

Fats

The status of breastfeeding and complementary feeding practices is very dismal in India. According to the National Family Health Survey-3 (NFHS-3), only 24.5% of children are breastfed within the first hour of birth and about 50% initiate breastfeeding within first day of life. More than half of newborn infants receive prelacteal feeds, like milk other than breast milk, honey, sugar or glucose water, and plain water. The exclusive breastfeeding rate up to the age of 6 months is only 46.3%. Exclusive breastfeeding rapidly declines from first month to sixth month, and only about 20% children continue it by 6 months. Possible reasons for suboptimal breastfeeding are primarily due to lack of proper information to mothers, inadequate health care support, inability of the health care providers to help mothers experiencing breastfeeding difficulty, aggressive promotion of baby foods by the

The mature human breast milk contains 3.2–3.8 g/dL of fats. Fats provide 50% of the total energy content of the breast milk. Breast milk fat in immediate postpartum period contains fat needed for gray matter development and in later months, fat which is needed for myelination. Breast milk fat has steady higher level of cholesterol than animal milks and formula. Breastfed babies have significantly higher total cholesterol and low-density lipoprotein (LDL) cholesterol compared to mixed fed babies in the first 6 months of life with improving high-density lipoprotein (HDL) cholesterol/ LDL cholesterol ratio at 6 months. High cholesterol intake in infancy may have a beneficial long-term programming effect on synthesis of cholesterol by downregulation of hepatic enzymes. Human milk contains essential fatty acids and n-3 fatty acids (docosahexaenoic acid and eicosapentaenoic acid), which are needed for a baby’s growing brain and eyes and for healthy blood vessels. Human milk contains the enzyme lipase which helps to digest fat. Thus the fat in breast milk is more completely digested and more efficiently used by a baby’s body than the fat in cow’s milk or formula. The lipase in breast milk is called bile salt stimulated lipase because it starts working in the intestine in the presence of bile salts. The lipase is not active in the breast, or in the stomach before the milk mixes with bile.



n

Nutritional Composition of Breast Milk ­

A

Breastfeeding along with other foods remains an important and safe source of high quality protein, energy and other nutrients like vitamin A and vitamin C between 6 months and 24 months of life. It is, therefore, crucial in preventing undernutrition and morbidities. It can provide about onethird of energy needs, half of protein and 75% of the vitamin A requirements of a child of this age (Fig. 4.1.1). Thus, breast milk helps a child to get enough energy and high quality nutrients from breastfeeding during the second year of life. These nutrients may not be easily available from the family diet. Continuing to breastfeed during the second year can help to prevent malnutrition and vitamin deficiencies.

o

Continued Breastfeeding up to the ge of Two Years or Beyond

commercial industry and lack of proper support structures at the community and at work place which includes maternity entitlements and crèches. Cultural beliefs also appear to be important, e.g. breastfeeding initiation is delayed because of the belief that mother’s milk does not “come” at the time of childbirth but flows 2–3 days later.

Nu r

should be fed from three or more different food groups; two to three times a day (see more details in chapter 4.1).

Carbohydrates

 

Figure 4.1.1 Nutrition provided by breast milk in second year of life. reproduced with permission from “Infant and Young Child Feeding Counseling—a training course, the ‘4 in 1 course’ (integrated course on breastfeeding, complementary feeding and infant feeding and HIV). Breastfeeding Promotion Network of India (BPNI), Delhi”

Lactose is the main carbohydrate in human breast milk and provides about 50% of its energy content. Breast milk also contains oligosaccharides such as glucose, galactose, N-acetylglucosamine and sialic acid. These oligosaccharides attaches to the epithelial cell surface in the intestines and prevent adhesion of microorganisms thereby preventing their growth.

Proteins Proteins in breast milk provide amino acids for growth and anti-infective factors. Mature breast milk contain 0.9 g/dL of 119

vip.persianss.ir

The composition of breast milk is not always the same. It varies according to the age of the baby, and from the beginning to the end of a feed. It also varies between feeds, and may be different at different times of the day.

Colostrum The milk produced during the first few days after the delivery is known as colostrum, which is a special, thick, sticky, bright lemony yellowish fluid. It is secreted in small quantities for first 3–4 days of life. Although it is in small quantities, it is sufficient to meet the needs of the newborn baby. Colostrum contains more protein than later milk. Colostrum is considered the first immunization for newborn as it is rich in the anti-infective factors that helps protect the baby against diarrhea, respiratory and other infections. Colostrum contains more epidermal growth factors in comparison to mature breast milk, which help a baby’s immature intestine to develop after birth. This helps to prevent the baby from developing allergies and intolerance to other foods. Colostrum helps to clean baby’s intestine which is important to prevent jaundice in the newborn. Colostrum is also rich in vitamin A.

Transitional Milk During the transition from colostrum to the mature milk, the amount of immunoglobulin, proteins, vitamin A and vitamin E decreases, and amount of lactose, fats, energy and water-soluble vitamins increases.

Mature milk

The benefits of breastfeeding for infant, mother and community include: • Breastfeeding provides all the nutrients a baby needs for the first 6 months of life, after which it continues to provide a major portion of the infant’s nutrition along with appropriate family foods. It provides almost half of the nutritional requirements between 6 months and 12 months of age, and up to one-third between 12 months and 24 months of age • Breast milk is easily digested by the baby • Breast milk contains antibodies and other factors which protect the baby against diarrhea and other infections • Breast milk contains enough water which is sufficient even for very dry and hot climates • Breast milk is clean, safe and cheap • Breastfeeding provides a perfect opportunity for building a close bond between mother and baby • It helps the mother by reducing the postdelivery bleeding and thus preventing anemia • Breastfed babies are less prone to have diabetes, heart disease, eczema, asthma, rheumatoid arthritis and other allergic disorders later on in life • Breastfeeding enhances brain development, visual development and visual acuity leading to learning readiness • Breastfeeding has contraceptive effect for the mother if she exclusively breastfeeds her infant for first 6 months • Mothers have a lower risk of breast and ovarian cancers • Breastfeeding costs less in terms of health care expenses as breastfed infants get ill less often • Breastfeeding protects the environment.

isks of Formula Feeding

Infant formula, which is generally used as an artificial substitute for human breast milk, is time consuming, less nutritious and expensive. It is also fraught with innumerable risks for the infants and children in comparison with the breastfeeding. Some of these risks are depicted in the Table 4.1.2.

Science of Milk Transfer Understanding the structure of breast and the process of breast milk production and transfer to the infant is useful to provide effective skilled help to the lactating mother.

natomy of the Breast



After a few days, colostrum changes into mature milk. Mature milk is in large amounts and the breasts feel full, hard and heavy. Some people call this the milk “coming in”. Foremilk is the bluish milk that is produced early in a feed. Foremilk is produced in larger amounts, and it provides plenty of protein, lactose and other nutrients. Because a baby gets large amounts of foremilk, he or she 120 gets all the water that he or she needs from it. Hindmilk is the whiter milk that is produced later in a feed. It contains

Benefits of Breastfeeding

R

ynamic Composition of Breast Milk

more fat than foremilk. This fat provides much of the energy of a breastfeed. This is why it is important not to take a baby off a breast too quickly, not until he or she leaves the breast on her/his own.

A

D



ics f

e

i

t

ook o P d a r tb e

T x

protein while colostrum contains 2.3 g/dL. The breast milk protein contains more whey protein and less casein. Due to high whey to casein ratio, the breast milk forms softer curds which are easier to digest. In human milk, much of the whey protein consists of anti-infective proteins, which help to protect a baby against infection. The anti-infective proteins in human milk include lactoferrin (which binds iron and prevents the growth of bacteria which need iron) and lysozyme (which kills bacteria) as well as antibodies (immunoglobulin, mostly IgA). Animal milk and formula may lack the amino acid cystine, and formula may lack taurine which newborns need especially for brain growth. Human infant is ill equipped to handle phenylalanine and tyrosine, two amino acids which are in high concentration in the animal milk in comparison to breast milk.

The human breast consists of the nipple and areola, mammary tissue, the soft tissue including supporting connective tissue and fat, blood and lymphatic vessels and nerves (Fig. 4.1.2). The nipple is the area from which the milk comes out of the breast through multiple small

vip.persianss.ir

t

P

iti

Production of the breast milk is controlled by the hormone prolactin. When a baby suckles at the breast, sensory impulses go from the nipple to the brain. In response, the anterior part of the pituitary gland at the base of the brain secretes prolactin. Prolactin goes in the blood to the breast and makes the milk secreting cells produce milk. This process is known as the prolactin reflex (Fig. 4.1.3). This is evident that milk production is dependent on the suckling stimulus. If the baby suckles more, the breast will produce more milk. For the same reason if a mother has two babies, breast milk production increases accordingly. Prolactin is present in the blood for about 30 minutes after the baby finishes the feed. It makes the breast produce milk for the next feed. More prolactin is produced at night due to the inhibition of dopaminergic drive during sleep so breastfeeding at night is especially helpful for keeping up the milk supply. Prolactin suppresses ovulation so breastfeeding can help to delay a new pregnancy.

n

 



    

 

roduction of Breast Milk

o

•  Increased risk for infection from inherent and subsequent contamination of formula with microbes like Enterobacter sakazakii and Salmonella •  Increased risk of acute respiratory infections, diarrhea, otitis media and ear infections •  Increased risk of necrotizing enterocolitis •  Increased risk of asthma and other allergies •  Reduced cognitive development •  Increased risk of chronic diseases like type 1 diabetes, ulcerative colitis and Crohn’s disease •  Increased risk of cardiovascular disease, increased blood pressure, obesity, altered blood cholesterol levels and atherosclerosis in later adulthood •  Increased risk of side effects of environmental contaminants and harmful chemicals like melamine and bisphenol A (BPA)

Physiology of Lactation

Nu r

Table 4.1.2 Risks of formula feeding

Flow of Breast Milk When a baby suckles, sensory impulses go from the nipple to the brain. In response, the posterior part of the pituitary gland at the base of the brain secretes the hormone oxytocin. Oxytocin goes in the blood to the breast and makes the muscle cells around the alveoli contract. This makes the milk which has collected in the alveoli flow along the ducts toward nipple. It makes the milk in the breast flow for this feed (Fig. 4.1.4). Sometimes the milk is ejected in fine streams. This is

 

Figure 4.1.2 Anatomy of the breast. (reproduced with permission from World Health Organization. Infant and Young Child Feeding: Model Chapter for Textbooks for Medical Students and Allied Health Professionals. Geneva: WHO Press; 2009)

Figure 4.1.3 Prolactin reflex. (reproduced with permission from World Health Organization. Infant and Young Child Feeding: Model Chapter for Textbooks for Medical Students and Allied Health Professionals. Geneva: WHO Press; 2009)  

openings. This area of breast is very richly supplied with nerves. The nerve endings in the nipple are important to provide stimulus for the hormonal reflexes important for production and release of the milk from breast. The areola is the dark skin surrounding the nipple. The milk ducts beneath the areola are filled with milk and become wider during a feed. Areola is an important anatomical landmark as it is important to ensure that majority of areola is in baby’s mouth during the feed to achieve an effective suckling. The mammary tissue is composed of alveoli, which are small sacs, made up of millions of milk secreting cells. Milk produced in the alveoli is carried toward the nipple via tubular structures called ducts. These ducts open outside at the nipple area. The alveoli are surrounded by myoepithelial tissue which helps in pushing the milk present in the alveoli toward nipple.

121

vip.persianss.ir

A

ics

Positioning and ttaching the Baby at the Breast

f

P

e

i

t

ook o P d a r

For effective milk transfer from mother to the infant, good breastfeeding skills including proper positioning of the baby and good attachment at the breast are required.

ositioning

 



the oxytocin reflex or the milk ejection reflex. Oxytocin can start working before a baby suckles, when a mother expects a feed. The oxytocin reflex is positively affected by mother’s sensations and feelings like thinking lovingly about the baby; touching, smelling or seeing the baby; or hearing the baby cry. If the oxytocin reflex does not work well, the baby may have difficulty in getting the milk. This may happen if the mother is emotionally disturbed or experiencing pain and discomfort. In such a condition, mother needs support to make her physically and/or emotionally comfortable to make the oxytocin reflex work again and let the milk flow. Signs of an active oxytocin reflex are a tingling sensation in the breast before or during a feed, milk flowing from breasts when mother thinks of the baby or hears him/her crying, milk flowing from the other breast when the baby is suckling, milk flowing from the breast in streams if suckling is interrupted, and uterine pain or a flow of blood from the uterus during the feed. However, absence of these signs does not indicate an inadequate oxytocin reflex.

The mother should be explained how to support the breast with her hand while offering it to the baby: • With her fingers and palm placed on her chest wall below the breast so that her first finger forms a support at the base of the breast • With her thumb pressing on the top of the breast so that it is easier for her baby to attach well.

Figure 4.1.4 Oxytocin reflex. (reproduced with permission from World Health Organization. Infant and Young Child Feeding: Model Chapter for Textbooks for Medical Students and Allied Health Professionals. Geneva: WHO Press; 2009)

The mother should be explained how to bring the baby to the breast: • Touch baby’s lips with her nipple • Wait until baby’s mouth is wide open • Move the baby quickly onto the breast from below.

e

T x

tb

A woman can feed her baby in any comfortable position such as sitting, lying or even standing. If the baby suckles properly from the breast he or she will get sufficient milk. However, for a good attachment on breast, some basic principle need to be observed for relative positioning of the baby while breastfeeding. These are: • Baby turned towards mother and his or her ears, shoulder and buttock are in a straight line • His face should face the breast with nose opposite the nipple • Mother should hold the baby close to her • In a newborn, she should support his bottom with hand and not just his head and shoulders.

Attachment This is important how baby’s mouth is attached to mother’s breast for a successful suckling (Figs 4.1.5 and 4.1.6). In good suckling position, baby is suckling with nipple and a larger breast tissue having in his or her mouth. In poor suckling position, baby is suckling with nipple only or nipple with a small breast tissue in his or her mouth.

Signs of good suckling attachment are: • •

The baby’s chin touches the breast His mouth is wide open

Breast Milk Inhibitor

Figure 4.1.5 Good and poor attachment. External signs. (reproduced with permission from World Health Organization. Infant and Young Child Feeding: Model Chapter for Textbooks for Medical Students and Allied Health Professionals. Geneva: WHO Press; 2009)  

Breast milk production is also controlled within the breast itself. Sometimes one breast stops making milk, while the other breast continues to make milk although oxytocin and prolactin go equally to both breasts. There is a substance in breast milk which can reduce or inhibit milk production. If a lot of milk is left in a breast, the inhibitor stops the cells from secreting any more. If breast milk is removed by suckling 122 or expression, the inhibitor is also removed and the breast makes more milk.

vip.persianss.ir

Nu r

Practices for Successful Breastfeeding To ensure adequate milk production and flow for 6 months of exclusive breastfeeding and thereafter continued breastfeeding, certain practices are very important. • The infant should be fed as frequently and for as long as he or she wants to, during both day and night. The suckling should be allowed until the infant spontaneously releases the nipple. This is called demand feeding. Restricting length of the breastfeeding session may result in the baby getting less of the energy rich hindmilk. The 24-hour average intake of milk is about 800 mL per day during the first 6 months. • At the time of delivery, before breastfeeding is initiated, no prelacteal feed should be given to the infant. Apart from having the harmful effects on the infant like risk of infection, such a practice may interfere in the establishment of breastfeeding. Later on, in the first 6 months of life, no supplementary feed, like other milks, should be given to the infant. This may lead to a decreased supply of breast milk. • Sometimes, mother may have the perception that her milk is not sufficient for her infant. Adequacy of breastfeeding may be ascertained by documenting if the infant has regained the birth weight by 2 weeks of age, and the cumulative weight gain is more than 500 g in a month and the infant is passing adequate urine at least six times a day, while on the exclusive breastfeeding.

iti n

Components of Baby-Friendly Hospital Initiative A maternity facility can be designated “baby-friendly” when it has implemented ten steps given in the Table 4.1.3 to support successful breastfeeding.

Breastfeeding the Preterm Babies The nutritional management plays a large role in the immediate survival and subsequent growth, and develop ment of the preterm infants. The optimal diet for premature infants should support growth at intrauterine rates without imposing stress on the infant’s immature metabolic and excretory functions and ensures healthy short-term and ­

His lower lip is turned outwards One can see more of the areola above his or her mouth and less below. This shows that he or she is reaching with his tongue under the lactiferous sinuses to press out the milk. Poor attachment may lead to pain and damage to mother’s nipple and she may develop sores or fissures in nipple. It may also lead to engorgement of the breast due to improper milk removal. The baby remains hungry and frustrated that leads to refusal to suck. Ultimately, it leads to production of less milk in the breast; baby is not able to feed properly, leading to weight loss. Common causes of poor attachment are use of feeding bottle, inexperience of mother and lack of skilled support.

• •

Table 4.1.3 Ten steps of baby-friendly hospital initiative  

 

Figures 4.1.6A and B (A) Good attachment and (B) poor attachment inside the infant’s mouth. (reproduced with permission from World Health Organization. Infant and Young Child Feeding: Model Chapter for Textbooks for Medical Students and Allied Health Professionals”. Geneva: WHO Press; 2009)



B

Maternity homes and health care practices should support exclusive breastfeeding during the first 6 months of life and continued breastfeeding along with appropriate complementary feeds thereafter. To ensure successful breastfeeding, the World Health Organization (WHO) and the United Nations Children’s Fund (UNICEF) launched the Baby-Friendly Hospital Initiative (BFHI) in 1991. The initiative is a global effort for improving the role of maternity services to enable mothers to breastfeed babies for the best start in life. It aims at improving the care of pregnant women, mothers and newborns at health facilities that provide maternity services for protecting, promoting and supporting breastfeeding. Since its launching, BFHI has grown with more than 20,000 designated facilities in 152 countries around the world over the last 15 years. The initiative has measurable and proven impact, increasing the likelihood of babies being exclusively breastfed for the first 6 months.

o

a

t

Hospital Practices and Breastfeeding

1. Have a written breastfeeding policy that is routinely communicated to all health care staff. 2. Train all health care staff in skills necessary to implement this policy. 3. Inform all pregnant women about the benefits and management of breastfeeding. 4. Help mothers initiate breastfeeding within a half-hour of birth. 5. Show mothers how to breastfeed and maintain lactation even if they should be separated from their infants. 6. Give newborn infants no food or drink other than breast milk unless medically indicated. 7. Practice “rooming in”—allow mothers and infants to remain together 24 hours a day. 8. Encourage breastfeeding on demand. 9. Give no artificial teats or pacifiers (also called dummies or soothers) to breastfeeding infants. 10. Foster the establishment of breastfeeding support groups and refer mothers to them on discharge from the hospital or clinic.

123

vip.persianss.ir



condition is known as inverted nipple (Fig. 4.1.7). The mother needs support in such a situation. She should be reassured that with some help she will be able to breastfeed her infant successfully. Help is most important soon after delivery when the baby starts breastfeeding. A mother with the inverted nipple may be helped with the syringe method as follows (Fig. 4.1.8): • Cut the nozzle end of a disposable syringe (10–20 mL). • Introduce the piston from the ragged cut end side. • Ask the mother to apply the smooth side of the syringe on the nipple and gently pull out the piston and let her wait for a minute. • Nipple would then protrude into the syringe. Ask the mother to slowly release the suction and put the baby to breast; at this time it helps the nipple to erect out and baby is able to suckle in the proper position.

ics f

e

i

t

ook o P d a r tb e

T x

long-term outcomes. Breast milk produced during early postpartum period offers nutritional advantage because of its higher protein and electrolyte concentrations. Preterm infant fed preterm milk demonstrate increase in weight, length and head circumference as well as retention rates of various nutrients comparable to those for the fetus of similar postconception age. Fat absorption in preterm babies fed their own mother’s milk is significantly higher in comparison to infants fed cow’s milk formula. Long-chain polyunsaturated fatty acids (LCPUFAs), which are important for mental and visual development, are also higher in human milk. Preterm infants fed breast milk have lesser incidence of necrotizing enterocolitis in comparison to feeding with formula milk. Even if the disease occurs in infants fed with breast milk, the course of disease is less severe and the prevalence of intestinal perforation is lower. This is due to various protective factors in breast milk like immunoglobulin, erythropoietin, interleukin-10 (IL-10), epidermal growth factor, platelet activating factor, acetylhydrolase and oligosaccharides which are in greater quantity than in term milk. These factors may prevent intestinal attachment of enteropathogens by acting as receptor homologues resulting in the suppression of enteral colonization with harmful microorganisms. Breast milk also prevents a host of neonatal infections, a leading cause of neonatal mortality across the globe. Use of human milk can be adopted as an important health care intervention in neonatal units. D

Breast Conditions and ifficulties In Breastfeeding Figure 4.1.7 Flat and inverted nipple. (adapted with permission from Gupta A, Kushwaha KP, Sobti JC, Jindal T (Eds). Breastfeeding and Complementary Feeding. Delhi: BPNI; 2001)  

­

There are several common breast conditions which sometimes cause difficulties with breastfeeding. Manage ment of these conditions is important both to relieve the mother and to enable successful breastfeeding. The difficulties in breastfeeding can be overcome by careful guidance, reassurance and encouragement to the mother during antenatal period to prepare for breastfeeding and by providing skilled counseling after birth.

Flat Nipple

Figure 4.1.8 Syringe method for correction of inverted nipple (adapted from Infant and Young Child Feeding Counseling—a training course, the “4 in 1 course” (integrated course on breastfeeding, complementary feeding and infant feeding, and HIV). Breastfeeding Promotion Network of India (BPNI), Delhi  

Many a times, mother becomes apprehensive that a flat nipple is a hindrance in successful breastfeeding. However, in a good suckling attachment, the infant takes the nipple and the breast tissue underlying the areola into his mouth to form a “teat”. The anatomical nipple only forms about onethird of the “teat” of breast tissue in the baby’s mouth. This is therefore evident that shape of the nipple is immaterial for successful suckling. The nipple is just a guide to show where the baby has to take the breast. A woman with flat nipples should be reassured that she has normal nipples even if they look short provided her nipples protract easily.

Inverted Nipple

124

Sometimes a nipple does not protract and on attempting to pull out the nipple, it goes deeper into the breast. The

vip.persianss.ir



Engorgement of the breast can be prevented by avoiding factors mentioned above. If the baby is able to suckle, he or she should feed frequently. If pain and tightness of the breast does not allow suckling, expressed milk may be given to the infant with cup/spoon. Once the mother feels comfortable, she should be advised again to breastfeed the infant on demand. Edema of the breasts may be reduced by applying cold compress. Engorged breasts may cause mild fever, which subsides spontaneously within a day or two. A

Mastitis and bscess Mastitis is inflammation of the breast which becomes red, hot, tender and swollen. The mother feels sick, has fever and severe pain in breast. Mastitis usually affects a part of the breast and usually unilateral. Mastitis may develop in an engorged breast, or it may follow a condition called blocked duct. Mastitis must be treated promptly and adequately. If treatment is delayed or incomplete, there is an increased risk of developing breast abscess. An abscess is when a collection of pus forms in part of the breast. The most important part of treatment is supportive counseling and improved drainage of milk from the affected part of the breast. The mother needs clear information and guidance about all measures needed for treatment, how to continue breastfeeding or expressing milk from the affected breast. This is important to help the mother to improve infant’s attachment at the breast with frequent unrestricted breastfeeding. If necessary express breast milk by hand or with a pump until suckling is resumed. Antibiotic should be given if laboratory tests indicate infection, symptoms are severe, or symptoms do not improve after 12–24 hours of improved milk removal. Pain should be treated with an analgesic and warm packs to the breast. Incision and drainage should be done if abscess develops.

Sore and Cracked Nipples The most common cause of sore nipples is poor attachment in which the infant pulls the nipple in and out as he or she sucks and rubs the skin of the breast against his or her mouth. If the baby continues to suckle in this way, it damages the nipple skin and causes a crack or fissure. Oral thrush in the infant’s mouth is another important cause of

t iti n

Breastfeeding and Maternal Illness Maternal illnesses can have adverse effects on lactation. A sick woman may perceive that her milk supply has gone down because of illness. She may also believe that her milk will make the baby ill. These factors may lead to discontinuation of breastfeeding. Minor illnesses such as cold and other mild viral infection, which are self-limiting, should not prevent a mother to continue breastfeeding. However, major illness requires a more careful approach. The potential role of breastfeeding in the transmission of infections must also be acknowledged and appropriate precautions should be taken. If the mother has tuberculosis, the mother-infant dyad should be treated together and breastfeeding should be continued. Similarly, in case of hepatitis (A, B and C) breastfeeding can continue normally as the risk of transmission by breastfeeding is very low. In HIV-positive mother, mother should be provided with counseling and support for appropriate infant feeding practice. With adequate and appropriate antiretroviral drugs to mother and infant, exclusive breastfeeding for first 6 months of life is now preferred recommendation in India. Certain maternal drugs may affect the breastfed infant adversely as they are secreted in the breast milk. Breastfeeding should be avoided if mother is consuming cytotoxic drugs, like cyclophosphamide, methotrexate and doxorubicin, radioactive compounds like gallium 67 (67Ga), indium 111 (111In), iodine 131 (131I) and technetium 99m (99mTc).

Infant Feeding uring Emergencies D

If breasts are not emptied, the milk gets collected in the breast leading to engorgement. The engorged breast is tight, shiny (because of edema) and painful. Also, the milk may stop flowing. The factors which cause engorgement of breasts are: • Giving prelacteal feeds to the baby • Delayed initiation of breastfeeds • Long intervals between feeds • Early removal of the baby from the breast • Bottle-feeding and any other restrictions on breastfeeding.

o

Engorgement of Breasts

sore nipple but it usually develops when a baby is few weeks old. The situation is very painful for the mother. If a mother has sore or cracked nipples, improving infant’s attachment to the breast relieves the pain. Medicated creams are best avoided as they may worsen the soreness. Hindmilk, which is rich in fat, should be applied on the nipple after feeding. For oral thrush 1% gentian violet should be applied over the nipple as well as inside the baby’s mouth.



After feeding the nipple may retract back, but doing it each time before feeding over a period of few days will help to solve the problem.

Nu r

•

In disasters and emergencies like earthquakes, floods, typhoons and tsunami, breastfeeding is the safest, often the only reliable choice for infants and young children. It provides adequate and appropriate nutrition to the affected infants in a situation where child survival is a key issue. In disasters, infants are more likely to become ill and die from malnutrition. Uncontrolled distribution of breastmilk substitutes during disasters may lead to early and unnecessary cessation of breastfeeding. For the vast majority of infants, emphasis should be on protecting, promoting and supporting breastfeeding and ensuring timely, safe and appropriate complementary feeding.

Protecting Breastfeeding from Commercial Influence During last many decades, extensive promotion by the infant 125 food manufacturing companies through advertisements,

vip.persianss.ir

•

meeting, conference, educational course, contests, fellowship, research work or sponsorship, etc. by the manufacturers, supplier or distributors of the products mentioned above. It prescribes standards for the infant milk substitute, infant foods and feeding bottles.



















1. American Academy of Pediatrics. Section on breastfeeding: breastfeeding and the use of human milk. Pediatrics. 2005;115:496-506. 2. Anand RK, Kumta NB, Kushwaha KP, Gupta A (Eds). The Science of Infant Feeding. New Delhi: Jaypee Brothers Medical Publishers (P) Ltd.; 2002. 3. Breastfeeding in modern medicine. In: Lawrence RA, Lawrence RM. Breastfeeding: A Guide for the Medical Profession, 5th edition. St Louis: Mosby; 1999. 4. Breastfeeding Promotion Network of India. Infant and young child feeding counseling—a training course, the “3 in 1 course” (integrated course on breastfeeding, complementary feeding and infant feeding and HIV). New Delhi: Breastfeeding Promotion Network of India; (2010). 5. Butte NF, Lopez-Alarcon MG, Garza C. Nutrient Adequacy of Exclusive Breastfeeding for the Term Infant during the First Six Months of Life. Geneva: World Health Organization; 2002. 6. Horta BL, Bahl R, Martines JC, et al. Evidence on the LongTerm Effects of Breastfeeding: Systematic Reviews and MetaAnalysis. Geneva: World Health Organization; 2007. 7. Infant and Young Child Feeding Chapter, Indian Academy of Pediatrics, Rajeshwari K, et al. Infant and young child feeding guidelines: 2010. Indian Pediatr. 2010;47:995-1004. 8. Ministry of Women and Child Development, Food and Nutrition Board, Government of India. National Guidelines on Infant and Young Child Feeding, 2nd edition. New Delhi: 2006. 9. Ministry of Women and Child Development, Government of India. (2003). The Infant Milk Substitutes, Feeding Bottles and Infant Foods (Regulation of Production, Supply and Distribution) Amendment Act, 2003. [Online] Ministry of Women and Child Development website. Available from http://wcd.nic.in/IMSamendact2003.pdf [Accessed September 2012]. 10. World Health Organization, United Nations Children’s Fund. Global Strategy for Infant and Young Child Feeding. [online] WHO website. Available from http://www.who.int/nutrition/ publications/gs_infant_feeding_text_eng.pdf [Accessed September 2012].



Bibliography



ics f

e

i

t

ook o P d a r tb e

T x

free samples, gifts to mothers and health workers has led to convince them that formula feeding is as good as breastfeeding. This has also made a dent in the confidence of lactating women in her capacity to optimally breastfeed and has contributed to the decline of breastfeeding rates. Recognizing this trend, the Indian Parliament enacted the “Infant Milk Substitutes, Feeding Bottles and Infant Foods (Regulation of Production, Supply and Distribution) Act 1992 (IMS Act)”. The IMS Act was further amended in the year 2003. The IMS Act controls marketing and promotion of infant milk substitutes, infant foods and feeding bottles. Some salient features of the IMS Act include: • It bans any kind of promotion or advertisement of infant milk substitutes, infant foods and feeding bottles to the public including electronic and print media. • It prohibits providing free samples of infant milk substitute, infant foods and feeding bottles and gifts to any one including pregnant women, mothers of infants and members of the families. • It prohibits donation of free or subsidized supplies of infant milk substitute, infant foods and feeding bottles for health care institutions except donations to the orphanages. • It prohibits display of posters of infant milk substitutes, infant foods and feeding bottles at health care facilities, hospitals and health centers. • It prescribes rules for information on the containers and labels of infant milk substitutes and infant foods including a specific statement in English and local languages that “Mother’s milk is best for the baby” in capital letters. • It prohibits having pictures of infants or women or phrases designed to increase the sale of the product on the labels of the products. • It prohibits any contact of employers manufacturing and distributing company with pregnant women even for providing educational material to them. • It prohibits direct or indirect financial inducement or gift to health worker or to any members of his family by the producer, supplier or distributor of the infant milk substitute, infant foods and feeding bottles. • The IMS Act also prohibits offering or giving any contribution or pecuniary benefit to a health worker or any association thereof including funding of seminar,

126

vip.persianss.ir

nfant and Young Child Feeding

I



Nutritio

4.2

n

RK Anand “We know that shamefully large numbers of children in both wealthy and poverty-stricken regions suffer malnutrition: the malnutrition of excess amounts of inappropriate foods and the malnutrition of insufficient nutritious foods. As adults we should feel embarrassed that so many small children are so poorly fed. We can change this situation if we want to and many people are already working to this end. Good nutrition for children harmonizes with good nutrition for adults…and it would be good for the world if we worked to this end.” Gabrielle Palmer



Complementary Feeding

T

It is the process of giving a child other food while continuing breastfeeding, when her or his nutritional demands can no longer be fulfilled by breastfeeding alone. Appropriate complementary feeding should be timely, culturally acceptable, nutritionally adequate, safe and responsive.

imely Feeding

It is recommended that all infants be exclusively breastfed for 6 months and adequate complementary foods be added after that. Complementary feeding indicators in India are far from satisfactory (Fig. 4.2.2). According to the NFHS 3, introduction of complementary feeding along with continued breastfeeding in children of 6–8 months is only about 55%. Addition of anything other than breast milk before 6 months is fraught with danger for the following reasons: • Addition of foods and other liquids (including water, soup, juice, rice water, dal water, etc.) interfere with -

127

 

Figure 4.2.1 Optimal infant and young child feeding

-

-

-

-

Optimal infant and young child feeding (IYCF) is an evidence based measure for improving child nutrition and child survival. The “WHO/UNICEF Global Strategy for Infant and Young Child Feeding and the National Guidelines on Infant and Young Child Feeding 2010” recommended by the IYCF subspecialty chapter of the Indian Academy of Pediatrics stress that for proper growth and development, infants should be exclusively breastfed with no other food or drink—not even water in the first 6 months of life (see Chapter 4.1). This must be followed by sequential addition of nutritionally adequate, preferably home made semisolid and solid foods to complement (not to replace) breast milk, till the child is gradually able to eat normal family food after 1 year while breastfeeding is continued up to 24 months of age or beyond (Fig. 4.2.1). Adequate nutrition for adolescent girls and pregnant and lactating mothers is also important for child nutrition.

The period after 6 months, when other foods are added is also referred to as weaning. Some wrongly interpret it as weaning the baby away from the breast. Complementary feeding is a better term than weaning.



ptimal nfant and Young Child Feeding I

O

While adequate nutrition is important throughout child­ hood, it is crucial during the first 5 years of a child’s life, particularly so, in the first 2–3 years when rapid growth occurs and when the child is entirely dependent on the mother and the family for food.

vip.persianss.ir

f

c

extbook o Pediatri s T

 

Figure 4.2.2 Complementary feeding indicators in India (National Family Health Survey-3)

-

A

Nutritionally dequate Complementary Feeding

-

-



-



To be nutritionally adequate, the complementary foods should contain all food groups—the staple, proteins, vitamins and minerals (Fig. 4.2.3, and Tables 4.2.1 and 4.2.2). After 6 months, add home made porridge or a fruit like ripe banana. Porridge can be made with the staple cereal used by the family like whole wheat flour (atta), rice, semolina (suji or rawa), broken wheat, ragi (nachni) or millet. Breast milk or any other milk can be used to make the porridge. Pieces of chapatti could also be soaked in milk, mashed properly and passed through a sieve to provide a soft semisolid food for the infant. Sugar and cream (malai) can be added to make it energy dense. Boiled or well cooked mashed vegetables (pumpkin, peas, cauliflower, carrots, leafy vegetables, sweet and 128 other potato, beet, tomato) should be added to provide vitamins and iron. Also, offer other seasonal fruits. Gradually

 

 

introduce khichdi with ghee/butter/oil, seera or halwa, upma, poha, dhokla, idli, dosa, pongal, missi roti (paratha made with a batter of wheat flour, gram flour, spices and dal). If foods of animal origin are acceptable to the family, flesh foods should be encouraged. Start with only one food at a time. Wait for a week before introducing another food so that we know whether or not the child is tolerating it. Children who do not eat at a time should be offered food, fruits or a milk feed every 2–3 hours. The child should be encouraged but not forced to eat. Some children may choke a little while learning to eat. Parents may be told not get scared but to be with the child to ward off any trouble. When children are helped to use a spoon, let it be dipped into a preparation like shrikhand or phirni. A bit will stick to the spoon and give children the joy of getting something into their mouth on their own. A child eating well around 7 months or so may suddenly become disinterested in eating. The parents should be told -

•

Figure 4.2.3: Food square for the young child. Source: [Elizabeth KE. Current concepts on nutritional requirements of infants and children. Ind J Pract Pediatr. 2011;13(1):5-11]



•



•

-

-

•

optimal breastfeeding. They may fill up the child’s stomach and quench the thirst, and consequently may lead to less suckling at the breast with reduced milk production. Increased risk of allergic disorders due to allergens passing through the not yet fully mature gut of the infant. It takes about 6 months after birth for the intestine to become reasonably mature. Enzymes needed to digest foods other than breast milk are also produced around 6 months. The tongue thrust reflex is active before 6 months. Infants tend to push out with the tongue anything other than liquids. Foods other than breast milk may result in more gastrointestinal and other infections and malnutrition. They may put unnecessary load on the kidney and lead to obesity, hypertension and coronary artery disease later in life. Less frequent suckling also increases the possibility of the mother becoming pregnant again.

vip.persianss.ir

Nutritio

 

Table 4.2.1 Foods rich in iron Foods rich in iron (mg/100 g of edible portion) Cereals

Cauliflower leaves

40

Ragi

3.9

Chaulai

22.9

Maize flour

2.3

Muli leaves

18

Barley

1.67

Suva ni bhaji

17.4

Rawa

1.6

Pudina

15.6

Dry fruits

Arvi pan green

10

Black til

56.7

Carrot leaves

8.8

Til

9.3

Green onion

7.43

Coconut (dry)

7.8

Kothmir

1.42

Kaju

5.81

Palak

1.14

Badam

5.09

Arvi pan black

0.98

Walnut

2.64

Sargava pan

0.85

Singdana

2.5

Milk and its products

Pulses

Mawa-khoa

5.8

Soybean

10.4

Cheese

2.1

Moth

9.5

Chauli

8.6

Sugars Gud

2.64

Lentil (masoor)

7.86

Sago

1.3

Mutter (dry)

7.05

Channa dal

5.3

Meat Liver (sheep)

6.3

Rajma

5.1

Egg (hen)

2.1

Mung

4.4

Cereals Rice flakes (poha)

20

Mung bean Urad dal

3.9 3.8

Bajra

8

Tuvar dal

2.7

Rice puffed (mamra)

6.6

Wheat flour

4.9

Poppy seeds

15.9

Jowar

4.1

Vermicelli-sev (wheat sev)

2

not to panic but to try some new preparation. In any case, children should never be forced to eat more than what they want. Even if half a spoon is left in the bowl and the child is not interested to eat any more, the parents should respect the child’s appetite. Water can be given, once the child starts taking foods other than breast milk and fruits. It should be offered from an ordinary glass. Children learn to sip from the glass within a few days. Few general guidelines about the nutritive value of foods are: • To retain the nutrients, vegetables including potatoes should be scraped instead of peeling them. The water in which rice or vegetables have been boiled for cooking should be used and not thrown away. The vegetables

n

Vegetables

•

should not be overcooked. Children should be encouraged to get used to the taste of properly washed raw vegetables from an early age. In general, some amounts of spices are good for health. Most children can tolerate moderate amount of spices used for cooking in most homes. Dry fruits are good, but they must also be rationed, partly because they are expensive and more so because some of these, like dates, figs and raisins, can remain stuck between the teeth leading to caries. Foods that may lead to choking in children should be avoided in those below 3 years. Examples of these foods are: raw carrots, roasted grams, peanuts, other nuts, popcorn, hard candies, berries and whole grapes. 129

vip.persianss.ir

extbook o Pediatri s

Foods rich in calcium (mg/100 g of edible portion)

Foods rich in zinc (mg/100 g of edible portion)

Milk products and dry fruits

Gingelly seeds

12.2

Til

1,450

Bengal gram (desi)

6.1

Cheese

790

Cashew nut

5.99

Khoa

650

Safflower seeds

5.2

Coconut (dry)

400

Mustard seeds

4.8

Black til

300

Cow pea

4.6

Badam

230

Omum seeds

4.52

Milk (buffalo)

210

Rajma

4.5

Curd

149

Soybean black

4.4

Pista

140

Poppy seeds

4.34

Milk (cow)

120

Groundnut

3.9

Watermelon seeds

100

Samai almond

3.7

Walnut

100

Betel leaves

3.44

Soybean white

3.4

Cereal and pulses Ragi

344

Black gram (whole)

3.3

Rajma

260

Coriander seeds

3.26

Soybean

240

Bajra

3.1

Moth

202

Lentil dal

3.1

Channa

202

Red gram (whole)

3.1

Urad dal

154

Fenugreek seeds

3.08

Vlung

124

Black gram (dal)

3

Tuvar dal

73

Green gram (whole)

3

Channa dal

56

Sanwa millet

3

Cashew nut

50

Bengal gram (kabuli)

2.9

Poppy seeds

1,584

Cardamom

2.81

Almond

230

Maize (dry)

2.8

Foods like idli and parathas made from whole wheat flour should be preferred to white bread. -

•

Foods that may be Avoided in the First Year of Life



In families with a strong history of allergy, peanuts and other nuts should be avoided. If any member of the family is known to react adversely to a particular food, avoid that as well. Also fried foods, foods containing too much sugar, artificial sweeteners, monosodium glutamate (MSG) (ajinomoto) and high in salt should be avoided. As recommended by the National Institute of Nutrition, the following points need to be kept in mind: • The recipes for complementary foods should be based on locally available food stuffs • The cooking methods must be simple • The cost should be minimal

130

• •

The recipes should be acceptable in taste and consistency Gradually the child should be introduced to healthy foods eaten by the rest of the family.

Consistency of Complementary Foods (Fig. 4.2.4) • To provide more calories from smaller volumes, food must be thick in consistency—thick enough to stay on the spoon without running off when the spoon is tilted. • Foods, such as nuts, which can pose choking hazard, should be avoided. Introduce lumpy or granular foods and new tastes by about 9–10 months. Missing this age may lead to fussy eating later. Avoid using mixers to make the food too smooth. • Three to four teaspoons of roasted groundnut powder can be added to the daily diet of the infant. The meal can also be made energy dense by adding ghee/butter/ oil and sugar/jaggery. -

T

f

 

c

Table 4.2.2 Foods rich in calcium and zinc

vip.persianss.ir

Nutritio

Method of Feeding

n

-

-

When required, take suitable amounts (say 3 tablespoons) of any one of the above ready to use infant weaning food and mix with a small amount of hot water. Add more sugar or jaggery, if required, before feeding.

Amount and Frequency of Food to Be Offered (Table 4.2.3)

-

This depends on the capacity or the size of the child’s stomach, which is usually 30 mL/kg of the child’s body weight. A child who weighs 8 kg will have a stomach capacity of 240 mL, about one large cup full and cannot be expected to eat more than that at one meal. Parents may not realize that a child of 1 year needs about 1,000 calories each day—almost half of what an adult may take. So some guidance to parents may be given in this respect. After that, children should be left to decide how much they want to consume. A good guide that children were having optimum quantity of food is their level of activity and weight gain. S

eady-to-use nfant Weaning Foods

afe Complementary Feeding

I

R

 

Figure 4.2.4 Consistency of complementary food

The following recipes can be prepared in bulk and kept ready at hand for feeding infants.

Bajra Infant Food

-



esponsive Feeding (Fig. 4.2.5)

While feeding young children, the caregivers should provide psychosocial stimulation to the child through age appropriate play and praise. Children sitting on the lap of a caregiver or eating with loved ones learn to enjoy eating. Self feeding must be encouraged even if the child makes a mess (Fig. 4.2.6). Forced feeding, threatening and punishment interfere with development of proper feeding habits. Distractions during meals and feeding in front of the television should be avoided.

-

Use 45 gm of ragi prepared as given below instead of bajra in the above formula. Soak ragi in water overnight. Drain the water, spread the grains on a plate and allow them to germinate by covering with a damp cloth for one day. Dry the germinated ragi in sun and roast till it develops a malted flavor. Powder and store in an air tight tin.

 

Table 4.2.3 Amount of food to be offered Age Texture

-

-

Ragi (Nachni) Infant Food

R











Bajra (dehusked, roasted) 3 tablespoons Roasted greengram dal 1½ tablespoons (or any other dal) Roasted groundnut 3/4 tablespoon Roasted decorticated till 1 tablespoon (gingelly) seeds Sugar 2 tablespoons Powder all the roasted ingredients individually; mix them in the proportions suggested, and store in air light containers.

All utensils used for feeding must be washed thoroughly. There is no need to sterilize the utensils. Eating by hand need not be discouraged. Finger foods, which the child can hold and chew, may be given. However, the hands of the caregiver and the child must be washed thoroughly with soap and water before and after eating. Microbial contamination of complementary foods is more in hot weather. It is slower if the food is refrigerated. When that is not possible, the food should be eaten within 2 hours of its preparation. Even food kept in the refrigerator should be consumed within a day or two.

Frequency

Average amount of each meal

6–8 months

Start with thick porridge, well-mashed foods

2–3 meals per day plus frequent breastfeeding

Start with 2–3 tablespoonful

9–11 months

Finely chopped or mashed foods, and foods that baby can pick up

3–4 meals plus breastfeeding. Depending on appetite offer 1–2 snacks

1/2 of a 250 mL cup/bowl

3–4 meals plus breastfeeding. Depending on appetite offer 1–2 snacks

3/4 to one 250 mL cup/bowl

12–23 months Family-foods, chopped or mashed if necessary. As per appetite offer 1–2 snacks

If baby is not breastfed, give in addition: 1–2 cups of milk per day, and 1–2 extra meals per day. The amounts of food included in the Table are recommended when the energy density of the meals is about 0.8–1.0 kcal/g. If the energy density of the meals is about 0.6 kcal/g, recommend increasing the energy density of the meal (adding special foods) or increase the amount of food per meal. Find out what the energy content of complementary foods is in your setting and adapt the Table accordingly.

131

vip.persianss.ir

-



f

-

c

extbook o Pediatri s

the children get so used to them that they may not accept home made foods. Families must be equipped with means and knowledge to feed their children without the need for processed foods. All effort must be made to ensure that government policies protect public health before private profit. Tinned food/juices, cold drinks, packaged drinks and packaged wafers, health drinks, nutrition supplements promoted for “picky eaters”, bakery products, drinks with low nutrient value such as tea, coffee and sugary drinks should be avoided.

T

E

Picky aters  

Figure 4.2.5 Responsive feeding

 

Figure 4.2.6 Self-feeding

Feeding during and after Illness The appetite during an illness may go down. However, even sick babies continue to breastfeed quite often. They should be encouraged to take enough liquids and small quantities of nutrient rich food that they like to eat. After the illness, the nutrient intake can be increased by adding one or two extra meals in the daily diet for about a month by offering nutritious snacks between meals, by giving extra amount at each meal and by continuing breastfeeding.

S

Junk and Commercial Nutrition upplements -

-

Commercial ready made cereals, though convenient to use, are not preferred over home made foods. Besides high cost, the smooth consistency of such products may make

Of late, a nutritional supplement is being aggressively promoted for so called “picky eaters” for better growth and height. Before the parents start using such a product, they should be given the following information: • An infant grows rapidly in the early months of life. In the second year, the growth will be slower, the appetite may decrease and vary from day to day. Between 15 months to 3 years, the child often passes through a phase of negativism and does the opposite of what the parents want. If the child is unwell, the appetite may suffer even more though the mother’s milk is often not refused. • Keeping the above in mind, the child should not be forced to eat. Of course, various healthy food options must be offered at frequent intervals. Allow children to eat with their hands, even if it turns out to be a messy affair. Then let them learn to use the spoon. The parents may fill the spoon off and on or may offer the child some food with a separate spoon, while gradually encouraging the child to eat independently. • Parents need not get upset if the child does not eat “nourishing” foods for a few days. Children have their moods; for some days, they may eat less of certain foods, but if left to themselves, they may start eating the same again after a gap of few days. • Children, who are small at birth, may not weigh as much as their peers. The parents should be told that so long the child follows the growth curve, they should be happy. In fact, if these children are given food or products too high in calories and become obese, they become potential candidates for developing diabetes and cardiovascular diseases. • Make sure the child is not anemic and does not have urinary or any other infection responsible for anorexia. • Convey to the parents that the product promoted for “picky eaters” is expensive, not wholesome and comes in the way of the child developing healthy food habits. Also, one may get a false sense of security while the underlying causes for fussy eating mentioned above are missed.

132

vip.persianss.ir

1. Anand RK. Health workers and the baby food industry: World Health Organization acts to end conflict of interest and promote breastfeeding. Br Med J. 1996;312:1556 7. 2. Bellad R. Complementary feeding. In: Bharadva K, Tiwari S, Chaturvedi P, Bang A, Agarwal RK (Eds). Feeding Fundamentals: A Handbook on Infant and Young Child Nutrition. 1st edition. Jaipur: Pedicon 2011; 2011. pp. 89 93. 3. Kushwaha KP. Complementary feeding of breastfed children. In: Anand RK, Kumta NB, Kushwaha KP, Gupta A (Eds). The Science of Infant Feeding. 1st edition. New Delhi: Jaypee Brothers Medical Publishers (P) Ltd; 2002. pp. 117 34. 4. Low Cost Nutritious Supplements, 2nd edition. Hyderabad, National Institute of Nutrition; 2000. 5. Palmer G. Complementary Feeding: Nutrition, Culture and Politics, 1st edition. London: Pinter and Martin; 2011. 6. Wiessinger D, West D, Pitman T. The Womanly Art of Breastfeeding, 8th edition. New York: Ballantine Book; 2010. 7. World Health Organization. Complementary Feeding of Young Children in Developing Countries: A Review of Current Scientific Knowledge. Geneva: World Health Organization; 1998. 8. World Health Organization. Infant and Young Child Feeding: Model Chapter for Textbooks for Medical Students and Allied Health Professionals. Geneva: World Health Organization; 2009.

B

n

-













-





-





-





ibliography





I

O

K

• Initiate breastfeeding as early as possible after birth, preferably within 1 hour • With the exception of any essential medicine, practice exclusive breastfeeding from birth to 6 months of age and introduce complementary foods at 6 months (180 days) • Continue frequent on-demand breastfeeding until 2 years of age or beyond • Start complementary foods at 6 months of age with small amount. Increase the quantity and frequency as the child gets older, while maintaining frequent breastfeeding • Gradually, increase food consistency and ensure that all nutrient needs of the child are met • Practice responsive feeding, applying the principles of psychosocial care • Practice good hygiene by handwashing with soap and water before preparing food, before feeding the child and after using the toilet • Increase fluid intake during illness, including more frequent breastfeeding, and encourage the child to eat soft favorite foods. After illness, encourage the child to eat more often • Ensure adequate nutrition including control of anemia in infants, young children, adolescent girls and pregnant and lactating mothers. Vitamin and mineral supplements must be given if required • Support the implementation and monitoring of IMS Act.

Nutritio

ey Messages for ptimum nfant and Young Child Feeding

133

vip.persianss.ir





Malnutrition Meenakshi Mehta

“Underlying every other condition is malnutrition, due to both calorie and protein deficiency. Though poverty is the main contributing cause, it is greatly aggravated by lack of proper dietary knowledge.” (the orange ribbon) an awareness ribbon for malnutrition

t

t

Tex book of Pedia rics

4.3

-

D

efinitions and lassification O

The World Health rganization (WH ) defines PEM as range of pathological conditions arising from coincidental lack in varying proportions of proteins and calories, occurring most frequently in infants and young children, and commonly associated with infection. The extent of weight loss and

-

-

-

-

Eighty percent of the world’s undernourished children live in just 20 countries. of an estimated 178 million under five stunted children, most live in sub Saharan Africa and South Central Asia. Despite having economic growth, progress and largest food and nutrition program, India is home to over one third of world’s malnourished children, and for 5.6 million deaths due to malnutrition out of 10.4 million child deaths per year (Fig. 4.3.1). NFHS 3 (2005–2006)

C

Magnitude of the Problem

reports a prevalence of 48% for stunting (including 24% severely stunted), 43% for underweight (including 16% severely underweight) and 20% for wasting in under five children. Prevalence of malnutrition varies state wise with highest in Madhya Pradesh (55%), and lowest in Kerala (27%). PEM has higher incidence in nutritionally vulnerable groups: young children (especially between 6 months and 2 years) and women during pregnancy and lactation as the nutritional requirements are larger relative to their size than in older children and adults. The damage caused by malnutrition in the intrauterine life or in the first 2 years of life may be irreversible due to impairment in developing brain (Fig. 4.3.2).

O

-

-

Protein energy malnutrition (PEM) is one of the most widespread health and nutritional problem of the deve­ loping countries. Annually, undernutrition kills or disables millions of children. It often causes disease and disability in the survivors and prevents millions more from reaching their full intellectual and productive potential. Stunting, severe wasting and intrauterine growth restriction (IUGR) together accounted for 2.2 million deaths and 21% of disability adjusted life years (DALYs) for under five children.

Figure 4.3.2 Causes of under-five deaths  

 

Figure 4.3.1 Country-wise prevalence of malnutrition in under-five children

134

vip.persianss.ir

Nu ri io

 

Table 4.3.1 Classification of malnutrition Classification Definition Gomez Weight below % median WFA

t

Grading Mild (Grade 1) Moderate (Grade 2) Severe (Grade 3) Z-scores (SD) below median WFH Mild Moderate Severe Z-scores (SD) below median WFH Moderate Severe Z-scores (SD) below median WFA Moderate Severe MUAC divided by occipitofrontal head circumference Mild Moderate Severe Z-scores of BMI for age Grade 1 Grade 2 Grade 3 Percentage of standard weight for age (50th centile of Normal Harvard standard) Grade 1 Grade 2 Grade 3 Grade 4 Mid-arm circumference Normal Mild-moderate Severe PEM Presence/absence of edema 2 years

Type III (mild, Kugelberg-Welander disease)

> 18 months

Stand and walk

Adult

Spinal muscular atrophy is the most common anterior horn cell disorder encountered in children. It will be discussed briefly below. A

S

pinal Muscular trophy Spinal muscular atrophy (SMA) is characterized by degeneration of motor neurons of the spinal cord, which





results in hypotonia and muscle weakness. Spinal muscular atrophy is divided into three clinical types (Table 6.15.12). Ninety-six percent children with 5q13 linked SMA shows SMN1 gene deletion or gene conversion from SMN1 to SMN2. Four percent patients have intragenic SMN1 mutations. Type 1 disease presents with profound hypotonia and flaccid weakness (Fig. 6.15.5). Respiratory weakness, poor swallowing and tongue fasciculations are common. Aspiration pneumonia is an important cause of morbidity and mortality. Children with type II disease are usually able to sit unaided. They may develop kyphoscoliosis, tremors (polyminimyoclonus), poor swallowing and respiratory insufficiency. Type III patients are usually able to walk. They may mimic LGMD or myopathy. Treatment is usually supportive. Various therapeutic 405 strategies, being evaluated, are enlisted in Table 6.15.13.

D

nterior Horn ell isorders C

A

onset is recommended. The treatment is warranted in non-ambulatory patients but their role in mildly affected GBS patients who are mobile is uncertain. Patients who have not responded to initial IVIG treatment may benefit from a second course of IVIG. General supportive care includes cardiorespiratory care, physical therapy, nutritional management, management of neuropathic pain, bladderbowel care and prevention of deep vein thrombosis.

vip.persianss.ir

Tex book of Pedia rics

ibliography

• Detailed history and examination, in a child with suspected neuromuscular disease, is essential to localize the lesion in the motor unit. • Family history with pedigree drawing is imperative to reach appropriate diagnosis. • Investigations must be planned judiciously while managing children with neuromuscular disorder. • Never miss potentially treatable conditions like neuromuscular junction disorders which may mimic















ey Messages



K



SMN1 gene replacement: Gene therapy SMN2 activation: Histone deacetylase inhibitors, hydroxyurea Promotion of exon 7 inclusion: Anti-sense oligonucleotides Stabilization of SMN protein: Aminoglycosides Cell replacement: Cell therapy Neuroprotection: Riluzole, gabapentin



•  •  •  •  •  • 



 

Table 6.15.13 Therapeutic approaches in spinal muscular atrophy

1. Bushby K, Finkel R, Birnkrant DJ, et al. Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis and pharmacological and psychosocial management. Lancet Neurol. 2010;9:77-93. 2. Darras BT, Friedman NR. Metabolic myopathies: a clinical approach; Part I. Pediatr Neurol. 2000;22:87-97. 3. Darras BT, Friedman NR. Metabolic myopathies: a clinical approach; Part II. Pediatr Neurol. 2000;22:171-81. 4. England JD, Gronseth GS, Franklin G, et al. Evaluation of distal symmetric polyneuropathy: the role of laboratory and genetic testing (an evidence based review). Muscle Nerve. 2009;39:116-25. 5. Jackson CE. A clinical approach to muscle disease. Semin Neurol. 2008;28:228-40. 6. Joint Task Force of the EFNS and the PNS. European Federation of Neurological Societies/Peripheral Nerve Society Guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy. Report of a Joint Task Force of the European Federation of Neurological Societies and the Peripheral Nerve Society. J Peripher Nerv Syst. 2005;10:220-8. 7. Moxley III RT, Tawil R. Channelopathies: myotonic disorders and periodic paralysis. In: Swaiman KF, Ashwal S, Ferriero DM (Eds). Pediatric Neurology, Principles and Practice, 4th edition. Elsevier; 2006. p. 2085. 8. Tawil R, van Der Maarel SM. Facio-scapulo-humeral muscular dystrophy. Muscle Nerve. 2006;34:1-15.



 

Figure 6.15.5 Frog like posture in a child with spinal muscular atrophy type II

B

t

t

common muscle disorders. They may also have potentially fatal complications if unrecognized. • Genetic counseling and pre-natal diagnosis are important in selected clinical scenarios. When we come across families with multiple sibs affected with common conditions like SMA and DMD, the medical fraternity carries the blame for not offering appropriate genetic counseling and pre-natal diagnosis.

406

vip.persianss.ir

A

ental etardation and utism R

M





Cerebral Palsy,

Diseases of Central ervous ystem

6.16

MS Mahadeviah

Cerebral palsy at present is broadly classified to spastic dyskinetic, mixed, ataxic and hypotonic.

pastic diplegia: It refers to the spasticity predomi­ nantly of the lower extremities with minimal or no involvement of upper extremities. The major causes include prematurity, ischemia and infections leading to periventricular leukomalacia. These children have relatively better cognitive functioning.

•

pastic hemiplegia: It refers to involvement of left or right side of the extremities; upper usually greater than lower. Spastic hemiplegia has the best prognosis for ambulation; but is more prone to develop seizures. In 20–30% the pathology is contralateral infarcts.

•

pastic quadriplegia: It refers to equal involvement of all the four extremities with generalized spasticity. They have considerable clinical manifestations of drooling, difficulties in feeding, seizures along with speech, hearing, visual and intellectual disabilities. The most common pathologies are periventricular leukomalacia and multicystic cortical encephalomalacia. The prognosis for independent ambulation is poor.

E

tiology



At present 75–80% of causes of CP point to antenatal factors, which are responsible for abnormal development of brain. Main etiological factors in 10–20% of children with CP are intrapartum asphyxia and exposure to maternal infections such as chorioamnionitis, sepsis, urinary tract infections and fever with elevated levels of cytokines. Prematurity, especially infants weighing less than 1,000 g is a major risk factor resulting in intraventricular hemorrhage and periventricular leukomalacia. Perinatal and neonatal causes such as sepsis, neonatal seizures, cerebral ischemia and low Apgar scores are present in substantial number of children with CP.

Clinical Features The most common presentation of CP is delay in motor development such as head control, sitting, standing and walking. All types of CP are characterized by abnormal muscle tone, reflexes or motor development and coordination. Other features include asymmetrical reaching for objects, abnormal movements and postures. Associated manifestations include delay in speech, visual difficulties, intellectual disability and seizures.

•

S

S

•

S

Spastic type of CP accounts for 70–75% of the cases with CP. Spastic CP is further classified to spastic diplegia, spastic hemiplegia, spastic quadriplegia, spastic monoplegia and spastic triplegia, as described below.

Definition of Cerebral Palsy Cerebral palsy describes a group of permanent disorders of the development of movement and posture causing activity limitation. It is also attributed to nonprogressive disturbances that occurred in the developing fetal or infant brain. The motor disorders of CP are often accompanied by disturbances of sensation, perception, cognition, communication and behavior, epilepsy and secondary musculoskeletal problems. Cerebral palsy excludes motor disorders of spinal, peripheral nerve, muscular or mechanical origin. Although it is stated that there is no explicit upper age limit, CP usually refers to the first 2–5 years when there is active growth of the brain.

N

Spastic Cerebral Palsy

S

Cerebral palsy (CP) as it is commonly known is the commonest cause of physical disability in children. It encompasses a group of nonprogressive and noncontagious disorders causing physical disability mainly in the various areas of body movement. In spite of all the progresses in newborn care its prevalence remains at 2–2.5 per 1,000. The prevalence in India is not definitively established. Although CP is described as a static encephalopathy, the neurological features may change over the time.

Classification

S

Cerebral Palsy

pastic monoplegia and triplegia: These are rare.

Dyskinetic Cerebral Palsy These include athetoid or choreoathetoid children with dyskinetic CP presenting with hypotonia, head lag, variable tone, drooling, tongue thrust, rigidity and dystonia. In the past dyskinetic CP was common with neonatal hyperbilirubinemia secondary to Rh incompatibility, which may still be a cause in developing countries. Other factors include severe birth asphyxia, lessons in basal ganglia and mitochondrial disorders.

Differential Diagnosis It is important to rule out disorders of motor disability. Detailed history and examination should be directed to rule out progressive neurological disorders (degenerative), muscular disorders (myopathies and muscular dystrophies), spinal cord tumors and genetic syndromes.

407

vip.persianss.ir

I

R

ental etardation ( ntellectual Disability) M

I

At the present state of the art of medical practice, an MRI scan of the brain is necessary. Ophthalmic evaluation, hearing tests, speech and language evaluation, psychological educational evaluation, EEG and other investigations should be done depending on the clinical profile.

Definition

Counseling the Parents

Prevalence

anagement

Intellectual disability affects the population in varying proportions; however, 75% of these cases fall under mild intellectual disability. Severe intellectual disability accounts to less than 5% of the population of affected children.

-

Counseling the parents is the greatest challenge the professional faces. It is important to teach the parents regarding the activities of daily living such as feeding, bathing, dressing and follow up of therapy that is being done by the physiotherapist and occupational therapist. Frequent follow up, addressing the special needs of children and parents should be the most important service provided by the center.

-

-

Management of children with CP involves a multidisciplinary team, which should include a developmental pediatrician, physiotherapist, occupational therapist, orthotic specialist, orthopedic surgeon, psychologist, educational specialist, speech pathologist and social worker along with consultants in neurology, psychiatry, ophthalmology, assistive technology and otolaryngology as necessary.

Mental retardation (MR) is defined as “significantly sub average general intellectual functioning, existing concurrently with deficits in adaptive behavior and mani­ fested during the developmental period that adversely affects a child’s educational performance,” according to the US Special Educational Plan, The Individuals with Disabilities Education Act. Many consider the use of the term “MR” as offensive and the term intellectual disability or intellectually challenged is now preferred by most advocates in most English speaking countries.

M

d

c

extbook of Pe iatri s T

nvestigations

-

Pathogenesis

Management includes prevention of contractures, which is the main goal of physiotherapist and orthopedic surgeon.

Surgical Procedures

-

tiology

Intellectual disability (MR) is multifactorial in etiology. The causes include socioeconomical to genetic, metabolic, endocrine and infectious disorders, and induced by toxins and trauma. Mild forms, which are the most common one, occur four times greater in women with poor education and socioeconomic population. A list of common causes of intellectual disabilities is given in Table 6.16.1.

Clinical eatures F

-

Surgical procedures such as tendo achilles lengthening, adductor tenotomy, psoas transfer and soft tissue release to prevent dislocation of hip. Posterior rhizotomies are useful in selected cases with spastic diplegia.

Mild intellectual disability (MR): IQ level 50–70 Moderate intellectual disability (MR): IQ level 35 50 55 Several intellectual disability (MR): IQ level 25 35 40 Profound intellectual disability (MR): IQ level below 20–25. -

• • • • E

Physiotherapy

Classification

-

Some of the common drugs used are muscle relaxants such as lioresal, baclofen, dantrolene sodium and benzo­ diazepines. One of the most popular drugs that is used to relieve spasticity is botox (botulinum toxin), which when injected to specific muscle groups is effective in reducing spasticity. However, it is important to have specific evaluations for such therapies because of the cost involved. Levodopa and carbamazepine are recommended in children with dystonia and rigidity.

-

Drugs

There are no specific pathological correlates for intellectual disability. The more severe the intellectual disability, greater are the chances of finding abnormalities in the brain. These include reduction in brain volume (microcephaly), disorders of cell migration, heterotopias, polymicrogyria, pachygyria and dendritic changes.

Prognosis Cerebral palsy is a lifelong disorder and hence parents and adolescents require constant counseling. Independent ambulation is the dream of every parent. Children with hemiplegia and diplegia have better prognosis than children with quadriplegia. Anticipatory guidance should be provided regarding ambulation and education for future planning. A gross motor function classification system 408 provides the best guidance to clinician for prognosis.

Any dysmorphic feature such as microcephaly, macrocephaly and facial features as present in Down syndrome and Turner syndrome should raise suspicion of intellectual disability. Developmental delay involving milestones such as head control, sitting, standing, walking and talking are the earliest presentations. Special attention should be paid to speech and language development. Children with mild intellectual disability may later present with scholastic backwardness with global difficulties in learning.

vip.persianss.ir



 

 

 

 

 

 

 

 

 

 

 

 

 

I

Parental causes •  Consanguinity •  Maternal age and nutrition •  Infections •  Toxemias •  Metabolic diseases - diabetes mellitus •  Drugs - alcohol (fetal alcohol syndrome) Perinatal and neonatal •  Prematurity •  Low birth weight •  Fetal distress and asphyxia •  Difficult labor •  Multiple births •  Sepsis •  Seizures •  Hyperbilirubinemia Postnatal •  Malnutrition •  Infections - viral and bacterial (TORCH, HIV) •  Trauma resulting in head injury •  Child abuse and neglect Genetic, chromosomal and dysmorphic syndromes •  Down syndrome •  Klinefelter syndrome •  Turner syndrome •  Fragile X syndrome •  X-linked mental retardation •  Williams syndrome •  Cornelia De-Lange syndrome •  Neurocutaneous syndromes (tuberous sclerosis) •  Neurofibromatosis Inborn errors of metabolism •  Phenylketonuria •  Galactosemia •  Mucopolysaccharidosis Endocrine •  Hypothyroidism and iodine deficiency

nvestigations

-

M

anagement

S

Since intellectual disability is a lifelong condition, manage­ ment requires a multidisciplinary team that consists of a pediatrician, psychologist, education specialists, speech pathologist, social worker and occupational therapist. Consultants should include pediatric neurologist, psychi­ atrist and ophthalmologist. The key to the success of management depends on the inclusion of parents as equal partners in management. Early diagnosis in infancy is important. Medical professionals should pay attention to parents’ concerns regarding child’s development. A definitive diagnosis of developmental delay, if not the definitive cause can easily be done in the majority of cases by 2 years. Delay in neck control by 6 months, sitting by 12 months, walking by 18 months and talking by 2 years should alert every pediatrician of the possibility of intellectual disability.

Drugs There is no scientific evidence for the use of medications to improve brain functions. Perhaps the only drug that successfully cures MR is thyroxine for hypothyroidism if initiated in the newborn period. Antiepileptic drugs such as phenobarbitone, phenytoin, carbamazepine, valproates are used to treat various seizure disorders which are present in children with intellectual disability.

The list is endless. To make a broad statement, any adverse effect on the developing brain from conception to 18 years can cause intellectual disability.

• Primary prevention: These include counseling regarding proper maternal and infant nutrition, healthcare, prenatal immunization with rubella vaccine, folic acid supplements, genetic counseling and if indicated prenatal screening for malformations. The specific measures for children include proper nutrition, health education in school and universal immunization. • econdary prevention: Early diagnosis and rehabili­ tation frequent developmental screening. • ertiary prevention: Research and liaisons with governmental agencies for proper funding and support to the families. T

S

Prevention

According to the Diagnostic and Statistical Manual of Mental Disorders (DSM IV), three criteria must be met for a diagnosis of MR: (1) an IQ below 70; (2) significant limitations in two or more areas of adaptive behavior (as measured by an adaptive behavior rating scale, i.e. communication, self help skills, interpersonal skills and more) and (3) evidence that the limitations became apparent before the age of 18. An accurate medical history is the most important part of the diagnosis. The history should include prenatal, perinatal, postnatal, developmental, dental and visual, general health, school performance and behavior. Detailed history about family should include parental education, family income, socioeconomic status, history of the intellectual disability, epilepsy, genetic disorders and mental health. -

-

Diagnosis



N

Ideally newborn screening for thyroid function and metabolic disorders should be done depending on the clinical diagnosis and age of the child. Further investigations such as cranial CT scans, MRI of brain, EEG, hearing, speech and language studies, ophthalmic, psychological and psycho educational evaluations should be performed.



 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

A.             B.                 C.          D.                    E.       F.   

Diseases of Central ervous ystem

A detailed physical examination should note general physical appearance, dysmorphic features, neurocutaneous markers, eye contact, detailed developmental and neuro­ logical examination should be done.

Table 6.16.1 Common causes of intellectual disability

409

vip.persianss.ir

E

Since 75–80% of children with intellectual disability have mild degree of MR, attaining independent living and fair educational skills are good.

developmental disorders with considerable genetic etio­ logical factors.

tiology

Monozygotic twins have 90% concordance rates and dizygotic twins 30%. Many genetic syndromes including fragile X syndrome, Turner syndrome, Prader Willi, Angelman syndrome, tuberous sclerosis, neurofibromatosis and few more seem to have features of autism in some of the cases. Prenatal, perinatal and postnatal factors do not play a role in the etiology of autism.

Mild Mental Retardation

-

Given excellent rehabilitation, these children with MR who have minimal impairment in sensory motor area can hardly be differentiated from normal population. They develop good social and communication skills. Academic skills up to fifth to sixth standard can be achieved by teenage years. As adults they can be self supportive and can lead independent lives. They however need to be supervised and assisted during social and economic stress.

Prevalence

-

Moderate Mental Retardation

Prevalence rates have changed dramatically from 4–6/10,000 of early 60s to 40–60/10,000 according to recent reports. This increase seems to be due to many factors. • Autism is one of the spectrums • Better awareness among the parents and professionals • Availability of better clinical services • Perhaps a real increase, the exact cause of which is not known.

They constitute 10% of children. Most of them can acquire communicative skills, which can be provided by vocational training. They however have limited academic skills. They face considerable difficulties in adolescent period because of lack of social skills and peer relationships. As adults they will require sheltered workshops and need supervision.

F

n U

Pervasive Developmental Disorders

Pervasive developmental disorders, which include autistic disorders, Asperger syndrome, Rett syndrome and child­ hood disintegrative disorder (Heller syndrome) are of particular importance to the pediatrician as there seem to be an increasing prevalence in recent times. These disorders are characterized by marked impairment in many areas of child development. The common features include impairment in reciprocal social interactions, communication and the presence of stereotyped behavior, interest and activities. A

utism

-

-

Autism is a disorder of neural development characterized by impaired social interaction and communication, and by restricted and repetitive behavior. The diagnostic criteria require that symptoms become apparent before a child is 3 year old. It is perhaps the commonest of the pervasive

Pathophysiology and euroanatomical indings N

They constitute only 1–2%. These children have identifiable neurological and genetic diagnosis. With intense training they can develop some communication skills. However, they will require home care with individual supervision and treatment of associated conditions.

Occurrence of seizures points to the involvement of cerebral cortex. Anatomic changes are also noted in anterior cingulated gyrus—an area of brain associated with feelings, thoughts and decision making. Deficits in reticular activating system as well as abnormalities in prefrontal and temporal lobe are also common. However, these are not necessarily consistent findings. I

-

ti e

Profound Mental Retardation

Childhood autism has its manifestations usually at around 18–24 months of age and is well established by 3 years of age. The signs usually develop gradually, but some autistic children first develop more normally and then regress. Essential features are impairment of social interaction, communication and imagination and restricted interests and repetitive behavior. Deviant social skills include poor eye contact, gaze avoidance and failure to respond when name is called. Failure to gesture, point, lack of reciprocal smile, solitary play, hand flapping, making unusual sounds, and delay in acquisition of speech and language are the major features for the diagnosis of childhood autism.

nvestigations

Diagnosis is based on behavior and not by tests, cause or mechanism. There are specific psychological tools used to assess the child. Additional tests such as speech and language evaluation, psychological, educational, psychiatric assessments, EEG and MRI of brain may be necessary because of the comorbidities such as epilepsy, intellectual disability (MR), communication deficits and behavioral oddities.

They constitute 3–5% of children. Some of the children can profit to a limited extent by academic input. They should concentrate on acquiring self care skills. Adult years have to be spent at home or community homes.

G R

Clinical eatures

V d

Severe Mental Retardation

F

d

c

extbook of Pe iatri s T

Prognosis

410

vip.persianss.ir

S

A

-

–

N

S

B

S

-









-





-





-





-





1. American Psychiatric Association. DSM 5 development. 2010. 2. Denckla MB, James LS. An update on Autism: a developmental disorder. Pediatrics. 1991;87(Suppl 5). 3. Francis K. Autism Interventions: a critical update. Dev Med Child Neurol. 2005;47:493 9. 4. Nelson KB. The epidemiology of cerebral palsy in term infants. Ment Retard Dev Disabil Res Rev. 2002;8:146 50. 5. Palisano R, Rosenbaum P, Walter S, et al. Development and Reliability of a system to classify gross motor function in children with cerebral palsy. Dev Med Child Neurol. 1997;39:214 23. 6. Rutter M, Scopler E. Autism and pervasive developmental disorders. Concepts and diagnostic issues. J Autism Dev Disord. 1987;17:159 86. 7. Stromme P, Hagberg G. Etiology in severe and mild mental retardation: a population based study of Norwegian children. Dev Med Child Neurol. 2000;42:76 86. 8. The Definition and Classification of Cerebral Palsy. Dev Med Child Neurol. 2007;49:1 44. -

-

-





-

ibliography



– – –



Autism is not a progressive neurological disease and hence improvement will occur in all areas over the period of intervention. Children who start out with good speech and language skills to begin with and good cognition will do better. Many will grow up to have gainful employment and independent listing. Twenty five percent of cases of autism are of the regressive type.

G R

V d

ti e

n U

Prognosis

ett yndrome

Rett syndrome is a disorder of early development of brain. Rett syndrome is caused by mutations in the gene MECP2. Infants have normal head size till 6 months of age and then decrease is noted in brain growth. Major clinical features are the hand writing movements and loss of purposeful use of hands. Autistic behavior is noted at 2–3 years of age. Children with Rett syndrome develop seizures. Death occurs in adolescence usually in the third decade from cardiac arrhythmias.



–

–

–

–

R

–

–

–

–

-

M

sperger yndrome

Asperger syndrome differs from autistic disorder in that there are no delays in language. Single words are spoken at 2 years and phrases at 3 years and no delays are noted in cognitive development. These children have appropriate self help skills and adaptive behaviors. However, there is impairment in social interaction, restricted, repetitive behavior, interest and activities. Asperger syndrome may manifest later than autistic disorder. Social interaction becomes more noticeable in school age. Major milestones may be delayed and children with Asperger syndrome exhibit clumsiness. Asperger syndrome is a lifelong disorder and is more common among males.

anagement

Management requires a multidisciplinary team consisting of developmental pediatrician, psychologist, educational specialist, speech pathologist, occupational therapist, psychiatrist social worker and most importantly a behavioral therapist. Present interventions include the following: • Psychoeducational and behavioral – Teach–treatment and education of autistic and related communication handicapped children – Applied behavioral analysis – Alternative communication – Social skills teaching – Parental involvement • Psychopharmacological – Conventional antipsychotics – Selective serotonin reuptake inhibitors – Beta blockers – Mood stabilizers • Less traditional or complimentary (which are of doubtful value) – Megavitamin therapy – Gluten and casein free diet – Sensory and auditory integration. Autism is perhaps the most difficult developmental disability and requires all the skills to communicate and counsel the parents. It is a lifelong disability causing severe anxiety, physical and mental exhaustion. Parents need all the help and support that professionals can give for many years. Majority of the children require special education because of intellectual disability. Seizure disorders should be appropriately treated with antiepileptic drugs. Psychiatrist should actively be involved in evaluation and follow up when medications are prescribed.

Diseases of Central ervous ystem

Karyotyping is advised to rule out genetic syndromes such as fragile X syndrome.

411

vip.persianss.ir

D

A

D

D





Sunil Karande Learning disabilities (LD) and attention deficit hyperactivity disorder (ADHD) are two common neurodevelopmental disorders that occur in up to 5–10% of children.

•

t

t

Tex book of Pedia rics

Learning isabilities and ttention eficit Hyperactivity isorder

6.17

D

D

Learning isability efinition •

E

Learning disability is a group of neurodevelopmental disorders, which manifest in childhood as persistent difficulties in learning to efficiently read (dyslexia), write (dysgraphia) or do mathematical calculations (dyscalculia) despite normal intelligence, conventional schooling, intact hearing and vision, adequate motivation and sociocultural opportunities.

tiopathogenesis

F

C

linical eatures

ti e

n U

D

A history of language delay, or of not attending to the sounds of words (trouble playing rhyming games with words, or confusing words that sound alike), along with a family history, are important red flags for dyslexia. Children with LD present with persistent “academic problems” such as reading slowly and incorrectly, skipping lines while reading aloud, making repeated spelling mistakes, untidy/ illegible handwriting with poor sequencing and inability to perform even simple additions and subtractions. These children invariably fail to achieve school grades that are commensurate with their intelligence.

iagnosis



A conclusive diagnosis of LD cannot be made until the child is about 7–8-years old. Unlike children having LD, some children are “normal late developers” who by the age of 7–8 years on their own outgrow their learning problems. An evaluation for LD should be considered for all children presenting with learning problems in preschool/school. A 412 multidisciplinary team comprising of pediatric neurologist, counselor, clinical psychologist and special educator is needed to assess each child referred for poor school performance. The sequence of evaluations done is as follows:

G R

V d •

Learning disability is believed to be a result of functional disruption in neural systems and is genetically inherited. Dyslexia or “specific reading disability” is the most common and most carefully studied, affecting 80% of all those identified as learning-disabled. Children with dyslexia have deficits in “phonologic awareness”; viz. they have difficulty developing an awareness that words, both written and spoken, can be broken down into smaller units of sound and that in fact, the letters constituting the printed word represent the sounds heard in the spoken word.

Audiometric and ophthalmic examinations are done to rule out hearing and visual deficits, as they are common causes of poor school performance. Also, children with non-correctable hearing and visual deficits (of ≥ 40% disability) do not qualify for a diagnosis of LD, but are diagnosed as having “hearing impairment” and “visual impairment” respectively The pediatric neurologist then takes a detailed clinical history and does a detailed clinical examination to exclude medical causes, e.g. hypothyroidism, chronic lead poisoning; and neurological causes, e.g. cerebral palsy, Wilson disease; and to identify behavioral causes, e.g. ADHD, depression, conduct disorder (CD) or oppositional defiant disorder (ODD), of poor school performance The counselor takes a detailed interview of the parents/ family members to rule out that emotional problem due to stress at home/school is not primarily responsible for the child’s poor school performance The clinical psychologist conducts the standard Wechsler Intelligence Scale for Children test to determine that the child’s global intelligence quotient score is average or above average (≥ 85). This helps to exclude children having “borderline intellectual functioning” IQ score (71–84) also known as “slow learners” who also present with poor school performance The special educator assesses the child’s academic achievement by administering a standard educational test (e.g. Curriculum Based Test, Woodcock-Johnson Tests of Achievement) to assess the child’s performance in areas such as reading, spelling, written language and mathematics. An academic achievement of up to 2 years below the child’s actual school grade placement or chronological age is considered diagnostic of LD The child psychiatrist plays an important role in confirming the diagnosis of ADHD, a comorbid condition found in 12–24% of children with LD.

•

•

•

Treatment The cornerstone of treatment of LD is remedial (special) education and it should begin early when the child is in primary school. Using specific teaching strategies and teaching materials, the special educator formulates an Individual Educational Program to reduce or eliminate the child’s deficiencies in specific learning areas of reading, writing and mathematics identified during the child’s educational assessment. The child has to undergo remedial education sessions twice or thrice weekly for a few years to achieve academic competence. However, even after adequate remedial education, subtle deficiencies in reading, writing and mathematical abilities persist.

vip.persianss.ir

­



C t S t



 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Table 6.17.1 Diagnostic and statistical manual of mental disorders-IV-revised criteria for diagnosing attention deficit hyperactivity disorder Behavior domains Inattention (9 criteria) Hyperactivity-impulsivity (9 criteria) Hyperactivity (6 criteria) 1. Careless with details 1. Fidgets with hands or feet or squirms in seat 2. Fails to sustain attention in tasks 2. Leaves seat when should be seated 3. Appears not to listen 3. Runs about or climbs excessively and inappropriately 4. Does not finish instructed tasks 4. Cannot play or engage in leisure activities quietly 5. Poor in organizing tasks 5. Always “on the go” or “driven by a motor” 6. Avoids tasks that require sustained mental effort 6. Talks excessively 7. Loses things Impulsivity (3 criteria) 8. Easily distracted by extraneous stimuli 1. Blurts out answer before question is completed 9. Forgetful in daily activities 2. Has difficulty awaiting turn 3. Interrupts or intrudes others’ conversations or games Note: Criteria for subtypes is 6/9 on either list, or for combined subtype, 6/9 on both lists - together with certain pre-requisites (see text)

413

 

E

There is no data available from India of the prevalence rates of ADHD. Data from Western countries indicate that 8–12% of school-going children have ADHD.



D

A D

D

n U

pidemiology

Its diagnosis is made by ascertaining whether the child’s specific behaviors meet the diagnostic and statistical manual of mental disorders-IV-revised criteria (Table 6.17.1). These criteria define three subtypes of ADHD: 1. ADHD primarily of the inattentive type (ADHD/I) 2. ADHD primarily of the hyperactive-impulsive type (ADHD/HI) 3. ADHD, combined type (ADHD/C). A child meets the diagnostic criteria for ADHD by documentation of: • Presence of at least six of the nine behaviors described in the inattentive domain (ADHD/I), or the hyperactive/ impulsive domain (ADHD/HI), or in both domains (ADHD/C), and these behaviors should be occurring “often” and to a degree that is maladaptive and inconsistent with the child’s developmental level • Presence of these behaviors in two or more settings (for example, at home and at school) for at least past 6 months

V d

ti e

Attention deficit hyperactivity disorder is a neuro developmental disorder characterized by persistent hyperactivity, impulsivity and inattention that significantly impairs educational achievement and/or social functioning.

G R

iagnosis

D

Practice Guidelines

efinition

l

A history of being asked to leave preschool for disruptive behavior, or of being avoided by peers for play activities, along with a family history, are important red flags for ADHD.

It is well known that favorable outcome of LD is dependent on early diagnosis, regular remedial education for a few years, availing the necessary provisions, and a supportive home and school environment.

ttention eficit Hyperactivity isorder

N

linical eatures

C

Prognosis

ervous ys em

The ”academic problems” of children with LD also have an adverse impact on their self-image, peer and family relationships and social interactions.

Every pediatrician can facilitate early detection of LD by enquiring about every child’s school performance during a consultation and guiding the parents for getting their child’s psychoeducational assessment done, when LD is suspected.

D

E

omplications

Recent functional MRI brain studies indicate that the disorder may be caused by atypical functioning in the frontal lobes, basal ganglia, corpus callosum and cerebellar vermis. Pharmacological studies have also implicated dysregulation of frontal-subcortical-cerebellar catecholaminergic circuits (dopamine and norepinephrine neurotransmitter systems) in the pathophysiology of the disorder. Family studies have provided strong evidence that genetics plays a major role in conferring susceptibility to ADHD. Studies have indicated that low-birth weight and psychosocial adversity (for example, severe parental discord, low-social class, and foster placement) are predisposing risk factors for ADHD. F

C



tiopathogenesis

iseases of en ra

The management of LD in the more time-demanding setting of secondary school is based more on providing provisions (accommodations). These provisions, e.g. exemption from spelling mistakes, availing extra time for all written tests, dropping a language and substituting it with work experience, dropping algebra and geometry and substituting them with lower grade of mathematics and work experience, are meant to help the child cope up in a regular mainstream school.

vip.persianss.ir

C

Between 18% and 35% of children with ADHD have one or more associated psychiatric disorders such as anxiety disorder, depression, ODD and CD. Also, up to 15–20% of children with ADHD have associated LD. It is known that children with combined LD and ADHD have more severe learning problems than children who have LD but no ADHD, and also more severe attention problems than children who have ADHD but no LD.

Treatment Optimal treatment of ADHD requires integrated behavioral and medical (combined) treatment.

Behavioral Therapy Parents are taught how to • Reinforce positive behaviors by praise or by using daily contingency charts (star or “happy face” charts) • Extinguish negative behaviors by active ignoring • Effectively punish for intolerable behaviors. Simple psychoeducational interventions at school such as seating the child near the teacher to minimize classroom distractions, or assigning a specific teacher to review daily assignments with the child are effective in improving the behavior and academic performance of affected children.

Prognosis



Practice Guidelines

ey

M

Pediatricians should initiate an evaluation for ADHD when a child presents with symptoms that include academic underachievement and failure, disruptive classroom behavior, inattentiveness, poor self-esteem, or problems with establishing or maintaining social relationships. Those familiar with its management can even initiate the medication management, but will need to liaise with a child psychiatrist/developmental pediatrician when intolerable side effects or treatment failure occur or if comorbid conditions are suspected.

essages

B

­

• With appropriate remedial education and provisions children with LD achieve academic competence in regular mainstream schools. • Optimal treatment of ADHD requires integrated behavioral and medical treatment.









G R

K

ti e

n U

It is well known that favorable outcome of ADHD is dependent on early diagnosis and treatment and a supportive home and school environment. If ADHD remains undetected the child may experience academic failure, rejection by peers, and develop low-self-esteem. Recent studies suggest that 30–60% of affected children continue to show significant symptoms of ADHD into adolescence and young adulthood. Adolescence may bring about a reduction in the over-activity but inattention, impulsiveness and inner restlessness remain. It is now well recognized that the presence of a child with ADHD results in increased likelihood of disturbances to family and marital functioning, disrupted parentchild relationships and increased levels of parent stress, particularly when ADHD is comorbid with ODD or CD.

V d

Medications Medications are not recommended for use in children who are below 6 years of age. Methylphenidate (MPH) and atomoxetine are the two drugs, which are being currently prescribed. Most children with ADHD improve on the stimulant MPH and maintain their improvement without intolerable adverse events. Short-acting MPH is now available in our country. Its behavioral effects begin within 30 minutes of oral administration and last for 3–5 hours. The daily dose should be individualized by titration and careful monitoring and it ranges from 5 mg to 20 mg twice daily to three times daily. Side effects include anorexia, stomach ache, headache, irritable mood, tics and sleep difficulties. These side effects are usually mild and responsive to dose adjustment and often abate with continuous use. Continuous use has been associated in some children with slowing of physical growth (approximately 1 cm/year during the first 1–3 years of treatment), which is transient and of unclear cause. In Western countries, long-acting MPH is available and it permits once-daily administration (18–54 mg) and its behavioral effects last for 10–12 hours. It is used to ensure compliance in children who feel embarrassed to take medication in school. Atomoxetine a non-stimulant highly selective nora drenaline reuptake inhibitor is a relatively new drug with efficacy comparable to MPH. The starting once-daily dose is 0.5 mg/kg/day and increased after 4 days to 1.2–1.4 mg/kg/day. Its most commonly reported adverse effects are transient and include dyspepsia, nausea, vomiting, decreased appetite and weight loss. In general, medication is best titrated against 414 desirable effects such as behavioral control, improved educational achievement and peer group relations, and development of intolerable adverse effects. Medication

omplications

ibliography 1. Biederman J, Faraone SV. Attention-deficit hyperactivity disorder. Lancet. 2005;366:237-48. 2. Demonet JF, Taylor MJ, Chaix Y. Developmental dyslexia. Lancet. 2004;363:1451-60.

•

can be discontinued on Sundays and school holidays. Also, periodic (yearly) discontinuation for a brief period during summer vacations is often used to reaffirm the need for continuing medication.



•

Presence of some symptoms of ADHD before 7 years of age Clear evidence of clinically significant impairment in academic or social functioning, or in both. These symptoms not occurring exclusively during the course of a pervasive developmental disorder, schizophrenia or another mental disorder.



t

t

Tex book of Pedia rics

•

vip.persianss.ir



n U

G R

There is no definitive clinical picture as GDD is a symptom complex associated with a variety of conditions. Child invariably presents with significant delay in two or more major developmental domains. Expected features include microcephaly or occipitofrontal circumference 2 SD below the mean, dysmorphic features and identifiable neurological deficits. Global developmental delay from prenatal causes may be associated with growth retardation, multisystem involvement (congenital heart disease, hepatosplenomegaly, seizures, etc.). Associated sensory problems in hearing, vision and/disequilibrium reactions may be seen with a specific etiological yield. Adaptive problems and behavioral alterations are common in both prenatal and postnatal insults. A family history of miscarriages or difficulties in learning in family members and a history of intrapartum complications may be elicited in some children. A history of birth asphyxia, seizures and abnormal neurological findings in the newborn period and infancy are often predictors of GDD, especially in the presence of dysmorphism. Clinically identifiable features emerge as the child grows and follow-up evaluation of babies-at-work often points to an underlying condition. Clinical evaluation of the growing child with GDD must include a follow-up for global mental functions, particularly looking at the evolving capacities. These include: the degree/quality of awareness and alertness, orientation, intellectual functions including cognitive development, 415



There is a paucity of studies related to GDD in the population. The relatively new concepts and the prevailing controversies regarding terminology, coupled with the difficulties in ascertaining diagnosis in young children whose capacities are evolving, pose challenges to epidemiological studies. The exact incidence and prevalence in India is not known. The double burden of infectious diseases along with poverty correlates such as malnutrition and risks associated with urbanization, exposure of mothers and infants to toxic substances may contribute to the growing numbers of children with GDD in developing countries.



E

pidemiology

N

Clinical Picture





Global developmental delay is defined as “a significant delay in two or more of the following developmental domains: gross/fine motor, speech or language, cognition, social/ personal-adaptive and activities of daily living (ADLs)”. In practice, however, the term GDD is usually reserved for children younger than 5 years of age, showing the symptom complex with an as yet undefined nosologic profile that includes poor cognitive and psychomotor functions, whereas the term “mental retardation” or “intellectual disability” is applied to older children when IQ testing is more reliable and valid.

Evidence from several studies during this decade points to the heterogeneous nature of the underlying diseases. Studies from China and India suggest that the predominant categories of cerebral dysgenesis, chromosomal disorders, multiple malformation syndromes, hypoxic ischemic encephalopathy and antenatal toxin exposures account for global functional delays in nearly two-thirds of children. At present time, our knowledge of the prime movers for observed etiology (e.g. gene responsible for a particular cerebral dysgenesis) may not be sufficient and one may be describing in pathogenic terms (e.g. periventricular leukomalacia and cerebral dysgenesis). However, an approach of conceptualizing etiology as a diagnostic aid that can translate into useful clinical information for prognosis and management is gaining momentum, consistent with practice parameters and guidelines proposed by the Child Neurology Society and the American Academy of Neurology.

V d

ti e



Definition

tiopathogenesis





Developmental disabilities are a group of interrelated neurologic disorders occurring in childhood, characterized by delay, deviation or dissociation in streams of early child development. Developmental delay is simply a complaint referring to a condition whereby infants or young children do not achieve developmental milestones at the expected age. The impact of impairments responsible for the delay in the major streams of development including motor, perceptual, speech, cognition and behavior is seen as functional limitation and decreased ability of the child to participate in expected activities as one grows older. Deviation is said to be present when a child with developmental lag also shows abnormal or distorted features such as persistence of neonatal reflexes and inability to progress from one stage of development to another. Dissociation refers to developmental lag in some domains being more than in others. The quality or quantity of changes in performance/development appears locked in one stage in a certain domain and progressing in another over a period of observation. Global developmental delay (GDD) constitutes a subset of developmental disabilities.

E

Sunanda K Reddy

S

I

E





Global Developmental Delay and arly ntervention

Diseases of Central ervous ystem

6.18

vip.persianss.ir



I

E

anagement and arly ntervention Practice Guidelines 416

Early intervention is a term traditionally associated with the services managed by professionals with a transdisciplinary



­

­



n U

The basic screening may include thyroid profile testing, TORCH screen and MRI/CT scan of brain. One should consider referring children to care settings with good diagnostic facilities whenever etiological diagnosis is important to prognosticate and provide appropriate intervention. Neuroimaging and karyotyping may be done for all with dysmorphism and congenital anomalies. Metabolic testing (e.g. serum amino acids) and biochemical analysis are additionally undertaken in tertiary settings for specific management. A suggested approach as a diagnostic strategy linked to intervention for children with GDD is described in Flow chart 6.18.1. The contemporary approach to diagnostic assessment of children with developmental disorders is based on the guiding principle that diagnostic assessment is a “dead end” unless it generates a focus/plan for intervention.

M

V d

ti e

nvestigations

G R



I





Establishing an accurate etiologic diagnosis is not always possible, nevertheless the diagnosis process is absolutely necessary for accurate genetic counseling and rehabilitative planning. Early diagnosis of GDD is possible by regular newborn follow-up and periodic evaluation for developmental lag in the first 2 years of life. A developmental assessment based on in-depth history and clinical examination is paramount. Quite often, a detailed developmental history including prenatal, perinatal, neonatal history and developmental patterns in infancy may suggest diagnosis. Evidence of regression and poor capacity for self-help/ADLS expected for the age calls for a detailed diagnostic evaluation. Tests for adaptive functioning and psychomotor development using standard developmental scales appropriate for the age must be undertaken. The Bayley Scales of Infant Development, Wechsler Scales (WPPSI-III), Vineland Adaptive Behavior Scale are some tests administered for the purpose in children less than 5 years of age. A diagnostic formulation in terms of maturation and development can also form the basis for the early intervention (EI) and identification of special education needs (SEN). The process also confirms diagnosis by ruling out conditions such as autism, ADHD, etc.

approach for a child with developmental disability during the preschool years to help overcome the developmental lag. In the resource-poor settings, EI or help in the early years starts as a high-risk follow-up clinic linked to primary care and extends to become a cost-effective family-centered model. Drawing from the fundamental neurodevelopment perspectives, the caregiver who is often a developmental therapist or a pediatrician, works to create a developmental setting in which the key to a child’s progress is mother-child participation. The basis for EI is that there are critical periods of development and interventions, to be most effective, should happen before those periods. The second reason is that the CNS has the feature of neuroplasticity or intrinsic ability of neural structure to form functional neuronal networks for new learning. Helping the child with GDD during the early years is critical for later development. Developmental gains may not always be measurable in quotients but some benefits are obvious. Early intervention prevents developmental decline and deviant patterns of development that are seen in GDD. Professional intervention is particularly effective in addressing the motor and sensory impairments and seizures that are often seen as comorbidities. Even in a child with severe impairment providing sensory inputs and appropriate experiences early in life contributes to information gains (early infant stimulation). Guidance to parents is a crucial component of EI. An early acceptance of the problem and an early involvement to help the child overcome the biological disadvantage helps parents understand their child better and cope with the difficult situations. In children with special learning needs from GDD, EI brings down inappropriate behaviors and improves social competence. The components for EI depend on the primary impair ment and can vary with the child’s needs. Developmental therapy focuses on mobility, manipulation and communi cation. In the presence of GDD without significant motor impairment, activities promoting sensory integration, balance and communication become important. Challenging behaviors (aggression, oppositional defiant behaviors) may require behavior management techniques as well as environment engineering. Medication is of little use, except for short term psychopharmacological management in select cases. Family counseling and respite care should be integral part of EI and management plan. Support care instituted early with interdisciplinary management should be multimodal to address the diverse needs of the child: health, education, recreation, social activities and direct efforts to ameliorate the effects of associated impairments and behaviors in order to improve functional outcomes and participation. A program for “early infant stimulation” is facilitated by early identification of problems at birth, or at discharge from nursery. Early and intensive support through home visits by health workers, and multicomponent stimulation programs

Diagnostic valuation E

d

c

extbook of Pe iatri s T

global psychosocial functions or interpersonal skills to establish reciprocal social interactions, temperament, attention, energy and drive functions. Abnormalities in these areas constitute soft neurological findings for GDD and the developmental variability must alert the pediatrician to look for developmental deviations or dissociation.

vip.persianss.ir

N

 

Diseases of Central ervous ystem

Flow chart 6.18.1 Diagnostic strategy linked to intervention for children with global developmental delay

S

V d

ti e

n U

G R

417

vip.persianss.ir

extbook of Pe iatri s



­

B













1. First LR, Palfery JS. The infant or young child with developmental delay. NEJM. 1994;330:478-83. 2. Majnemer A, Shevell MI. Diagnostic yield of the neurologic assessment of the developmentally delayed child. J Pediatr. 1995;127:193-9. 3. Pediatrician’s role in the development and implementation of an Individualized Education Plan (IEP) and/or and Individual Family Service Plan (IFSP). Pediatrics. 1999;104:124-7. 4. Shevell MI, Ashwal S, Donley D, et al. Practice parameter, evaluation of the child with global developmental delay: report of the quality standards subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology. 2003;60:367-80. 5. Shevell MI, Majnemer A, Rosenbaum P, et al. Etiologic yield of subspecialists’ evaluation of young children with global developmental delay. J Pediatr. 2000;136:593-8. 6. Srour M, Mazer B, Shevell MI. Analysis of clinical features predicting etiologic yield in the assessment of global developmental delay. Pediatrics 2006:118:139. 7. Tang M, Lin C, Lin W, et al. The effect of adding a home program to weekly institutional based therapy for children with undefined developmental delay: a pilot randomized clinical trial. J Chin Med Assoc. 2011;74:259-66. 8. Tikaria A, Kabra M, Gupta N, et al. Aetiology of global developmental delay in young children: experience from a tertiary care centre in India. Nat Med J India. 2010;23::324-8.





In the field of developmental impairments, prevention includes prospective interventions to avert progression to disabilities. Prevention at the primary level is possible by efforts to 418 reduce the contributory illness and determinants of GDD and/or mental retardation. Good prenatal services and



Prevention

ibliography





n U

Global developmental delay more often than not, evolves into a more specific developmental disorder, the commonest being intellectual disability (MR). The ultimate impact of GDD is shaped by the nature and extent of the impairments, the developmental patterns and cadence of change, services available for EI, family supports and other environmental factors. Some infants with mild delay may do well in terms of functioning in later years due to neuroplasticity and effect of CNS maturation coupled with EI. However, the majority may be diagnosed in late childhood as having communication disorders, autism, specific syndromes such as Rett syndrome, etc. Those with limitations in activities of daily living (ADLs) and poor adaptive abilities may grow into adults requiring extensive supports. Appropriate management with a lifespan approach and social support services may help them perform simple tasks in supervised settings and live in the community.

V d

ti e

Prognosis

G R

obstetric care, good nutrition and administering measlesmumps-rubella vaccine to all girls, fortification of food with iodine, iron, folic acid, and general awareness campaigns for promoting health seeking behaviors, as well as screening for early identification, can go a long way in decreasing the magnitude of GDD. Approaches and interventions for prevention at various levels are summarized in Table 6.18.1. Primary prevention strategies remain the best for developing countries.



that are culture-sensitive and context-specific can promote developmental outcomes. The key care provider and the team of professionals (developmental therapist, speech therapist, pediatrician, clinical psychologist and other members) in a transdisciplinary/interdisciplinary mode of EI look to addressing risks and enhancing promotion of health and abilities as well as to prevent secondary problems through direct support to the child and the family.



T

d

 

c

Table 6.18.1 Prevention of global developmental delay Level Approach Interventions Primary prevention (Preventing Health promotion •  Health education, especially for adolescent girls the occurrence of MR/GD) •  Improvement of nutritional status in the community, optimum health care facilities •  Improvements in pre, peri and postnatal care Specific protection •  Universal iodization of salt •  Rubella immunization for girls and women before pregnancy •  Folic acid administration in adolescence and early pregnancy •  Genetic counseling •  Prenatal screening for congenital malformation and genetic disorders •  Detection and care of high-risk pregnancies •  Universal immunization for children Secondary prevention (Health Early diagnosis and treatment •  Neonatal screening for treatable disorders Disease Progression) •  Intervention for babies ‘at risk’ for neurodevelopmental impairments •  Early detection of developmental delay and early intervention Tertiary prevention Disability limitation and •  Stimulation, training and education, and vocational opportunities (preventing complications and rehabilitation •  Mainstreaming/integration maximization of function) •  Support for families •  Parental self-help groups Source: Girimaji S et al. Mental Retardation: From Knowledge to Action (2001), WHO Document

vip.persianss.ir











7.1 7.2 7.3 7.4 7.5 7.6















Section

7

Diseases of Cardiovascular System

V d

G R

Section Editor : Srikanta Basu

Clinical Approach to Pediatric Cardiology: S Srinivasan Congenital Heart Disease: R Krishna Kumar Rheumatic Fever: Savitri Shrivastava Congestive Heart Failure: Diagnosis and Management: Anita Saxena Diseases of Endocardium, Myocardium and Pericardium: Srikanta Basu Cardiac Arrhythmias in Children: S Srinivasan

n U

ti e

vip.persianss.ir

cs





Clinical Approach to Pediatric Cardiology S Srinivasan

The objectives of clinical examination of the cardiovascular system in newborns, young infants and older children are: 1. To ascertain without delay the presence of any life threatening critical congenital heart disese (CHD) that jeopardize the child’s survival 2. To institute appropriate resuscitative measures before referral 3. To identify the underlying cardiac conditions in terms of structural involvement 4. Its etiology-congenital/acquired, genetic (chromosomal/ dysmorphogenetic) 5. To assess its severity in terms of functional restriction 6. To document the presence or absence of other related complications and its impact on child’s nutritional, growth and developmental status.



General Physical Examination









ti e



n U

Cardiac Symptoms Symptoms of CHD can be very varied and subtle at times and may manifest anytime from fetal period to adulthood (Table 7.1.2). Many of these symptoms may not be the 420 presenting problem and therefore it has to be elicited by asking pertinent questions.

G R

V d

History Taking The mother must be allowed to give her own account of the presenting symptoms, which compelled her to bring her child to medical attention and narrate the evolution of these symptoms from the time of their onset. Only open-ended questions must be used to get the most relevant and useful information. The historical points of interest, of course, will vary considerably, depending upon the age of the patient, and the presenting signs, symptoms and complaints. If the child is brought in a severe state of breathlessness or cyanosis or shock, precious time must not be wasted in taking elaborate history. Neonates with marked shortness of breath, cyanosis and pallor with lethargy need immediate attention, and clinical and investigative assessment to rule out life-threatening, acute cardiac emergency states, e.g. ductus dependent congenital cardiac lesions that restrict pulmonary or systemic blood flow. Other relevant and important details in history taking that have bearing on the diagnosis of the cardiac condition, its severity, and in planning treatment and counseling have been summarized in Table 7.1.1. Moreover, while taking the history, one should take note of the evolution of symptoms according to chronology, mode of presentation, duration, progression, severity, relieving factors, aggravating factors, associated symptoms and functional impairment.

Examination of an infant and young child needs a lot of tact, and the general principles of examining a child should be applied. Although it is an usual routine to follows a definitive order in clinical examination, in a young child, such an orderly examination may not be always possible because of their apprehensive and anxious nature. In a sleeping child, it is better to perform auscultation of the precordium for the nature of cardiac sounds and presence of murmurs and other sounds. Infants and young children must be comfortably seated in the mother’s lap with their dresses removed earlier before sitting in front of the examiner. The standard format of general physical examination has been summarized in Table 7.1.3. It is important to remember that vital signs should be examined first before undertaking detailed cardiac examination. The pertinent points to note are airway, breathing (rate, type and sign of distress), circulation (capillary filling time, details of pulse, blood pressure) and temperature.

Cardiac Examination Precordium

The objectives of inspection of the chest is to identify chest wall asymmetry if present, in the form of precordial prominence, pectus excavatum, carinatum, kyphosis, scoliosis, Harrison’s sulcus, etc., abnormal pulsations in the neck, suprasternal notch, parasternal region, epigastric region and over the back, the location of apical impulse—left side or right side, and scars of previous operative procedures.

Jugular Venous Pulse and Pressure The jugular venous pulse (JVP) waveform consists of three positive upstrokes—a, c and v and two negative waves—x descent after a and c waves and y descent after v wave. The abnormalities of various wave forms have been summarized in Table 7.1.4.

Arterial Pulse Pulse is the peripherally transmitted waveform of propa gated arterial blood from the aortic root along the arterial tree generated by systolic contraction of left ventricle. The travel speed of the arterial pulse wave is 10 times faster than that of the blood column. The importance of palpating the arterial pulse at various sites, e.g. both radials, femorals, brachials, popliteal, posterior tibials, dorsalis pedis, carotids and superficial temporal arteries should be kept in mind at the beginning of each clinical posting and perhaps throughout life so as to ensure that one does not miss coarctation of aorta and thromboembolic phenomena need to be repeatedly emphasized to the students at every ­

e

t

f

T x book o P

ed

i

atr

i

7.1

vip.persianss.ir

seases f ard ar

ul

vasc

io stem

Sy

 

History of functional limitations Treatment history

o C

Family history

Di

Table 7.1.1 Important documentation of history relating to causative or precipitating factors History of abnormal facial features Many chromosomal disorders like Down’s syndrome, Turner’s syndrome, other trisomies and other genetic, metabolic or minor or major congenital and isolated malformations may indicate the presence of associated congenital heart diseases. malformations Past history Any physical symptoms, health problems, hospital visits or admissions in the past for recurrent chest infections, bacterial endocarditis, symptoms suggestive of rheumatic fever such as arthritis, abnormal movements, tuberculosis and delayed motor development, rashes, skin peeling in palms, soles, tips of fingers and toes, sore throat preceding onset of joint pains Antenatal history •  History of acute or chronic infections in various trimesters—fever, rashes •  Other maternal illness—metabolic disorders like gestational diabetes, autoimmune disorders like SLE •  Drugs—phenytoin, lithium, other anticonvulsants, etc. •  Substance abuse; alcohol, addictive drugs, etc. •  Exposure to irradiation, pollutants, others Birth history, Delayed crying and breath initiation, bluishness, sucking and feeding difficulty, lethargy, importance of finding out immediate postnatal and neonatal the gestational age, birth weight, need for resuscitation, need for breathing and oxygen support, prolonged stay history** in neonatal unit, need for tube feeding or intravenous fluids, type of medications given during neonatal stay in the hospital and subsequent to discharge Nutritional history Children with cardiac conditions with frequent heart failure or recurrent infections will have loss of appetite and their nutritional intake will be greatly compromised. History of growth and •  Growth impairment and failure to thrive in the first few years of life is commonly noted in moderate-to-severe developmental impairment cardiac structural lesions. Tall stature or short stature, delayed gross motor milestones have a bearing on underlying cardiac disease. •  In a child with tall stature, the possibility of Marfan’s syndrome must be considered. In a female child with short stature, Turner’s syndrome and associated coarctation of aorta must be ruled out. •  Moderate-to-severe congenital heart diseases will delay the gross motor milestones in relation to other areas of development. Chromosomal and dysmorphogenetic syndromes with associated heart diseases may exhibit global developmental delay. •  Record any of the following present in the family members: sudden death, SIDS, rheumatic fever, structural or congenital cardiac abnormalities in siblings, genetic or dysmorphogenic syndromes in the family •  The estimated recurrence risk of CHD is 2–6% when a previous sibling is affected, nearly 30% with two siblings affected and 3% if one of the parents is affected. School attendance and absenteeism, academic performance, inability to participate in games and sports activities Past and present treatment details

Personal/socioeconomic history

In a developing country with majority of the population being below the poverty line, long-term treatment facilities including surgical corrections at appropriate age may not be possible. Hence, children and adults at all ages may have congenital heart diseases with or without advanced complications like PAH. ** Preterms have higher incidence of PDA. These preterms also go into heart failure early in their first week with even moderate-sized defects with left to right shunts. Abbreviations: SLE, Systemic lupus erythematosus; SIDS, Sudden infant death syndrome; CAD, Congenital heart disease; PAH, Pulmonary arterial hypertension; PDA, Patent ductus arteriosus

Precordial Palpation The learning objectives of palpation of the precordium are: (1) to identify the site and type of apical impulse whether normal or abnormal, (2) to identify the presence of abnormal pulsations, heaves and thrills in specified precordial sites—left parasternal and epigastric regions, suprasternal notch, second left intercostal space (LICS) and (3) to ascertain enlargement of right ventricle, left ventricle or both, or dilatation of great arteries— aorta and pulmonary arteries.

Apical Impulse Apical impulse refers to the outermost and lowermost definitive palpable outward thrust of cardiac impulse

in the lower precordium imparted by the anterior movement of left ventricular (LV) apex during early systolic contraction of the left ventricle. Its location, amplitude, duration, size, character or contour must be recorded. In the first 3–4 years of life after birth, the normal location of the apical impulse is in the fourth LICS 1–2 cm lateral to the midclavicular line (MCL). Between 4–8 years, the location is in the fifth LICS on the MCL. Beyond 8 years, it lies in the fifth LICS 1–2 cm or half an inch medial to the MCL. The apex beat is shifted to the left in cardiomegaly, scoliosis, pectus excavatum and contralateral pneumothorax or effusion. It is shifted on the right side in cases of congenital dextrocardia, acquired dextroposition (heart pushed or pulled to the right side) and diaphragmatic hernia. Table 7.1.6 summarizes 421 different types of apical impulse.

posting of theirs. The various types of abnormal pulses have been depicted in the Table 7.1.5.

vip.persianss.ir

cs

i atr

i ed e

t

f

T x book o P

Other Palpable Sounds The second heart sound (S2) may be palpable in pulmonary hypertension. The third heart sound (S3) may be rarely palpable in severe mitral regurgitation (MR) or dilated cardiomyopathy (DCM). The fourth heart sound (S4) is more often palpable in adults than in children in the following conditions: hypertrophic cardiomyopathy (HCM), severe aortic stenosis (AS), acute MR and long-standing hypertension. Suprasternal pulsations are seen and palpable in patent ductus arteriosus (PDA), AS, aortic regurgitation (AR), coarctation of aorta and aortic aneurysm. Pulsations in the parasternal area without a perceptible or palpable left parasternal lift is characteristic of right ventricular (RV) volume overload conditions like atrial septal defect (ASD),

tricuspid regurgitation (TR), moderate to large ventricular septal defect (VSD) without pulmonary arterial hypertension (PAH) besides hyperdynamic high output states. Epigastric pulsations are seen and the impulse is felt in RV volume overload states. Apart from these sounds, murmurs are also palpable in various areas.

Parasternal Heave Parasternal heave is both seen and felt by the ulnar border of the examiner’s hand over the left parasternal region Table 7.1.3 Proforma for general examination in a cardiac patient General appearance

Comfortable, consolable, state of distress

Posture/decubitus  

Table 7.1.2 Presentations of cardiac problems

Body build and proportion Nutritional and growth status

Anthropometry

Features of chromosomal/genetic/ Down’s syndrome, Turner’s dysmorphogenic syndromes syndrome, other trisomies, Williams syndrome, Noonan syndrome, etc. External isolated/multiple congenital external malformations

Short neck, webbing, polydactyly, syndactyly, camptomelia

Oral cavity, teeth and pharynx

Sore throat, caries

Skin

Rashes, erythema marginatum, nodules, desquamation

Joints

Rheumatic fever, rheumatoid arthritis

Abnormal movements

Chorea

Dependent edema

Sites–feet, legs, back, eyelids

Lymph nodes

Kawasaki’s disease, tuberculosis

Clubbing of fingers and toes

Cyanotic heart defects, bacterial endocarditis















Fetus (diagnosed by fetal echocardiography) •  Hydrops, arrhythmias, cardiomegaly Neonate and infant •  Heart failure (tachypnea with feeding or activities, labored breathing/ dyspnea, feeding difficulties, sweating on head), failure to thrive •  Cyanosis, circulatory failure, collapse, abnormal heart rate/rhythm, murmur, weak femoral pulses •  Noticeable excessive precordial activity or pulsations observed by mother, sleep disturbances, recurrent lower respiratory infections •  Dysmorphism, noncardiac congenital abnormalities Older child •  Heart failure (exercise intolerance, shortness of breath with activities), palpitation •  Increased precordial pulsations or prominence*, dizzy or fainting spells, syncope •  Chest pain on activity, recurrent lower respiratory infections, failure to thrive, murmur •  Hypertension, as a part of multisystem disease (rheumatic fever, Kawasaki’s disease), seizures (presenting as a complication of cyanotic heart disease), cyanotic spells, squatting

General health

Central cyanosis, differential cyanosis

 

Table 7.1.4 Abnormal JVP and its clinical interpretation “a” wave abnormalities Large “a” waves

Forcible contraction of right atrium against obstruction Tricuspid stenosis, RA myxoma at AV valve area Increased resistance offered by noncompliant right Severe PAH, PS (moderate-to-severe), RV cardiomyopathy, acute ventricle pulmonary embolism

Cannon or very large Contraction of right atrium against a closed tricuspid Tricuspid atresia, AV dissociation, complete heart block giant “a” waves valve

Absent “a” waves

Regular cannon waves

Junctional rhythm, isorhythmic AV dissociation

Irregular cannon waves

Complete heart block, classic AV dissociation, ventricular pacing, ventricular ectopics

Ineffective atrial contraction

Atrial fibrillation, marked sinus tachycardia

“v” wave abnormalities Large “v” waves

422

Rapid and excessive RA filling

TR, TAPVD, ASD with MR

Abbreviations: JVP, Jugular venous pulse; AV, Atrioventricular; RA, Right atrial; PAH, Pulmonary arterial hypertension; PS, Pulmonary stenosis; RV, Right ventricular; TR, Tricuspid regurgitation; TAPVD, Total anomalous pulmonary venous drainage; ASD, Atrial septal defect; MR, Mitral regurgitation

vip.persianss.ir

Di seases

 

Table 7.1.5 Abnormal pulses Pulsus parvus Low-volume pulse with slow rise; its presence suggests the possibility of severe AS or severe low-output states like severe heart failure Pulsus tardus Slow rising pulse with a late peaking closer to the second sound and is reported in fixed LV obstructive lesions Hypokinetic pulse Weakly felt, small-volume pulse felt with difficulty in cases of low cardiac output states, such as shock, severe heart failure, myocarditis, cardiac tamponade, constrictive pericarditis, severe left heart obstructive lesions like MS and AS and aortic outflow obstructions like aortic arch syndromes and coarctation of aorta Hyperkinetic High amplitude, large-volume pulse with rapidly rising ascending wave felt in augmented high cardiac output states with (bounding) pulse decreased peripheral arterial resistance and wide pulse pressure, e.g. anxiety, exercise, fever, anemia, thyrotoxicosis, arteriovenous fistulas, beriberi, moderate MR, moderate-sized ventricular septal defects, AR, PDA Water hammer/ The pulse is elicited by suddenly and gently elevating the child’s arm above his/her shoulder while the radial pulse is continuously collapsing pulse felt by the palmar surface of the examiner’s hand instead of the usual three finger tips. It is a large-volume pulse with sudden and rapid upstroke with equally rapid downstroke. The term “water hammer” refers to a famous Victorian era English toy. Collapsing pulse indicates rapid and reverse diastolic run off into the left ventricle in AR and rapid run off to the peripheral vessels due to decreased systemic peripheral vascular resistance. AR, PDA, AP window, RSOV into the right ventricle and arteriovenous fistulas. Collapsing pulse in a cyanotic child suggests truncus arteriosus with truncal regurgitation, pulmonary atresia with AP collaterals, TOF with AP collaterals or PDA. Collapsing pulse is also felt in hyperkinetic states like anemia, fever, thyrotoxicosis, pregnancy and beriberi. Bisferiens pulse Pulse with two beats in a single systolic wave upstroke; when the examiner’s palpating finger feels two positive upward peaks during systole, it is termed as pulsus bisferiens, better appreciated over the carotids than radials. They indicate the presence of severe AR or AR with AS or HOCM. Corrigans pulse Prominently visible dancing pulsation over the carotids in AR Brisk/jerky pulse Pulse with a sharp, tapping upstroke with normal volume and downstroke is characteristic of HCM Anacrotic pulse It is felt in severe AS, is a small volume (low amplitude), slow rising (ana = up and crotos = beat) upstroke wave with late peaking. The anacrotic notch occurs during the early half of the ascent; earlier it is felt, severer is the AS. In severe MS, the notch is felt in the later half of the ascending limb. Dicrotic pulse It refers to pulse wave forms with two peaks in each wave, one in systolic ascending limb and the second prominent one in the descending limb of the wave during diastole. This is seen in cardiac conditions with low stroke volume and decreased peripheral arterial resistance—typhoid fever, LV failure, dilated cardiomyopathies and cardiac tamponade. Pulsus paradoxus It occurs when there is an exaggerated decrease in pulse volume and blood pressure more than 10 mm Hg recorded during inspiration. In normal individuals there is usually a normal physiological drop of systolic blood pressure during inspiratory phase ranging from 5 mm Hg to a maximum of 10 mm Hg, which may be appreciated by experienced clinicians on feeling the arterial pulse in the absence of tachycardia. Hence the term paradox used as adjective is not true. It is seen in cardiac tamponade, pericardial effusion, constrictive pericarditis, acute severe asthma and in conditions that obstruct superior vena cava return to right atrium as noted in anterior and superior mediastinal tumors. Pulsus alternans It refers to occurrence of alternating pulse of high and low amplitudes or volumes in the presence of normal rhythm and is indicative of moderate-to-severe LV failure. Pulsus bigemini, pulsus It refers to coupling of two or three beats followed by a pause with a stronger beat after the pause. It occurs in AV block and trigemini sinoatrial block with ventricular escape.

ar

ul

vasc

io

ard

f

o C stem

Sy

Abbreviations: AS, Aortic stenosis; LV, Left ventricular; MR, Mitral regurgitation; AR, Aortic regurgitation; PDA, Patent ductus arteriosus; AP, Aortopulmonary; RSOV, Ruptured sinus of Valsalva; TOF, Tetralogy of Fallot; HOCM, Hypertrophic obstructive cardiomyopathy; HCM, Hypertrophic cardiomyopathy; MS, Mitral stenosis; AV, Atrioventricular

Table 7.1.6 Characteristics of apical impulse Tapping apical impulse Palpable S1 noted in MS; it represents a hypokinetic, underfilled left ventricle. Tapping apical impulse is also felt in ASD. Hyperdynamic apex Suggests LV dilatation seen in high cardiac output states, and ventricular volume overloads seen in MR and AR, VSD or PDA. In hyperdynamic apex, the apical impulse, besides being shifted outwards and downwards, has increased amplitude with brisk upstroke, is felt in more than one ICS, usually over two or three ICS exceeding 3 cm. It is ill sustained with the duration of impact between one-third and half of systole. Heaving apex It is said to be present when the systolic thrust is sustained and increased in duration almost like a wave felt for more than two-third of systole with increased amplitude and is confined to one ICS. It is indicative of pressure overload and LVH seen in AS, systemic arterial hypertension or coarctation of aorta. Retractile apical impulse Indicative of constrictive pericarditis where there is systolic retraction away from the chest wall, and forward or positive diastolic thrust. Double apical impulse It suggests AS with AR, HOCM, LBBB and in adults LV aneurysm. Triple or quadruple impulse may be felt in HOCM. Absence of apical impulse Dextrocardia, pericardial effusion, obesity, apex under the rib, emphysema or large pleural effusion on the left side Abbreviations: S1, First heart sound; MS, Mitral stenosis; ASD, Atrial septal defect; LV, Left ventricular; MR, Mitral regurgitation; AR, Aortic regurgitation; VSD, Ventricular septal defect; PDA, Patent ductus arteriosus; ICS, Intercostal space; LVH, Left ventricular hypertrophy; AS, Aortic stenosis; HOCM, Hypertrophic obstructive cardiomyopathy; LBBB, Left bundle branch block

423

vip.persianss.ir

i

Heart Sounds

T x book o P

The first heart sound (S1) is produced by closure of mitral and tricuspid valves marking the beginning of systole. It is best heard over the apex and in mitral area. The S2 is produced by closure of semilunar valves of aorta and pulmonary arteries. The normal variable split of S2 is because the pulmonary component (P2) is normally heard 30 msec after the aortic component (A2) during inspiration (increased RV ejection time and pulmonary artery hangout interval) and within 20 msec during expiration. The S3 is low pitched,

e

t

f

ed

atr

cs

i

of the precordium overlying the heart. It indicates RV enlargement.

may be normally heard in early diastole and is caused by rapid ventricular filling. It may also be heard in athletes and pregnant women. If S3 occurs in a child with cardiac symptoms, one should suspect diastolic overload conditions or LV dysfunction (dilated left ventricle, decreased ejection fraction, constrictive pericarditis) or LV failure. The S3 gallop refers to an exaggerated sound with a cadence heard when significant tachycardia occurs with the above conditions. The S4 is often an abnormal sound and it is low pitched, late diastolic sound produced by forceful atrial contraction against certain degree of resistance and decreased ventricular compliance during late ventricular filling. The characteristics of various heart sounds have been summarized in Table 7.1.7.

 

Table 7.1.7 Characteristics of different heart sounds Intensity of S1

Cardiac conditions

Loud

Exercise, sinus tachycardia, short PR interval states like WPW syndrome, MS, tricuspid stenosis, atrial myxoma

Soft

Long PR interval, pericardial effusion, MR, TR, calcific MS, myocarditis and cardiomyopathy, proximal LBBB

Split S1

Ebstein anomaly, RBBB, large ASD

Intensity of S2 Loud S2

Soft S2

Increased A2: systemic hypertension, aortic root dilatation, L-TGA, TOF

Decreased A2: severe AS, AR, calcific aortic valve

Increased P2: pulmonary hypertension, ASD

Decreased P2: PS, TOF, TGA

Splitting of S2

Cardiac conditions

Wide (both A2 and P2 audible in inspiration and expiration)

Due to early A2 closure: VSD, MR Due to late P2 closure: PAH, PS, RBBB, pulmonary embolism, LV ectopic beat Increased hangout interval due to decreased impedance to pulmonary artery: ASD, PS, TAPVC, Ebstein, idiopathic dilatation of pulmonary artery

Wide and fixed split

ASD, severe RV failure

Paradoxical or reverse split

Delayed A2

P2, then A2 (split audible in expiration but not in inspiration)

Mechanical: AS, hypertension, CAD Electrical: Proximal LBBB; RV ectopics Decreased impedance to aorta: PDA, poststenotic dilatation in AS

Single S2

Absent A2: HLHS, aortic atresia Absent P2: Pulmonary atresia, truncus arteriosus, absent pulmonary valve Very soft/masked P2: TOF, TGA, Severe PS Synchronous A2 and P2: Severe PAH, PPH, Eisenmenger VSD

S3

424

LV overload states

MR, VSD, PDA

RV overload states

TR, ASD

LA S4 gallop

Aortic valvular stenosis, systemic arterial hypertension

Right atrial S4 gallop

PAH, PS, Ebstein’s anomaly, ASD, prolonged PR interval

Abbreviations: S1, First heart sound; WPW, Wolff-Parkinson-White; MS, Mitral stenosis; MR, Mitral regurgitation; TR, Tricuspid regurgitation; LBBB, Left bundle branch block; RBBB, Right bundle branch block; ASD, Atrial septal defect; S2, Second heart sound; A2, Aortic component of S2; L-TGA, Levotransposition of great arteries; TOF, Tetralogy of Fallot; AS, Aortic stenosis; AR, Aortic regurgitation; P2, Pulmonary component of S2; PS, Pulmonary stenosis; VSD, Ventricular septal defect; LA, Left atrial; PAH, Pulmonary arterial hypertension; LV, Left ventricular; TAPVC, Total anomalous pulmonary venous connection; RV, Right ventricular; CAD, Coronary artery disease; PDA, Patent ductus arteriosus; HLHS, Hypoplastic left heart syndrome; PPH, Primary pulmonary hypertension; S3, Third heart sound

vip.persianss.ir

Di seases

 

Table 7.1.8 Characteristics of organic heart murmurs Organic systolic murmurs Ejection

Mid/late systolic

MR TR VSD

AS PS ASD VSD

MVP

ard

f

o C

Pansystolic

Organic diastolic murmurs AR PR

MS TS Carey Coombs murmur of mitral valvulitis Austin flint murmur in AR

Severe MS

vasc

Presystolic

ar

ul

Mid-diastolic

io

Early diastolic

Sy

Secondary flow murmurs (ASD, VSD, PDA, MR , TR) Acyanotic

Secondary collaterals in

Combination of defects

•  PDA •  Aortopulmonary window rupture of sinus of Valsalva into right ventricle or pulmonary artery •  Coronary arteriovenous fistulas •  Systemic arteriovenous fistulas •  Peripheral pulmonary artery branch stenosis •  ALCAPA Cyanotic •  Pulmonary arteriovenous fistulas •  Truncus arteriosus •  Blalock Taussig shunt

•  TOF •  Aortic coarctation •  Aberrant LCA with RCA collaterals

•  VSD + AR •  AR + AS •  MR + AR/TR + MR + AR

stem

Organic continuous murmurs

Abbreviations: MR, Mitral regurgitation; TR, Tricuspid regurgitation; VSD, Ventricular septal defect; AS, Aortic stenosis; PS, Pulmonary stenosis; ASD, Atrial septal defect; MVP, Mitral valve prolapse; AR, Aortic regurgitation; PR, Pulmonary regurgitation; MS, Mitral stenosis; TS, Tricuspid stenosis; PDA, Patent ductus arteriosus; ALCAPA, Anomalous left coronary artery from the pulmonary artery; TOF, Tetralogy of Fallot; LCA, Left coronary artery; RCA, Right coronary artery

Clicks, opening snap and pericardial friction rub are the other added sounds one should try to detect during auscultation.



Heart Murmurs Heart murmurs are audible sound waves in the range of 20 Hz–2,000 Hz that occur as a result of turbulent blood flow due to functional or structural hemodynamic alterations in the pattern of blood flow in the heart chambers across the valves or the atrial or ventricular septum or in the vessels as in coarctation of aorta, renal artery stenosis or pulmonary arteriovenous collaterals or malformations. When the presence of murmur over the precordium is appreciated by the student, it becomes essential to make a clinical decision whether the murmur is functional/ innocent or organic/pathological due to an underlying cardiac disorder. When one or more of the following are present, the murmur is likely to be pathologic and requires further evaluation: symptoms, cyanosis,

systolic murmur that is loud (grade 3/6, harsh and long in duration), diastolic murmur, abnormal heart sounds, presence of a click, and abnormally strong or weak pulses. The characteristics of organic heart murmurs have been summarized in Table 7.1.8.

Innocent Murmurs A murmur heard in a child without structural heart disease is termed innocent murmurs. The child has no cardiac related symptoms or other signs on careful history and physical examination, and basic investigations like chest X-ray, electrocardiogram (ECG) and echocardiogram are normal. A significantly large proportion of children have innocent murmurs beginning at 3–4 years of age. The characteristics of innocent or functional murmurs are usually the following: they are localized to specific areas, short, often early or mid-systolic, grade 1/6 or 2/6 (not associated with thrill) and vary in intensity with change in posture; decreasing in intensity when the child stands, sits up or strains during a Valsalva maneuver.

Other Abnormal Sounds

425

vip.persianss.ir





















B

cs

i atr

i ed

1. Allen HD, Philips JR, Chan DP. History and physical examination. In: Moss AJ, Allen HD (Eds). Moss and Adams’ Heart Disease in Infants, Children and Adolescents, 7th edition. Philadelphia: Lippincott; 2008.pp.66-80. 2. Fyler DC, Nadas AS. History, physical examination, and laboratory test. In: Keane J, Fyler DC, Lock J (Eds). Nadas’ Pediatric Cardiology. Philadelphia: Saunders; 2006.pp.10116. 3. Geggel RL, Fyler DC (Eds). History, growth, nutrition, physical examination, and routine laboratory tests. Nadas’ Pediatric Cardiology, 2nd edition. Philadelphia: WB Saunders; 2008.

4. Park MK. Basic tools in routine evaluation of cardiac patients. In: Park MK (Ed). Pediatric Cardiology for Practitioners, 5th edition. Philadelphia: Mosby Elsevier; 2008.pp.3-39. 5. Roy DL. Cardiovascular assessment of infants and children. In. Goldbloom RB (Ed). Pediatric Clinical Skills, 3rd edition. Philadelphia: Saunders; 2003.pp.169-89. 6. Thompson WR, Mehrotra SM. Approach to heart diseases in children: cardiac history and physical examination. In: Abdulla Ra-id (Ed). Heart Diseases in Children: A Pediatrician’s Guide. New York: Springer; 2011.pp.3-16. 7. Tandon R. Bedside diagnosis of congenital heart disease. In: Bahl VK (Ed). Cardiology Update 2008. Cardiological Society of India. 2008.pp.484-90.

e

t

f

T x book o P

ibliography

426

vip.persianss.ir





Diseases of Car iovas ular ystem

7.2

Congenital Heart Disease R Krishna Kumar



­



E

d

pidemiology

Congenital heart disease accounts for about 10% of infant deaths and nearly half of all deaths due to congenital malformations in developed countries. The prevalence of CHD diagnosed in the first 12 months is estimated at 6–8 per 1,000 live births. Of these, at least 25% are lifethreatening CHDs that require very early intervention. In India, for a population of 1.2 billion, it can be expected that there would perhaps be approximately 100,000 newborns born each year with CHD requiring some form of intervention early in life. It is estimated that only 3–5% of newborns and infants with CHD are receiving timely surgery in India. The vast majority of newborns with CHD escape timely detection. Congenital heart disease has a unique profile in India characterized by remarkable early attrition, late survival selected by natural history and significant numbers of older children with complications, such as pulmonary hypertension, overt (brain abscess and stroke) and covert (neurodevelopmental abnormalities) neurological damage. E



c





This chapter will expand on the above mentioned points. A brief description of common CHDs has been included in a section at the end of the chapter.



1. The most important role of the pediatrician today is to ensure that, as far as possible, serious CHD should not be missed. This is especially true for the neonatal period and during infancy where maximal attrition from CHD occurs. 2. History, physical examination, chest X-ray and ECG are the traditional tools available to the pediatrician. With this it is usually possible to identify whether or not CHD is present except perhaps in the early newborn period where CHD diagnosis can be challenging. 3. Despite numerous anatomic possibilities, the physiologic categories of CHD are limited and can be understood. Understanding the basic hemodynamic and physiologic concepts are important because they have an important bearing on clinical picture, interpretation of echocar diographic reports, natural history and decision making on timing of surgical or catheter intervention. 4. The traditional diagnostic tools allow classification of physiologic categories in a significant proportion of babies. The limitations of the traditional tools in identifying the precise anatomy are substantial and this is exposed by the widespread use of echocardiography. 5. Numerous recent advances have dramatically changed the outlook of children with CHD. As a result some form of palliation or correction is feasible for most CHD provided this is undertaken in a timely fashion. 6. The improved understanding of CHD today suggests that management needs to be individualized or tailored to each patient. Today, it is possible to identify and determine the severity of all the specific lesions through echocardiography. Recognizing the numerous combinations and specific features with each lesion, each patient needs to be approached according to the specifics of the lesion(s). 7. It is increasingly evident that a substantial proportion of children with CHD have significant problems in other

organ systems. The pediatrician has an important role in identifying them because they have significant bearing on eventual outcomes and counseling of the family. 8. In spite of significant recent advances, long-term concerns after palliation and many forms of “correction” of CHD are significant and many of these children need life-long follow-up.

S



Congenital heart disease encompasses a broad and extremely diverse range of conditions that can manifest anytime from prenatal to late adulthood. In common parlance, CHD refers to structural heart defects that are present at birth. Conditions such as HCM and mitral valve prolapse may actually have a congenital basis but these are often discussed separately. In this chapter we will be discussing common structural defects that are present at birth. A practical approach lies in remembering the following:

tiopathogenesis

The link between etiology and embryogenesis of CHD is often very complex. A lot of recent research has focused on the chain of events in early embryogenesis of the heart and great vessels. As a result there have been dramatic conceptual upheavals. The details are beyond the scope of this chapter. In simplified terms, the pediatrician should recognize today that a growing proportion of patients with CHD have an identifiable genetic basis. Table 7.2.1 summarizes common genetic conditions that are associated with CHD. The association with acquired conditions and teratogens and occurrence of CHD has been summarized in Table 7.2.2.

427

vip.persianss.ir

For many years, CHD has been broadly classified as cyanotic and acyanotic with certain specific subtypes in each category (Table 7.2.3). While broad classifications based on physiology works for many situations there are a number of patients who cannot be classified into common physiologic

categories. Additionally, there are often specific issues such as valve regurgitation that dictate the clinical presentation. The broad categories of CHD in Table 7.2.3 have limitations, and some patients may have a combination of lesions belonging to more than one category. For example, a child may have a large VSD with moderate pulmonary stenosis (PS) and mild pulmonary hypertension.

 

Table 7.2.1 Common genetic conditions that are linked to the occurrence of CHD Specific condition

Genetic basis

CATCH 22 syndrome

Microdeletion on long arm of chromosome 22, 22q11 Interrupted aortic arch, TOF, VSD, persistent truncus arteriosus, deletion; autosomal dominant inheritance double outlet right ventricle

Williams-Beuren syndrome

Microdeletion at chromosome 7q11.23 (Elastin gene Supravalvar AS, pulmonary arterial stenosis, aortic and mitral located in this segment); autosomal dominant inheritance valve abnormalities, adult systemic hypertension

Down syndrome

Chromosomal trisomy for chromosome 21 (rarely AV canal defect, perimembranous VSD, TOF, PDA Robertsonian translocation or mosaicism), sporadic mutation

Turner’s syndrome

Chromosomal (45, X karyotype and variants mosaicism, Predominantly left-sided lesions (BAV, coarctation); risk for structurally abnormal X chromosome) aortic root dilatation

Noonan’s syndrome

PTPN11 mutations in 50%; half of cases are de novo; PS (20–50%), HCM (20–30%), ASD autosomal dominant inheritance in the next generation

VATER association

No specific recurring etiology, sporadic; very small risk to VSD, TOF offspring

Holt-Oram syndrome

TBX5 mutation (70%); 15% familial, autosomal dominant Ostium secundum ASD, VSD, conduction defects inheritance

CHARGE

CHD7 mutation (60%) or deletion; most cases are de novo; Commonly right-sided lesions, branch pulmonary artery autosomal dominant inheritance stenosis and conotruncal lesions (TOF, VSD)

Alagille syndrome

Pulmonary valve, artery and branch pulmonary artery most JAG1 mutation (70%) or deletion (w5%); 50–70% of cases are commonly affected; TOF in 7–15% de novo; autosomal dominant inheritance

T

d

c

extbook of Pe iatri s

Classification

Cardiac lesions

Abbreviations: CHD, Congenital heart disease; VSD, Ventricular septal defect; AS, Aortic stenosis; AV, Atrioventricular; TOF, Tetralogy of Fallot; PDA, Patent ductus arteriosus; BAV, Bicuspid aortic valve; PS, Pulmonary stenosis; HCM, Hypertrophic cardiomyopathy; ASD, Atrial septal defect

 

Table 7.2.2 Etiology of CHD: prenatal exposure to acquired factors Acquired condition

Association with CHD

Diabetes

A variety of forms of CHD are now linked with maternal gestational and pregestational diabetes. The list includes transposition, AV septal defects, VSD, hypoplastic left heart syndrome, cardiomyopathy and PDA

Febrile illnesses in the first trimester

Any febrile illness during the first trimester of pregnancy may have a two fold increase in the risk of CHD

Maternal rubella

Specific cardiac manifestations of rubella embryopathy include: PDA, pulmonary valve abnormalities, peripheral PS and VSD

Epilepsy

The association is perhaps as a result of the risk of CHD from anticonvulsant medications

Lupus (apart from typical symptoms Maternal SLE is associated with risk of complete heart block in the offspring of SLE it may be useful to ask for history of previous abortions) Phenylketonuria

Phenylketonuria is associated with greater than six fold increase in the risk of CHD (specifically VSD, TOF, PDA and single ventricle)

Vitamin deficiency

Multivitamin supplements including folic acid derivatives have been shown to protect against occurrence of CHD in two studies. Multivitamins may also reduce the risk of CHD associated with febrile illnesses in the first trimester

Prenatal exposure to medications in The list of medications known to be associated with a risk of CHD in the offspring includes: anticonvulsants, the first trimester NSAIDs, trimethoprim-sulphonamide, thalidomide and vitamin A congeners Occupational exposure

428

Exposure to organic solvents, herbicides, pesticides and ionizing radiation may increase the risk of CHD in the offspring

Abbreviations: CHD, Congenital heart disease; AV, Atrioventricular; VSD, Ventricular septal defect; PDA, Patent ductus arteriosus; PS, Pulmonary stenosis; SLE, Systemic lupus erythematous; TOF, Tetralogy of Fallot; NSAIDs, Nonsteroidal anti-inflammatory drugs

vip.persianss.ir

Examples

Acyanotic congenital heart disease Simple left to right shunts Pre-tricuspid

Partial anomalous pulmonary venous drainage, ASDs

Post-tricuspid Ventricular

VSD

Great artery

Aortopulmonary window, patent arterial duct, coronary cameral fistula, ruptured sinus of Valsalva

Combination of pre- and post-tricuspid

AV septal defect, left ventricle to right atrial communications

Obstructive lesions Cor triatriatum, obstructive lesions of the mitral valve apparatus

c

Inflow: left-sided

Infundibular stenosis, pulmonary valve stenosis, branch pulmonary artery stenosis

Left ventricle

Subaortic membrane, valvular aortic stenosis, supravalvar aortic stenosis, coarctation of aorta

Miscellaneous lesions

Coronary artery abnormalities, congenital mitral and tricuspid valve regurgitation

S

Outflow Right ventricle

Cyanotic heart disease Reduced pulmonary blood flow Intact interventricular septum

d

 

Physiologic category

Diseases of Car iovas ular ystem

Table 7.2.3 Broad physiologic categories of CHD

PA with intact ventricular septum, critical PS with right to left shunt at atrial level, Ebstein’s anomaly, isolated RV hypoplasia

Unrestrictive ventricular communication All conditions listed under VSD with PS or PA physiology;* Miscellaneous conditions

Pulmonary arteriovenous malformation, anomalous drainage of systemic veins to left atrium

Increased pulmonary blood flow Pre-tricuspid

Total anomalous pulmonary venous communication Common atrium

Post-tricuspid

All single ventricle physiology lesions without PS, persistent truncus arteriosus

Pulmonary hypertension

Shunt reversal after development of pulmonary vascular obstructive disease in any of the conditions associated with increased pulmonary blood flow (Eisenmenger physiology)

*VSD with PS or PA physiology: This includes all conditions with an unrestrictive interventricular communication and PS or PA. Included in this list are the following: Tetralogy of Fallot, double-outlet right ventricle, single ventricle, transposition with VSD, corrected transposition with VSD, AV septal defect and tricuspid atresia. Abbreviations: CHD, Congenital heart disease; ASDs, Atrial septal defects; VSD, Ventricular septal defects; AV, Atrioventricular; PA, Pulmonary atresia; PS, Pulmonary stenosis; RV, Right ventricular



Another way of understanding the physiology of CHD is to list various physiological perturbations (Table 7.2.4) that are commonly associated with CHD. Here again, it is evident that many defects have more than one physiological perturbation. The table is, however, useful in that it illustrates various mechanisms by which CHD produce clinical manifestations.

Physiology Here, the focus will be on illustrating selected key physiological concepts that are important to understand a large number of lesions. These include the following: S

T

V

T

Pre- ricuspid ersus Post- ricuspid hunts Acyanotic heart disease with left to right shunts is traditionally classified as pre-tricuspid and post-tricuspid shunts.

Pre-Tricuspid Shunts Left to right shunts at the level of the atria are known as pretricuspid shunts. They include ASDs and partial anomalous pulmonary venous connection. The left to right shunt and the consequent excessive pulmonary blood flow is dictated by relative stiffness of the two ventricles. Since the right ventricle is relatively stiff (noncompliant) at birth and during early infancy, the shunt is small. Over the years, the right ventricle progressively enlarges to accommodate the excessive pulmonary blood flow. The pulmonary vasculature also becomes more capacious to gradually accommodate the excessive blood flow. This explains why ASD typically do not manifest with symptoms of pulmonary over circulation during infancy and childhood. The clinical signs are also easily explained by the physiology of pre- 429 tricuspid shunts. The diastolic flow murmur of ASD is across

vip.persianss.ir

 

Physiological derangement

Hemodynamic consequences

Clinical consequences

Examples

Increased pulmonary blood flow

Inefficient circulation, pulmonary congestion, pulmonary hypertension

Failure to thrive, heart failure symptoms, tachypnea, frequent respiratory infections

All post-tricuspid shunts, some large pre-tricuspid shunts

Reduced pulmonary blood flow

Hypoxia in the presence of an ASD or VSD; Cyanosis, fatigue reduced cardiac output if both septa are intact

Mixing of systemic and pulmonary venous return (single ventricle physiology)

Some hypoxia will result but the exact oxygen saturation will be dictated by pulmonary blood flow. If pulmonary blood flow is increased (shunt ratio of 2:1 or more) saturations are usually above 85%.

Cyanosis of varying severity depending on pulmonary blood flow

All forms of univentricular hearts; double inlet left ventricle or right ventricle, tricuspid atresia, primitive hearts associated with asplenia and polysplenia

Unfavorable streaming and parallel circulation

Hypoxia tends to be severe unless pulmonary blood flow is significantly elevated

Severe cyanosis in the face of excessive pulmonary blood flow

All variations of TGA; double outlet right ventricle with subpulmonic VSD (Taussig-Bing)

Pulmonary venous hypertension

Pulmonary venous congestion with pulmonary arteriolar vasoconstriction and resultant elevation in pulmonary vascular resistance

Tachypnea from pulmonary edema, severe elevation in pulmonary vascular resistance translates into reduced pulmonary blood flow and increased cyanosis

Obstructed total anomalous pulmonary venous connection, cor triatriatum, congenital MS, mitral atresia with restrictive ASD, pulmonary venous stenosis

Elevated pulmonary vascular resistance

Inability to increase cardiac output during exercise or stress, increased right to left shunting across associated defects

Fatigue on effort, patients with defects in the atrial or ventricular septum will have increasing cyanosis

All conditions associated with increased pulmonary blood flow increase the risk for development of increased pulmonary vascular resistance

Left-sided AV valve regurgitation

LV volume overload, elevation in LA pressures, pulmonary venous congestion

Tachypnea, fatigue on effort

AV septal defects, corrected transposition, ASDs (5–10% have associated mitral valve prolapse and MR), congenital MR, anomalous coronary artery from pulmonary artery

Right-sided AV valve regurgitation

RV volume overload, elevated RA pressures, systemic venous congestion

Fatigue, right heart failure

Ebstein’s anomaly, congenital anomalies of tricuspid valve

LV hypertrophy

Elevated filling pressures resulting in left heart failure

Tachypnea from pulmonary venous congestion

All forms of LV outflow tract obstruction, hypertrophic cardiomyopathy

RV hypertrophy

Elevated right-sided filling pressures with systemic venous congestion

Right heart failure, liver enlargement, fluid accumulation (edema and ascites)

Advanced forms of RV outflow obstruction: critical PS, advanced pulmonary hypertension

Reduced coronary perfusion

LV dysfunction

Tachypnea, fatigue

Anomalous left coronary artery from pulmonary artery

Pulmonary valve regurgitation

Dilatation of branch pulmonary arteries with airway compression, RV dilatation

Respiratory symptoms in infants with TOF and absent pulmonary valve, fatigue on effort in patients with long-standing pulmonary valve incompetence

TOF with absent pulmonary valve

Aortic valve regurgitation

LV dilatation

Fatigue and palpitation

Bicommissural aortic valve in association with subpulmonic and some perimembranous defects

T

d

c

extbook of Pe iatri s

Table 7.2.4 Physiological perturbations in CHD

All congenital heart defects that are associated with PS

Abbreviations: CHD, Congenital heart disease; ASDs, Atrial septal defects; VSD, Ventricular septal defect; PS, Pulmonary stenosis; TGA, Transposition of great arteries; MS, Mitral stenosis; AV, Atrioventricular; RA, Right atrial; LV, Left ventricular; LA, Left atrial; MR, Mitral re gurgitation; RV, Right ventricular; TOF, Tetralogy of Fallot

430

vip.persianss.ir

Post-Tricuspid Shunts

This refers to a group of conditions where there is complete mixing of pulmonary and systemic venous returns. In addition to single ventricle (double inlet ventricle), a variety of conditions come under the category of single ventricular physiology. Atresia of one of the AV valves, severe hypoplasia of one of the ventricles, severe straddling of one of the AV valves over a large VSD are all examples of situations where there is mixing of pulmonary and systemic venous returns. The clinical manifestations are dictated by whether or not there is PS. In absence of PS, there is excessive pulmonary blood flow especially in infants because of the relatively lower PVR. As a result, the proportion of oxygenated blood from pulmonary veins that mixes with the systemic venous return is high. This results in minimal cyanosis and measured oxygen saturation may be in the 90s. However, the price for this preserved oxygenation is HF and development of permanent elevation of PVR [pulmonary vascular obstructive disease (PVOD)]. Over time, if the child survives infancy, PVR progressively increases with increasing cyanosis. Single ventricle and its physiologic variants can be associated with varying degrees of PS. Here the situation is quite similar to VSD-PS physiology except for relatively severe hypoxia because of free mixing of systemic and pulmonary venous return.

S

c

d

V

L

Duct-Dependent esions An infant or a newborn with CHD that is dependent on the patency of the ductus arteriosus for survival can be termed as suffering form a duct-dependent lesion. Typically, these are newborns in which either the systemic blood supply is critically dependent on an open PDA [duct-dependent systemic circulation (DDSC)] or pulmonary blood flow is duct dependent [duct-dependent pulmonary circulation (DDPC)]. Closure of the PDA in DDSC results in systemic hypoperfusion (often mislabeled as neonatal sepsis). Examples of DDSC include hypoplastic left heart syndrome where the entire systemic circulation is supported by the right ventricle through PDA, interrupted aortic arch where the descending aortic flow is entirely through PDA. Severe coarctation and critical AS are also examples of DDSC. Closure of PDA in DDPC results in severe hypoxia and cyanosis in the affected newborn. Examples of DDPC include all forms of pulmonary atresia (irrespective of

This situation is characterized by a large communication at the ventricular level together with varying degrees of obstruction to pulmonary blood flow. Typically, this is in the form of subvalvar (infundibular), valvar, annular (small annulus) and occasionally supravalvar stenosis. The free communication between the two ventricles results in equalization of pressures. Severity of PS dictates the volume of blood flowing through pulmonary arteries and therefore amount of oxygenated blood returning via pulmonary veins. Severe PS results in right to left shunt across the VSD with varying degrees of hypoxia and, consequently, cyanosis. Cyanosis is directly proportionate to the severity of PS. Because the right ventricle is readily decompressed by the large VSD, heart failure (HF) is unusual. The best example of VSD-PS physiology is tetralogy of Fallot (TOF). In its least severe form, TOF is often not associated with cyanosis (pink TOF). Here PS is significant enough to result in a large pressure gradient across the right ventricular outflow tract (RVOT), but not severe enough to result in a reduction in pulmonary blood flow. Pink TOF is typically associated with a loud ejection systolic murmur because of a reasonable volume of blood flowing across the RVOT. As



D-P Physiology S

VS

Post-tricuspid shunts are different in that there is direct transmission of pressure from systemic to pulmonary circuit at the ventricular level (VSD) or great arteries (PDA and aortopulmonary window). For patients with large post-tricuspid shunts, symptoms begin in early infancy, typically after some regression of elevated pulmonary vasoconstriction in the newborn period together with progressive development of the pulmonary vascular tree. The excessive pulmonary blood flow returns to the left atrium and flows through the mitral valve resulting in an apical diastolic flow murmur that is a consistent marker of large post-tricuspid shunts. The left atrium and ventricle are dilated as a result of this extra volume. Elevation in pulmonary artery pressures is an inevitable consequence of large (or unrestrictive) post-tricuspid shunts and is labeled as hyperkinetic PAH. This needs to be distinguished from elevation of pulmonary vascular resistance (PVR) that results from sustained exposure to increased pulmonary blood flow.

ingle entricle Physiology



S

the severity of PS increases, pulmonary blood flow declines and the intensity of murmur declines progressively. Other examples of VSD-PS physiology include double outlet right ventricle (DORV) with subaortic VSD and PS, corrected transposition with PS and atrioventricular (AV) septal defect with PS. These conditions are often indistinguishable from TOF at the bedside.

Diseases of Car iovas ular ystem

the much larger tricuspid valve and is therefore subtle or even inaudible. The S2 split is wide and fixed because of the prolonged “hang-out” interval resulting from increased capacitance of the pulmonary circulation. Pulmonary arterial hypertension is typically absent or at most mild. The presence of moderate or severe PAH in ASD is worrisome and may suggest the onset of irreversible changes in the pulmonary vasculature.

431

vip.persianss.ir

Cyanosis

432

Cyanosis may be peripheral or central. Peripheral cyanosis almost exclusively involves lips and extremities. Normal neonates may have bluish extremities that respond to warming or moving the extremities. Saturations of 90% or lower while breathing room air beyond the first 20 minutes are considered abnormal. Similarly, some infants may have peripheral cyanosis following exposure to cold. Central cyanosis involving the mucous membranes and trunk along with the lips and extremities strongly suggests the likelihood of CHD. Unfortunately, however, cyanosis often remains unnoticed. This is particularly true in the Indian context, where it is difficult to detect cyanosis due to

F B

Tachypnea (respiratory rates consistently more than 60/min in infants less than 2 months, greater than 50/min in older infants, greater than 40/min after 1 year) is a characteristic manifestation of HF in newborns (preterm newborns may normally have respiratory rates of up to 60/min). For infants, subcostal or intercostal retractions together with flaring of nostrils (alae nasi) are frequently associated with tachypnea. I

requent espiratory nfections

Respiratory infections that are frequent, severe and difficult to treat are common in infants with CHD associated with large left to right shunts resulting in increased lung blood flow. Not infrequently, heart disease may be detected for the first time during an episode of respiratory tract infection. S

Clinical tigmata of pecific yndromes S

The manifestations of CHD are different in a neonate, an infant or a child. It is often easy to recognize the presence of CHD in older children. In infants and particularly in newborns, manifestations of heart disease can often be subtle. Further, a number of conditions that do not involve the cardiovascular system can result in clinical manifestations that overlap with those resulting from heart disease. Notwithstanding these difficulties, it is possible to identify the presence of heart disease in most infants and newborns after careful clinical evaluation. The following clinical features should alert the pediatrician regarding the presence of CHD.

Difficult reathing

Evidence of presence of chromosomal anomalies or other syndromes that are known to be associated with CHD should alert the clinician to the presence of specific cardiac defects that are known to be associated with these conditions. The list of such conditions is a long one. Trisomy 21 is the most common chromosomal anomaly that is associated with heart disease. Other common examples include: trisomy 13, trisomy 18, Turner’s syndrome, Noonan’s syndrome, velocardiofacial and the Di-Georges’s syndrome (Table 7.2.1).

Cardiovascular xamination E

ecognition of Congenital Heart Disease

The parent of an infant with CHD may complain that the child has difficulty with feeds. This is usually a feature of an infant with congestive heart failure (CHF) resulting from CHD. The history may be of slow feeding, small volumes consumed during each feed, tiring easily following feeds and requirement of periods of rest during feeds. Not infrequently, no history of feeding difficulty may be obtained, but examination of the growth charts will reveal that the child’s growth rate is not appropriate for age. A recent decline in growth rate (falling off the growth curve) or weight that is inappropriate for age (< 5th percentile) may result from a large left to right shunt. Characteristically, growth retardation affects weight more that height.

R

R

Unfavorable streaming refers to a situation where oxygen rich pulmonary blood flow is directed toward the pulmonary valve and poorly oxygenated blood toward the aortic valve. An extreme form of unfavorable streaming is the parallel circulation in transposition of great arteries (TGA) with intact ventricular septum. Here survival is dependent of the presence of a communication (ideally at atrial level) that allows mixing of pulmonary and systemic venous return. The presence of a VSD may improve the situation in TGA but significant cyanosis is usually present unless the pulmonary blood flow is torrential.

Difficult eeding and Poor Growth

S

S

nfavorable treaming and Parallel Circulation

presence of a dark skin complexion. Further, cyanosis is often masked by anemia. When central cyanosis is suspected, its presence should be confirmed and severity quantified by measuring oxygen saturation using a skin oxymeter probe. Unfortunately, however, the availability of this instrument is limited to a few selected institutions.

F

U

T

d

c

extbook of Pe iatri s

underlying heart defect) where the PDA is the predominant source of pulmonary blood flow. There are examples of pulmonary atresia where pulmonary blood supply is from major aortopulmonary collaterals. These children may survive even after the PDA closes. Critical PS (“nearlyatretic” pulmonary valve) can present as duct-dependent pulmonary blood flow. Neonates with duct-dependent physiology require prostaglandin E1 (PGE1) for survival. Early recognition of a duct-dependent situation allows early initiation of PGE1 and stabilization until definitive procedure can be accomplished.

A thorough and systematic cardiovascular examination provides valuable clues to the presence of heart disease. With practice, such an examination can be accomplished in a short time. For the pediatrician, a thorough familiarization

vip.persianss.ir

Does the precordium feel normal?

A





Are there visible precordial pulsations? Is the apex beat displaced, hyperdynamic or heaving? Is there a thrill palpable?

E

d c

The Checklist for Diagnosis of Congenital Heart Disease through Echocardiography 1. What is the visceral and atrial situs (solitus, inversus or ambiguous)? 2. What are the systemic venous connections like? Is the inferior vena cava intact? Is there a separate left superior vena cava? If so, how does it drain? 3. What is the relationship of the abdominal aorta to the inferior vena cava? 4. What is the appearance of the atria like? Is the atrial septum intact? Is there an ASD or a patent foramen ovale? If so, in what direction does blood flow? 5. How do the atria connect to the ventricles (concordant or discordant)? 6. Is it possible to identify two separate AV valves? 7. How are the AV valves in their appearance and function? 8. The ventricles: their size, shape and function? 9. Are various components of the interventricular septum intact? 10. Is there any outflow tract obstruction? 11. How do the ventricles connect to the great arteries? 12. What is the appearance and function of the semilunar valves? 13. What is the great artery relationship? 14. Are the branch pulmonary arteries in continuity? Is there any branch pulmonary artery stenosis? 15. What side is the aortic arch? What is its branching pattern? Is there a PDA or a coarctation? 16. What is the coronary artery anatomy? 17. How is the hemodynamics and physiology altered?

re the heart sounds normal?

S





Is the pulse volume normal or increased? Is there a discrepancy of pulsation in any of the four extremities? A careful evaluation of pulsations in all extremities should always be a part of physical examination. Coarctation is readily diagnosed when weak femoral pulsations are detected in comparison to brachials or radials. When a discrepancy in pulses is suspected, four extremity blood pressure measurements should be obtained. An automated noninvasive blood pressure instrument is preferred over manual recording for four extremity blood pressure measurement.

Echocardiography has revolutionized diagnosis of CHD and its high diagnostic yield makes this investigation very cost effective in most situations. This statement is, however, only true if it is performed by an experienced individual who understands anatomy and physiology of CHD. In many children with CHD, a thorough evaluation is possible and all the questions addressed in the check list (below) can be answered. This is particularly true for infants and newborns in whom excellent images are readily obtained. Such patients can undergo a definitive procedure based on the information provided by the echocardiogram. Transesophageal echocardiography can supplement transthoracic studies. This is particularly true for patients with poor echocardiography windows that do not permit adequate imaging. Unlike adults, children undergoing transesophageal echocardiography often require general anesthesia. Echocardiography has a few inherent limitations, however. It consistently fails to define certain structures, such as the pulmonary artery branches beyond the hila of the lungs. Here cardiac computed tomography (CT) or cardiac magnetic resonance imaging (CMR) work well. Newer modalities such as three-dimensional echocardiography are in the evolving phase and, at present, do not add significant value to twodimensional echocardiography.

lectrocardiogram and Chest -ray





If there is a suspicion of heart disease on the basis of history or physical examination, an ECG and a chest X-ray should be obtained. The ECG should be interpreted keeping the age of the child in mind. Interpretation of the chest X-ray should involve evaluation of the cardiac silhouette and lung vasculature. The X-ray also provides information about the location of abdominal organs (for determination of the situs) and the aortic arch. Beyond the neonatal period, a normal ECG and chest X-ray makes the diagnosis of a hemodynamically significant heart defect unlikely. In the newborn, however, ECG and chest X-ray changes may take a few days to evolve. For the newborn, particularly in the first few days, a normal ECG and a normal X-ray does not rule out serious heart disease.





X

E



Is it systolic or diastolic or both systolic and diastolic (diastolic murmurs always have a structural basis)? Is it loud (grade 3 or louder murmurs are seldom innocent)?



s (or are) there a murmur (or murmurs)? f so: I

I







Can the two components of the S2 be separated? Is there an additional heart sound in diastole (such as S3)? Are there any additional systolic sounds (ejection clicks)?



A

re the arterial pulsations normal?

chocardiography

Diseases of Car iovas ular ystem

with what is normal is a useful initial step. It is useful to answer the following questions that can serve as a checklist for a preliminary cardiac examination. This checklist is not at all comprehensive and is designed primarily for answering the question: Does the patient have heart disease? It can also help identify the broad physiologic category of heart defect.

433

vip.persianss.ir

urgery

Flow chart 7.2.2 Classification of common congenital heart operations

Abbreviations: ASD, Atrial septal defect, VSD, Ventricular septal defect; BT shunt, Blalock-Taussig shunt; ICR, Intracardiac repair; PA band, Pulmonary arterial band; PDA, Patent ductus arteriosus; TAP, Transannular patch; TOF, Tetralogy of Fallot; AV canal, Atrioventricular canal; TAPVC, Total anomalous pulmonary venous connection  

Surgery is still the best option for definitive treatment or palliation of most forms of CHD. A simple scheme of classification of surgery for CHD is shown in Flow chart 7.2.2. This classification is a practical one and is from the point of view of follow-up issues. A detailed description of all congenital heart operations is clearly beyond the scope of the chapter. Congenital heart operations are also broadly classified as open heart [requiring use of cardiopulmonary bypass (CPB)] and closed heart (not requiring CPB). Most corrective operations and many palliative operations fall under the category of open heart operations. These procedures are generally more significant and expensive undertaking because of the use of the CPB circuit and substantially larger number of disposable items. The morbidity of open heart operations is proportionate to the duration of exposure to CPB and the cross-clamp time (the period of time when heart beating is deliberately brought to a standstill through the use of cardioplegia).

has transformed the care of many children with CHD. Many simple defects such as secundum ASD, PDA, selected muscular VSD can now be closed in the catheterization laboratory. Additionally, balloon valvotomy is now the first line of treatment for congenital stenosis of the pulmonary and aortic valves. Additional details of catheter intervention procedures have been shown in Table 7.2.5. The advantages of catheter-based interventions are obvious and this has become an increasingly appealing option for many children. The procedures are far less traumatic than surgery,  

T

To ensure the best possible results of management of CHD, it is necessary to assemble a team of specially qualified individuals who should ideally be a part of a comprehensive pediatric heart program. The details are shown in Flow chart 7.2.1. Definitive treatment for CHD requires the collaborative (and not competitive) use of surgery and catheter-based interventions. S

d

c

extbook of Pe iatri s T

Definitive reatment and Palliation

I

Catheter nterventions for Congenital Heart Disease The scope of catheter interventions has grown by leaps and bounds in the last 2–3 decades and this treatment option

 

Flow chart 7.2.1 The constituents of a comprehensive pediatric heart program for optimal care of children with congenital heart disease (CHD). For the best and consistently reproducible results in newborn and infant heart surgery, the presence of such a team is vital

434

vip.persianss.ir

Remarks Only defects in the fossa ovalis region with sufficient margins are suited for device closure

PDA

Coil or device closure

The vast majority of PDAs can be closed in the catheterization laboratory with the exception of large PDA in small infants

Muscular VSD

Device closure

Device closure is an option for relatively older infants and children (> 8 kg)

Membranous VSD

Device closure

This is controversial because of the small but definite possibility of heart block

Pulmonary valve stenosis

BPV

Treatment of choice for most forms at all ages; dysplastic valves in Noonan’s syndrome may not respond well to BPV

Aortic valve stenosis

Balloon aortic valvotomy Initial treatment of choice at all ages; eventually the dilated aortic valves may need surgical management

Branch pulmonary artery stenosis

Balloon dilatation with stenting

Stenting is often preferred to surgery in branch pulmonary artery stenosis

Coarctation of aorta

Balloon dilatation with or without stenting

Surgery is the preferred option in the newborn period because recurrence after balloon is very common. For older infants, balloon is a reasonable alternative. Older children and adolescents (> 10 years) may be offered stenting for a more complete relief. Neither balloon or stenting has a proven advantage over surgery.

Coronary artery fistula

Coil or device closure

Treatment of choice

Pulmonary arteriovenous malformations

Coil or device closure

Treatment of choice when discrete. For diffuse malformations, coil or device closure is not an option

Duct-dependent pulmonary circulation

Stenting of the PDA

Stenting can be offered in carefully selected cases as an alternative to the Blalock-Taussig shunt

Pulmonary atresia with intact ventricular septum

Perforation of the valve followed by balloon dilatation

Emerging as the procedure of choice in selected institutions with the facility and expertise

Ruptured sinus of Valsalva aneurysm

Device closure

A reasonable option in carefully selected cases

TGA

Balloon atrial septostomy

Short-term palliation before definitive surgery (arterial switch operation)

S

c

Procedure Device closure

d

 

Lesion ASD

Diseases of Car iovas ular ystem

Table 7.2.5 Congenital heart defects amenable to catheter-based interventions

Abbreviations: ASD, Atrial septal defect; PDA, Patent ductus arteriosus; VSD, Ventricular septal defect; BPV, Balloon pulmonary valvotomy; TGA, Transposition of great arteries

nfective ndocarditis or ndarteritis

A



Pulmonary arterial hypertension occurs commonly in the context of CHD. With increasing emphasis on early correction, the magnitude of the problem is on the decline in most developed nations. Unfortunately, because most children with CHD do not receive timely attention, this is still a problem of considerable magnitude in the developing world. The lesions that have the greatest likelihood include cyanotic heart disease with increased pulmonary blood flow. Here irreversible changes in pulmonary vasculature develops rapidly often during infancy itself. Therefore, it is particularly important to correct or appropriately palliate these lesions early (ideally within the first few months of

E

Pulmonary rterial Hypertension

Complications

E

A number of adverse complications occur as a consequence of CHD. For the sake of simplicity, we will focus on selected common complications.

life). Large acyanotic post-tricuspid shunts are also prone to early development of PAH and should be ideally corrected early, preferably within the first year. In pre-tricuspid shunts, PAH develops slowly and unpredictably. Most patients with ASD will have mild or no PAH throughout their lives. A small proportion develops accelerated changes in the pulmonary vasculature. Some of the key features responsible for the development of PAH have been summarized in Figure 7.2.1. I

accomplished with greater ease, allow rapid recovery and leave behind no scars.

Endocarditis can complicate CHD with a suitable substrate. This includes the presence of a region of significant turbulence created by high-pressure gradients. Examples include restrictive VSD and PDA, TOF (pulmonary valve) and other VSD-PS situations, and LV outflow tract obstruction. Some surgical operations (such as the BT shunt) are also associated with increased risk of infective endocarditis (IE) or endarteritis. Lesions with little or no turbulent flows, such as ASD are not associated with increased risk. Typically, the risk of endocarditis increases after dentition begins. Current guidelines do not routinely recommend antibiotic 435 prophylaxis for many conditions (including some of the

vip.persianss.ir

d

c

extbook of Pe iatri s T

 

Figure 7.2.1 Natural history of uncorrected CHD. This is a simplified representation showing the survival for most common congenital heart defects Abbreviations: ASD, Atrial septal defect; APW, Aortopulmonary window; AV canal, Atrioventricular canal; HLHS, Hypoplastic left heart syndrome, LR, Left to right; PDA, Patent ductus arteriosus; TAPVC, Total anomalous pulmonary venous connection; TGA, Transposition of great arteries; TOF, Tetralogy of Fallot

yocardial Dysfunction and

M

M

yocardial Damage

Chronic volume overload results in ventricular enlargement and ventricular dysfunction that is typically revered after correction. A small proportion of patients with sever hypoxia also develop severe ventricular dysfunction involving both ventricles. Heart failure is mostly the result of hemodynamic consequences of increased pulmonary blood flow, mitral or tricuspid valve regurgitation and severe myocardial hypertrophy. Systolic dysfunction is a relatively less common 436 cause.

Polycythemia and its dverse Consequences A

This is affected in all forms of CHD and is particularly striking in large left to right shunts. Children with CHD show a high prevalence (59% in recent Indian studies) of significant malnutrition (weight is more affected than height) and this tends to improve after correction irrespective of the underlying lesion. However, catch up growth is slow especially if CHD is corrected late.

These have been listed in Table 7.2.6. They contribute substantially to morbidity in CHD. Chronic hypoxia, in utero hypoxia and hypoperfusion and open heart surgery (especially operations performed under total circulatory arrest) contribute substantially. Brain abscess is uniquely associated with cyanotic heart disease (typically beyond the age of 2 years) where the right to left shunt bypasses the pulmonary filter.

Older children with cyanotic CHD are prone to complications from a chronically elevated red cell turnover. These include symptoms of hyperviscosity, gout, renal failure and gall stones.

hythm Disorders and udden Death S

Growth and Nutrition

Neurologic and Neurodevelopmental Consequences

R

above lesions). However, the importance of maintaining good dental hygiene in all children with CHD cannot be overemphasized.

Chronic enlargement of heart chambers predispose to tachyarrhythmia. Chronic right atrial (RA) enlargement (such as in ASD, Ebstein’s and severe TR) predisposes to atrial flutter. This can be persistent and refractory. Chronic RV enlargement predisposes to malignant ventricular

vip.persianss.ir

Diseases of Car iovas ular ystem

Table 7.2.6 Management of hypercyanotic spells

c S

   









 

 





d



Immediate steps •  Check airway and start oxygen. If child is uncomfortable with mask or nasal cannula, deliver oxygen via tube whose end is held half to one inch away from nose. This corresponds to delivering 80% oxygen. •  Knee-chest position •  Sedate child with subcutaneous morphine (0.2 mg/kg/dose) or IM ketamine (3–5 mg/kg/dose). •  Obtain a reliable intravenous access. •  Sodium bicarbonate 1–2 mL/kg given as 1:1 dilution or can be diluted in 10 mL/kg of isolyte P, which is given bolus as the initial resuscitating fluid. •  Correct hypovolemia (10 mL/kg fluid bolus of isolyte P or dextrose normal saline). •  Keep the child warm. •  Correct anemia by packed cell transfusion. Hemoglobin levels < 12 g/dL merit correction through a blood transfusion in children with cyanotic spells. •  Start beta blockade. Beta blockade is fairly safe unless a specific contraindication like bronchial asthma or ventricular dysfunction exists. It should always be given with heart rate monitoring. Medications and dosages •  Intravenous metoprolol 0.1 mg/kg, given slowly over 5 minutes – Can repeat every 5 minutes for a maximum of three doses   –  Can be followed by infusion 1–2 µg/kg/min – Monitor saturation, heart rates and blood pressure   –  Aim to keep heart rate below 100/min. •  Intravenous esmolol 500 µg/kg over 1 minute as loading dose, 50 µg/kg/min for 4 minutes; if desaturation persists without a significant decrease in heart rate, the loading dose will need to be repeated and the infusion rate can be increased in 50 µg/kg/min increments until 300 µg/kg/min. This infusion should be maintained at the rate that produces the desired result. Esmolol is relatively expensive but has the advantage of being very short acting. •  Intravenous propranolol (0.1 mg/kg) If desaturation persists and there is still no significant trend toward improvement despite maximum beta blockage •  Start vasopressor infusion. Methoxamine given intravenously at dose of 0.1–0.2 mg/kg/dose or intramuscularly (0.1–0.4 mg/kg/dose). •  Phenylepherine 5 µg/kg as bolus and then 1–4 µg/kg/min as infusion •  If spells are persistent, consider paralyzing the child, elective intubation and ventilation and plan for surgery, which can be corrective or palliative (BT shunt) •  If convulsions occur, consider IV diazepam 0.2 mg/kg or IV midazolam 0.1–0.2 mg/kg/dose, as slow push. After a spell •  After a spell is successfully managed, a careful neurological examination is mandatory. In case of suspicion of neurologic insult during a spell, a CT scan is to be done to assess the presence and extent of cerebral infarcts. •  Initiate maximally tolerated beta blockade (propranolol 0.5–1.5 mg/kg/dose 8 hourly or 6 hourly). The dose can be titrated by the heart rate response. Beta blockade may help improve resting saturation and can decrease frequency of spells. •  Do a detailed segmental analysis by two-dimensional echo for complete diagnosis. •  Plan toward early corrective or palliative operation (depending on the age and anatomy). •  Continue therapeutic (if anemic) or prophylactic iron therapy (if not anemic). Prevention Parents of patients diagnosed to have a cyanotic congenital heart defect should be counseled if the possibility of occurrence of a spell is anticipated: •  Explain to them the circumstances when a spell may occur •  Avoid dehydration •  Rapid control of temperature whenever fever occurs •  Encourage early surgical repair. Obtaining IV access in a cyanotic child can precipitate spells. This can be avoided by sedating child with IM ketamine (3–5 mg/kg) and/or by using local anesthetic skin ointment (Emlap) before attempting for venous access or blood sampling. Abbreviations: BT shunt, Blalock-Taussig shunt; IV, Intravenous; IM, Intramuscular

S

Cyanotic pells All patients with the VSD-PS physiology are prone to cyanotic spells. Cyanotic spells are due to an acute decrease in pulmonary blood flow, increased right to left shunt

and systemic desaturation possibly due to the following reasons: • Infundibular spasm due to increased circulating cate cholamines as a result of effort of feeding or crying. • Activation of mechanoreceptors in right ventricle due to decrease in systemic venous return or that in left ventricle due to decrease in pulmonary blood flow, leading to peripheral vasodilatation and fall in systemic vascular resistance producing increased right to left shunt and systemic desaturation. Same mechanism can 437 account for occasional episodes of bradycardia. ­

tachycardia (VT) and can precipitate sudden cardiac arrest in a small fraction of patients. This is starting to become a significant long-term concern after TOF repair where the pulmonary valve is rendered incompetent. Similarly LV hypertrophy and dysfunction are associated with higher than usual risk of malignant VT.

vip.persianss.ir

 

Younger patients have a higher likelihood of spontaneous closure of defects. Most ASDs and most VSDs that are destined to close or become very small do so before the age of 3 years. PDAs have a tendency to close in the first 2–4 weeks after which they seldom close, particularly in a preterm infant.

Size of the defect

Larger defects have little likelihood of spon taneous closure. ASDs > 8 mm are unlikely to close spontaneously. Similarly, large unrestrictive VSDs are also unlikely to close.

Location of the defect

Only fossa ovalis ASDs have a tendency to close. Primum and sinus venosus type of ASDs do not close. Muscular VSDs have the highest likelihood of spontaneous closure. Perimembranous VSDs can also close spontaneously. Outlet (subpulmonic) VSDs may close by prolapse of the aortic valve with the risk of aortic regurgitation. Inlet VSDs and malalignment type of VSDs (such as those occurring in TOF) do not close spontaneously.

S

urvival of Patients with Congenital Heart Disease The natural history of common forms of CHD is shown in Figure 7.2.1. This figure is an approximation but serves to illustrate the fact that without correction, many children with CHD (especially those with cyanotic CHD) will not survive beyond infancy or early childhood. This is dramatically altered by correction through surgery and in some situations through catheter interventions. Following surgical correction, the natural history of many CHDs is dramatically improved. Flow chart 7.2.2 is a useful guide to outcomes after common operations for CHD. With accumulating follow-up data, however, it is becoming increasingly apparent that many “curative” operations have important long-term sequelae. The best example is surgery for TOF. For many years it was thought that TOF repair was curative. A transannular patch that sacrifices the pulmonary valve and leaves behind severe pulmonary regurgitation 438 was not thought to be of major importance. However, after 25–30 years of experience, it is clear that most patients will

­

Effect of likelihood of spontaneous closure

Age at evaluation

pontaneous Closure of Heart Defects

Abbreviations: ASDs, Atrial septal defects; VSDs, Ventricular septal defects; PDA, Patent ductus arteriosus; TOF, Tetralogy of Fallot

develop progressive RV dilatation with increased risk of late HF and sudden cardiac death. Similarly, there are growing long-term concerns after the arterial switch operation (aortic root dilatation, silent coronary occlusion), AV canal repair (AV valve regurgitation) and coarctation (residual hypertension, aortic aneurysm). Additionally, there are operations that involve placement of conduits (pulmonary atresia, Rastelli operations for TGAVSD-PS). These require replacement with growth. The Fontan operation, a final solution for all univentricular hearts (single ventricle physiology) is best described as palliative with several significant long-term concerns.

Some defects have a tendency toward spontaneous closure and this can influence the timing of intervention. The defects known to close spontaneously are ASD, VSD and PDA (during the first few months of life). The variables that influence likelihood of spontaneous closure include age at evaluation (the likelihood of spontaneous closure declines with age and most ASDs and many VSDs are unlikely to close after the first 3 years of age), size of the defect (smaller defects are more likely to close), location of the defects (fossa ovalis ASDs, perimembranous and muscular VSDs can close on their own). Table 7.2.7 summarizes the variables that influence spontaneous closure. It is important to recognize the possibility of spontaneous closure while interpreting echocardiogram reports in infancy.



Variable



S



Table 7.2.7 Spontaneous closure of heart defects

Prevention Education of the lay public on the dangers of consanguinity, drugs and teratogens in the first trimester of pregnancy, widespread immunization against rubella can prevent CHDs to limited extent. However, since most CHDs do not as yet have an identifiable etiology, there is no effective strategy available for their prevention in the periconceptional period. Fetal echocardiography is emerging as a modality for CHD prevention after 14–16 weeks gestation. Fetal echo is best suited to diagnose relatively severe forms of CHD. Conditions that are involving major chamber discrepancy (such as hypoplastic left heart syndrome), single ventricles, and common AV canal can be identified in a simple screening protocol (the four-chamber view). With some refinement (outflow tract and three-vessel views), additional conditions such as TOF, large VSD, TGA, persistent truncus arteriosus can be picked up as well.

d

c

extbook of Pe iatri s T

Cyanotic spell is a pediatric emergency, which requires prompt recognition and intervention to prevent disabling cerebrovascular insults or death. A cyanotic spell needs to be taken seriously not just because of the immediate threat but also because it indicates the need for early operation. It is commonly seen below 2 years (peaks between 2 months and 6 months). The onset is usually spontaneous and unpredictable and occurs more often in early morning, although can occur anytime in the day. The infant cries incessantly; he is irritable and often inconsolable. Tachypnea is prominent and a cardinal feature. Typically these infants have a pattern of deep and rapid breathing without significant subcostal recession. Cyanosis deepens as the spell progresses. Later gasping respiration and apnea ensues, which leads to limpness and ultimately anoxic seizures. Spells can last from minutes to hours. Auscultation reveals softening or disappearance of pulmonary ejection murmur. The management of cyanotic spell has been summarized in Table 7.2.6.

vip.persianss.ir





Based on anatomy, ASDs are classified as follows: • Fossa ovalis ASD: They are located in the central portion of the atrial septum in the position of foramen ovale. Only these defects are amenable to closure in the catheterization laboratory. • Sinus venosus ASD: They are located in the region of the superior vena cava-right atrium junction. These defects do not have a superior margin because the superior vena cava straddles the defect. • Ostium primum ASD: These defects are created by failure to seal the septum primum. They are in the lower part of the atrial septum and the inferior margin of the defect is formed by the AV valve. • Coronary sinus ASD: An unroofed coronary sinus is a rare communication between the coronary sinus and the left

V I

entricular eptal Defect ncidence

Ventricular septal defect accounts for 15–20% of all CHDs. It is the most common heart defect. A

A

natomy

d

­

Fossa ovalis defects with good margins can be closed percutaneously in the catheterization laboratory with occlusive devices. Others require surgical closure. Closure is recommended before school entry to prevent late complications. Small defects (< 8 mm) can be observed. Spontaneous closure is well recognized in small defects that are diagnosed in infancy or early childhood.

ncidence

Atrial septal defects occur as an isolated anomaly in 5–10% of all CHDs.

reatment

S

S

Heart failure is exceptional in infancy. Pulmonary hyper tension is also relatively rare. A small proportion of patients develop PAH. It appears that those who are destined to develop PAH do so relatively early (often in the second or third decade). Atrial septal defect closure is primarily recommended to prevent complications in late adulthood. These include atrial arrhythmias and HF. T

I

A

trial eptal Defect

Natural History and Complications

c

The physiology of ASD is that of a pre-tricuspid shunt (see the section on physiology of CHD). The heart murmur in ASD originates from the pulmonary valve because of the increased blood flow through this normal-sized valve; therefore, the murmur is systolic in timing. An increased flow through the tricuspid valve may result in a diastolic rumble at the lower left sternal border. The dilated RV cavity prolongs the time required for depolarization of right ventricle resulting in delayed P2. This delay also results from the prolonged “hang-out” interval because of the very low resistance in the pulmonary circulation. Additionally, the large ASD tends to abolish respirationrelated fluctuations in systemic venous return to the right side of the heart, thereby the fixed S2.

S

Physiology







atrium, which produces a clinical picture similar to other types of ASDs.

Diseases of Car iovas ular ystem

The most significant challenge of fetal echocardiography lies in identifying the population at risk. The low incidence of CHD together with relatively small proportion of CHD that occur in “high-risk” pregnancies makes fetal screening of CHD and inefficient exercise. Further, the expertise and equipment required for accurate fetal echocardiography is scarce in regions with poorly developed facilities for pediatric heart care. Once a serious CHD is identified, it is vital to counsel the families carefully and thoroughly about the postnatal manifestations, natural history, surgical options and their long-term outlook. Before 20 weeks of gestation, medical termination of pregnancy (MTP) is an option. After legal period of MTP, fetal echocardiography enables directed delivery at a center with a comprehensive pediatric heart program. This overcomes the logistic challenges of transporting a newborn with CHD and allows prompt institution of treatment in the newborn period (such as PGE 1 for duct-dependent lesions). It is a common practice to recommend fetal echocardiography for future pregnancies after diagnosis of serious CHD in a child. This practice is likely to have a low yield because only 2–8% of CHD tend to recur. The highest chance of recurrence is with obstructive lesions of the left heart. The detection of certain specific chromosomal anomalies in a child should prompt an evaluation of parents to identify a carrier state. Examples of these conditions include partial deletion of chromosome 22 (22q-11 or the CATCH 22 syndrome), translocation variants of trisomy 21) through fluorescence in situ hybridization (FISH) or karyotyping. The growing list of single gene mutations that are known to be associated with CHD that can be detected through laboratory tests may enable precise counseling for a larger proportion of families where a child is affected with CHD.

natomy

The ventricular septum may be divided into a small membranous portion and a large muscular portion. The muscular septum has three components: (1) the inlet, (2) the trabecular and (3) the outlet septum. The trabecular septum is further divided into central, marginal and apical portions. A VSD may be classified into perimembranous, inlet, outlet, central muscular, marginal muscular and apical muscular defects.

Pathophysiology The physiology is that of a post-tricuspid left to right shunt. The magnitude of the shunt is determined by the size of the 439 VSD and the level of PVR. The lower the PVR, the greater the

vip.persianss.ir

A

Patent Ductus rteriosus

A

It is the persistence of normal fetal channel connecting the aorta and pulmonary artery and accounts for 5–10% of all CHDs. Functional closure of the ductus occurs within 12–24 hours after birth due to contraction of the medial smooth muscle. Anatomic closure occurs between 2–3 weeks and is produced by fibrosis of the ductal tissue. Prematurity is associated with delay in the process of closure of the ductus.

440

natomical and Physiological Considerations

PDA needs to be understood in two circumstances: (1) as a source of pulmonary blood flow in cyanotic heart disease with reduced pulmonary blood flow and (2) as a cause of left to right shunts. In the former situation, the PDA tends to be vertical, often from the undersurface of the aorta, while in the latter it connects the roof of the main pulmonary artery with the proximal descending thoracic aorta. In this section, we will focus on the latter only. The PDA is programmed to close early in life. In cases where the ductus fails to close normally, blood will shunt from left to right into pulmonary artery and lungs. This occurs increasingly as the PVR drops and the pressure in the aorta exceeds that of pulmonary artery. The volume of shunted blood will increase pulmonary blood flow, increase venous return to left atrium and cause LA/LV

Persistent patency of ductus arteriosus in preterm infants is well recognized in the era of modern preterm care. The prevalence of PDA in babies less than 1,750 grams is 45% and it increases closer to 80% in very low-birth-weight babies weighing less than 1,200 grams. Patent ductus arteriosus with significant left to right shunt is associated with varied complications, such as necrotizing enterocolitis, intraventricular hemorrhage and chronic lung disease in preterm babies. Effective and timely treatment of PDA enhances preterm survival and reduces risk of complications. Studies showed that closure of hemodynamically significant PDA is beneficial but the timing and best method of closure is not clear. A hemodynamically significant duct is identified clinically by features of HF, bounding pulses, evidence of pulmonary over-circulation (tachypnea, tachycardia and continued requirement of mechanical ventilation). An easily audible murmur often accompanies the hemodynamically significant duct, but this is typically absent if the duct is large and unrestrictive. Echocardiography aids considerably in determining the hemodynamic significance of a PDA. A size of greater than 1mm/kg body weight is considered the minimum for the PDA to be of significance. Additional findings that suggest an important PDA include flow reversal in descending aorta and LA and LV enlargement.

Decisions to close a VSD is dictated by many considerations that include anatomic subtype, number and size of the defects, age at diagnosis, symptom status, comorbidity and logistics of follow-up. Infants with large defects should be referred to a center early for infant heart surgery. The consequences of delaying surgery in the hope that the defect will close spontaneously are, sometimes, quite devastating. They include pneumonia that is severe and refractory to treatment, severe malnourishment and premature pulmonary vascular disease. Catheter closure is usually unrealistic in early infancy because of technical challenges. The long-term results of surgical closure in infancy are excellent.

Clinical Circumstances Preterm Infants

Older Infants Older infants with PDA present like any other post-tricuspid shunts (such as VSD). T

T

reatment

volume overload. The flow in the PDA occurs throughout the cardiac cycle. This results in a murmur, which starts in systole after S1, peaks at S2 and continues in diastole. This is a continuous murmur. The passage of increased blood across the mitral valve produces a mid-diastolic murmur as in any other post-tricuspid shunt. The prolonged LV systole results in delayed closure of the aortic valve and a late A2. With large PDA, S2 may be paradoxically split. The excessive diastolic back flow from the aorta results in a low diastolic pressure and bounding pulses.

reatment

Three modalities of definitive treatment are available for PDA and they need to be tailored to anatomy and clinical circumstances. For the preterm infant, indomethacin or ibuprofen should ideally be the first line of treatment. The younger the gestational age of the infant, the lesser is the likelihood of a response to indomethacin or ibuprofen. Beyond 10 days postnatal age, the ductus rarely responds to indomethacin. In these circumstances, surgery should be considered. Catheter closure has emerged as a very promising option for all forms of PDA. Two methods are available. They include coil occlusion and closure with occlusive devices. Today, surgery is reserved for large ducts in small infants.

d

c

extbook of Pe iatri s T

magnitude of left to right shunt. In VSD, the left ventricle starts contracting before the right ventricle, and highpressure gradient is maintained between two ventricles throughout systole. Hence the murmur is pansystolic. Passing through a normal mitral valve, the relatively larger volume of blood results in a mid-diastolic murmur at the apex. This is a very useful clue indicating a large flow and operability of the lesion. Since the left ventricle has two outlets, it empties relatively early. This results in early A2. Since ejection into right ventricle and pulmonary artery is increased, P2 is delayed. Therefore the S2 is widely split but varies with respiration. In a large VSD with increasing PVR, S2 splitting becomes less and less obvious. It is single once PVR is significantly elevated because of the reduced “hangout” interval.

vip.persianss.ir

d c S



Coarctation of orta

S

Like in AS, PS may involve the valve or occur above or below the valve. Subvalvar or infundibular PS, (“doublechambered right ventricle”) results from hypertrophied muscle bundles within the RV cavity. In the common form of pulmonary valve stenosis, the valve is thickened with fused or absent commissures. This valve characteristically domes in systole; the resultant click is best heard during expiration when the excursion of the valve is exaggerated by a relatively underfilled RV cavity. Another form of pulmonary valve stenosis is the dysplastic valve (frequent in Noonan



Congenital Pulmonary tenosis

Isolated coarctation is more common in males (3:1) and characterized by narrowing of aorta typically located near aortic attachment of ligamentum arteriosum or PDA. It can be a localized or discrete narrowing or associated with long segment tubular hypoplasia of the segment proximal to the coarctation (isthmus). There are two types of presentations described: (1) symptomatic newborns or infants with HF, (2) relatively asymptomatic adolescent or young adults who may have “minor” symptoms in the form of headache, epistaxis and leg fatigue. Depending on severity of obstruction, the femoral pulses are both weak and delayed (due to delayed upstroke of arterial pulse in lower extremity sites) or absent. This is a valuable physical sign and an excellent screening test in newborns as well. In older children, a systolic murmur is present over descending aorta, distal to obstruction (in left interscapular region). Sometimes, a continuous murmur of collaterals between vessels arising from pre- and post-coarctation segment is present over the back of the chest. Hypertension with significant difference in upper and lower limb systolic blood pressures (> 20 mm Hg), which exaggerates on exercise, is an inevitable consequence in older children, and this accounts for most of the late morbidity. Additionally, vascular complications such as aortic dilatation with aneurysm formation, and berry aneurysms in cerebral vasculature are commonly seen. The ECG in symptomatic infant shows RVH while left ventricular hypertrophy (LVH) is present in older children and adult. Newborns with severe coarctation require surgery for 441 lasting relief. In older children, balloon dilatation is a less invasive option and in adolescents and adults, stenting of







The most common cause of congenital AS is bicommissural aortic valve. The partial or complete fusion of commissures between right-left or right noncoronary commissures results in variable degrees of severity of AS. Very severe stenosis presents early in newborn period with LV dysfunction and HF; less severe forms present later. Other forms of LV outflow tract obstruction are less common and include subaortic membrane and supravalvar AS. The latter is seen in association with the Williams-Beuren syndrome. The symptoms of AS are related to LV outflow obstruction and include effort angina, dyspnea, syncope and fatigue on effort. There is a significant risk of sudden death with sever AS especially in adolescent and young adults. The murmur of AS is loudest over the ascending aorta [i.e. upper right sternal border (URSB) or aortic valve area] and characteristically radiates to the carotid arteries. Constant systolic ejections click just after S1 is a consistent physical finding in bicommissural aortic valve. This is highly sensitive and specific in children and often better heard at the apex. The ECG is characterized by evidence of LV hypertrophy (voltage with strain in severe cases). Severe aortic valve stenosis can be relieved by balloon dilatation in the catheterization laboratory. Many of these patients require aortic valve surgery to deal with progressive AR. Other forms of AS require surgical relief.

A

S

A

Congenital ortic tenosis



L

O





These lesions can be divided into two groups: 1. Ventricular outflow obstructive lesions, i.e. • Aortic stenosis • Pulmonary stenosis • Coarctation of aorta 2. Stenosis of AV valves/ventricular inflow tract: • Congenital mitral stenosis • Supramitral ring • Cor triatriatum • Tricuspid stenosis Features common in ventricular outflow obstruction are concentric hypertrophy of the chamber proximal to obstruction due to pressure overload and an ejection systolic murmur (ESM) due turbulent flow of blood through the obstruction.

syndrome) that is characterized by tethering of the leaflets to the adjacent main pulmonary artery with narrowing of the main pulmonary artery at the sinotubular junction. Supravalvular PS often refers to the narrowing of branch pulmonary arteries. Most patients are acyanotic and well developed. Newborns with critical PS are cyanotic and tachypneic. Majority of patients are asymptomatic in infancy and childhood. Dyspnea and fatigue are mild as long as the right ventricle maintains normal stroke volume at rest and augments its stroke volume with exercise. The murmur of PS is loudest over main pulmonary artery (i.e. second LICS) and is associated with systolic thrill. The intensity and length of ESM is directly proportional to severity of PS. Wide spitting of S2 is due to delayed closure of pulmonary valve. The pulmonary component of S2 is soft. An inconstant ejection click (louder in expiration) is also audible at the site of the murmur. The interval between S1 and click varies inversely with the degree of PS. The ECG shows right ventricular hypertrophy (RVH). Neonates with critical PS may show predominant LV forces because of associated hypoplasia of right ventricle. Balloon valvotomy, a relatively simple intervention, is now accepted as the standard treatment for valvar PS. While dysplastic valves have a characteristically suboptimal response, balloon is still attempted in them.

Diseases of Car iovas ular ystem

bstructive esions

vip.persianss.ir

E

A

1. Allen HD, Shaddy RE, Driscoll DJ, et al. Moss Adams’ Heart Disease in Infants, Children and Adolescents, 7th edition. Philadelphia, USA: Kluwer/Lippincott William and Wilkins; 2008. 2. Kumar RK, Prabhu SS, Ahamed Z. IAP Specialty Textbook of Pediatric Cardiology. New Delhi: Jaypee rothers; 2008. 3. Kumar RK, Shrivastava S. Pediatric heart care in India. Heart. 2008;94;984-90. 4. Vaidyanathan B, Radhakrishnan R, Sundaram KR, et al. What determines nutritional recovery in malnourished children undergoing correction of congenital heart defects? Pediatrics. 2009;124:e294-9. 5. Vaidyanathan B, Sathish G, Mohanan S, et al. Clinical screening for congenital heart disease at birth: a prospective study in a community hospital in Kerala. Indian Pediatr. 2011;48:25-30. 6. Viswanathan S, Kumar RK. Assessment of operability in congenital cardiac shunts with increased pulmonary vascular resistance. Cathet Cardiovasc Interv. 2008;71: 665-70.

















b



ibliography



B

There is downward displacement of septal and posterior leaflets of the tricuspid valve into the RV cavity. A portion of right ventricle is incorporated in right atrium (atrialized right ventricle). This contracts asynchronously with the right ventricle resulting either in right to left shunting via a patent foramen ovale (majority) or HF (rare). Additionally, there is a leak through the severely deformed tricuspid valve. Presentation is variable and dependent on the severity of the involvement. Severe disease may manifest in the newborn period. Mild disease may resent for the first time in adulthood. Re-entrant arrhythmias are the frequent accompaniment and may result from accessory (bypass) tracts in the region of the displaced leaflets.





Among the cyanotic CHDs, TOF has a relatively favorable natural history that allows survival beyond infancy in about 75% of cases. As a result, it is the most common CHD encountered beyond the age of 1 year. The disease has been a subject of intense study over several decades. The physiology is that of VSD with PS and has been described earlier in this chapter. Anatomically, it is characterized by the classic tetrad: severe RV outflow obstruction, large VSD, aorta that overrides the VSD and RVH. Within this classic tetrad, there are numerous anatomical variations, most of which have an important bearing on treatment strategies. Corrective operation for TOF involves closure of the VSD and relief of the RVOT obstruction. There is growing emphasis on retaining the pulmonary valve during initial repair to prevent pulmonary regurgitation and its major late consequences (RV dilatation, arrhythmia, HF and sudden death). However, this is not often possible if the component(s) of the RVOT are small. Although corrective operation is feasible in very young infants, many centers opt for palliative options initially. Palliation is possible in the very young through the BlalockTaussig shunt, balloon dilatation of the pulmonary vale or stenting of the patent arterial duct (if present). Cyanotic spells are the major concern in severe TOF and the management and prevention has been elaborated upon in the section on complications.

bstein’s nomaly



etralogy of allot F

T

d

c

extbook of Pe iatri s T

the coarctation segment can yield maximum reduction in gradient. Irrespective of the treatment modality chosen, all patients with coarctation should ideally be followed lifelong for hypertension and vascular complications that may occur in spite of relief of the obstruction.

442

vip.persianss.ir

heumatic ever F

R



Diseases of Car iovas ular ystem

7.3

Savitri Shrivastava



Several studies have suggested that RF follows Group A streptococcal pharyngitis. Outbreaks of RF usually occur with epidemics of pharyngitis due to rheumatogenic strains of streptococci. Rheumatic fever and recurrences can be prevented with specific antimicrobial treatment. The most accepted postulation is that M proteins from the rheumatogenic strains share certain epitomes with cardiac myocytes and sarcolemmenal membrane protein. Antibodies produced in the host by these epitomes cross react with cardiac tissues and produce pancarditis (i.e. involving pericardium, myocardium and endocardium). The data is not clear if this host response is related to genetic programming, acquired alterations in host defense or immunological mechanisms. Pathologically, acute RF results in diffuse exudative and proliferative reaction resulting in infiltration with lymphocytes, polymorphonuclear leukocytes, histiocytes and eosinophils. In some areas, typical Aschoff nodules are formed with aggregation of multinucleated giant cells. These inflammatory changes are diffuse affecting heart, large joints, brain and subcutaneous tissue. Long-term sequelae occur as a result of involvement of endocardium of valvular and subvalvular region resulting in valvular heart disease. The most common involvement is of mitral valve (most common being MR) followed by aortic (AR is more common), tricuspid and very rarely pulmonary valve. Microscopically, there is edema, inflammatory cell infiltration and capillary proliferation. Inflammatory infiltrates are also frequently seen in myocardium with scanty injury and loss of myocardial fibers. Pericardial involvement results in fibrinous pericarditis, which may result in pericardial effusion which is serosanguinous. It usually resolves completely, never causing constrictive pericarditis. Small pericardial effusion is common but cardiac tamponade rarely occurs.

c

d



S

F

Clinical eatures The classical clinical picture of RF consists of streptococcal sore throat followed 10 days to few weeks later by various manifestations of RF, although the history of acute streptococcal sore throat is available only in 50% of patients. The guidelines for the diagnosis of acute RF were originally suggested by Dr T Duckelt Jones in 1944, subsequently revised by the American Heart Association in 1965, updated in 1992 and latest revised and updated (Table 7.3.1) by WHO in 2003. The guidelines consist of major, minor and essential criteria. Two major or one major and two minor criteria are required in the presence of essential criteria to diagnose acute RF. It is important to emphasize that these guidelines are meant to help a physician in making a firm diagnosis of RF and do not mean that a physician should not use his/her clinical judgment in diagnosing acute RF in the absence of these criteria. Diagnosis of acute RF requires two major or one major and two minor manifestations and evidence of group A streptococcal infection.

tiopathogenesis

Acute RF with carditis results in valvular regurgitation most commonly of the mitral valve of variable severity. This is due to plastering and thickening of posterior leaflet, thickening and retraction of anterior leaflet and lack of normal coaptation. With aortic involvement, AR is the most common abnormality. Rarely, the tricuspid valve is involved; pulmonary valve is usually not involved in the acute attack.

Table 7.3.1 Revised Jones criteria for the diagnosis of acute rheumatic fever Major criteria

Minor criteria

•  Carditis

•  Arthralgia

•  Chorea

•  Previous rheumatic fever or rheumatic heart disease (RHD)

•  Erythema marginatum •  Polyarthritis

•  Fever

•  Subcutaneous nodules

•  Elevated erythrocyte sedimentation rate (ESR) or C-reactive protein or leukocytosis

­

E

In early 20th century, there has been a marked decline in the prevalence of rheumatic fever (RF) in developed world. The earlier decline is related to the improved standards of living hygiene, health, literacy (particularly of females), medical facilities, etc. Further decline occurred with easy availability of penicillin to treat streptococcal throat infection. The reported incidence of RF in Indian series varies from 0.42–10.9 per 1,000 and the prevalence of rheumatic heart disease (RHD) using clinical criteria as reported varies from 0.56–11 per 1,000. Studies from some parts of India (Kerala/Chandigarh) suggest that there is a decline in the prevalence. Recent studies in India using echocardiography show a prevalence of RHD from 0.12–0.67 per 1,000.

•  Prolonged PR interval on ECG Essential criteria Evidence for recent streptococcal infection as indicated by: •  Increased levels of antibodies against streptococci •  Positive throat culture for Group A streptococcus •  Recent scarlet fever

443

vip.persianss.ir

Noncardiac Manifestations of Rheumatic Fever

444

rthritis: Rheumatic arthritis is a fleeting polyarthritis involving large joints like knees, ankles and elbows. Uncommonly smaller joints can be involved. The arthritis is typically migratory. The affected joints have redness, A

•

S E

M

inor Criteria

They are divided into clinical and laboratory.

Clinical ­

F

ever: Usually present in 90% of patients, the tem perature usually goes up to 39.5°C. • rthralgia: Subjective joint pains without physical signs are labeled as arthralgias. Many Indian authors take it as major criteria. • History of previous RF or RHD. A

•



Laboratory •

Acute phase reactants consisting of polymorphonuclear leukocytosis, increased sedimentation rate and pre sence of C-reactive protein. The leukocyte count usually is between 10,000/cmm and 15,000/cmm. The sedimentation rate is almost always elevated during acute RF and remains so for 4–10 weeks in almost 80% of patients. In a small proportion of patients, it may remain elevated even beyond 12 weeks. C-reactive protein is a beta globulin, which is increased uniformly in all patients of acute RF. Absence of raised C-reactive protein goes strongly against the diagnosis of acute RF. Presence of C-reactive protein, however, is not diagnostic since it becomes positive in many infections. • lectrocardiogram: Prolonged PR interval is a nondiagnostic criterion since it can occur in many infections. Higher grades of block like second-degree ­

E

M

The rheumatic carditis is a pancarditis involving pericardium, myocardium and endocardium. Carditis occurs in 34–90% of patients with acute RF. It is an early manifestation of RF so that by the time a patient seeks help, the patient already has evidence of carditis. Almost 80% of those patients who develop carditis do so within the first 2 weeks of the onset of RF. • Pericarditis: Pericarditis results in precordial pain, which may be quite severe. On auscultation, a pericardial friction rub is present. Clinical pericarditis is seen in approximately 15% of those who have carditis. The ECG may show ST and T changes consistent with pericarditis. As a rule, rheumatic pericarditis is associated with only small pericardial effusion and does not result in either tamponade or constrictive pericarditis. A patient of rheumatic pericarditis always has additional MR or AR murmurs or both. If after disappearance of the pericardial friction rub there are no murmurs, it is unlikely to be due to RF. • yocarditis: The features diagnostic of myocarditis are (1) cardiac enlargement, (2) soft S1, (3) protodiastolic (S3) gallop, (4) congestive cardiac failure and (5) Carey Coomb’s murmur. Carey Coomb’s murmur is a low pitched delayed diastolic mitral murmur heard during the course of acute RF. It tends to disappear after the myocarditis subsides. Most likely it is due to increased diastolic flow secondary to MR across inflamed rigid mitral valve. The disappearance can be explained by the decrease in the LV size following subsidence of myocarditis and better function of mitral valve— papillary muscle complex resulting in decrease in MR and subsidence of inflammation causing decrease in turbulent flow. • ndocarditis: Endocardial lesions are most important and tend to leave permanent scarring in most cases. The endocardial inflammation is most severe and common in the mitral and aortic valves, less common in the tricuspid valve and least in the pulmonary valve. The inflammation results in regurgitation murmurs. The most common finding of carditis is apical pansystolic murmur of MR occurring in 92–95% of patients. Aortic valve involvement results in early diastolic murmur of AR. Aortic valve is involved in 20–25% of cases; it is an isolated finding in only 5–8% of cases. Pathologically, tricuspid valve involvement is seen in 30–50% of cases. Clinical involvement of tricuspid valve in first attack of RF is extremely uncommon. Pulmonary valve involvement alone is almost never seen in acute RF.



M

Carditis

warmth, swelling, pain and limitation of movements. It is an early manifestation and occurs in 30–50% of cases in our country; there is no residual damage of the joints. • ubcutaneous nodules: Subcutaneous nodules appear as nontender, nonadhesive nodules varying in size from pin head to almond on body prominences like shin, wrist, elbow, knee, ankle, spine and occiput. They are late manifestations occurring around 6 weeks after the onset of RF, rarely as early as 3 weeks after the onset. In Indian series, their incidence is 5–20%. Usually they disappear in few days to few weeks rarely lasting even up to 1 year. Patients who have nodules always have carditis. Persistence of nodules is associated with chronic carditis. • Chorea: Chorea is characterized by purposeless jerky movements resulting in abnormal speech, muscular incoordination, dropping of articles, awkward gait and weakness. There is also emotional instability. Chorea is three to four times more common in females. It is a late manifestation occurring about 3 months after the onset of acute RF. It is reported to occur in 5–36% of cases. It is usually self limiting. • rythema marginatum: The rash is reddish, not raised above the skin and non-itching, with serpigenous margins. It starts as a red spot with a pale center, increasing in size to coalesce with adjacent lesions to form serpigenous margin. It increases by applying heat. Though specific but seen extremely rarely in India, probably due to dark complexion.

E

d

c

extbook of Pe iatri s T

ajor Criteria

vip.persianss.ir

T

reatment



There is no specific treatment. Management is symptomatic combined with suppressive therapy (Table 7.3.2). In suppressive therapy, either aspirin or steroids can be used. The total duration of aspirin or steroid therapy is generally 12 weeks. With aspirin, the full doses are given for 10 weeks and then tapered off in next 2 weeks. Full doses of steroids are given for 3 weeks and then tapered very gradually in the next 9 weeks. The most commonly used steroid is prednisone. The dose is 60 mg/day for patients weighing more than 20 kg. This is continued for 3 weeks and then reduced to 50 mg/ day for 1 week and 40 mg/day for another week. Following this, the reduction in dose is by 5 mg/week. In patients

c

d

Bed rest •  It is advised in all patients with carditis till activity subsides. •  Immobilization may have to be continued for 2–3 months especially in the presence of congestive failure. Diet •  Salt restriction is not necessary unless congestive cardiac failure is present. •  Easily digestible nutritious diet with vitamin supplements should be given. Antimicrobial therapy •  Penicillin: After testing skin sensitivity, procaine penicillin 400,000 units of, intramuscularly (IM), twice daily for 10 days followed by prophylactic injection of benzathine penicillin –  1.2 mega units every 21 days above 10 years of age. –  0.6 mega units 1 M every 15 days if the age is less than 10 years. •  Patients sensitive to penicillin should be advised to take erythromycin 20–40 mg/kg in two divided doses. Suppressive therapy Aspirin or steroids are given as suppressive therapy. •  For patients having carditis with congestive cardiac failure, use of steroids is generally mandatory. •  For carditis without congestive cardiac failure, one may use either steroids or aspirin; however, the author prefers use of steroids. •  For patients without carditis, it is preferable to use aspirin. Management of chorea •  Usually self limiting •  Complete physical and mental rest •  In resistant cases, phenobarbitone, 3–5 gm/kg/day •  Chlorpromazine, diazepam, diphenhydramine, haloperidol or promethazine can also be used to provide sedation.

S

­

The essential criteria include evidence of recent strep tococcal infection. • The best out of these is the presence of antibodies against streptococci. The most common in use is the antistreptolysin “O” titer. Considerable confusion exists regarding the levels, which should be considered significant as the normal values differ significantly in different areas. Elevated levels of antistreptolysin “O” only indicate previous streptococcal infection and not RF. The higher the level, more the likelihood of a recent streptococcal infection; lower level considered “normal” do not necessarily exclude a recent streptococcal infection. Rising titers of antistreptolysin “O” is a strong evidence of a recent streptococcal infection. To increase the sensitivity, other antibodies can also be tested. Deoxyribonuclease B (anti-DNase B), antihyaluronidase (AH), streptozyme (SZ) are used by many centers. Single antibody sensitivity is 70%. With two antibodies tested, it rises to 90% and with three antibodies tested, it rises to 95%. With paired sera testing of single antibody, the sensitivity is 80% rising to 99% if paired sera of two antibodies are tested. • Positive throat culture for streptococci is relatively uncommon when a patient presents with acute RF. Positive throat culture cannot be equated with the diagnosis of RF. Positive throat culture means that streptococci are present in the throat. The patient may or may not have RF. • The third feature suggestive of the diagnosis of recent streptococcal infection is the history of recent scarlet fever. The desquamation of skin of palms and soles indicates that the patient has had scarlet fever within the previous 2 weeks. Scarlet fever is rare in India. • Rapid streptococcal antigen test was also tried but because of low sensitivity although high specificity, it was not found very useful.

Table 7.3.2 Treatment of acute rheumatic fever

weighing 20 kg or less, the dose is 40 mg/day for 2 weeks and then reduced by 5 mg/week. The dose of aspirin is 90– 120 mg/kg/day in four divided doses (if facilities for blood salicylate level estimation are available, the dose is modified to maintain a blood salicylate level of 20–25 mg/dL for 10 weeks and later tapered off over 2 weeks).

Prevention It would be ideal to provide primary prevention of RF. Primary prevention requires identification of streptococcal sore throat and its treatment with penicillin in the population. The treatment modalities have been summarized in Table 7.3.3. Table 7.3.3 Drugs for primary prophylaxis of acute rheumatic fever  

E

ssential Criteria

Diseases of Car iovas ular ystem

AV block especially of the Wenckebach type may also be seen. Complete AV block is extremely rare as a manifestation of acute RF. Prolongation of QTc (corrected QT interval) is suggestive of myocarditis.

Drugs

Dose

Sore throat treatment (duration)

Benzathine penicillin G (deep IM injection) after sensitivity test (AST)

1.2 million unit (> 27 kg) 0.6 million unit (< 27 kg)

Single dose

Penicillin V (oral)

Children: 250 mg qid Adult: 500 mg tid

10 d 10 d

Azithromycin (oral)

12.5 mg/kg/day once daily

5d

Cephalexin (oral)

15–20 mg/kg/dose bid

10 d

445

vip.persianss.ir

itral egurgitation R

M

In pediatric cardiac practice, roughly 20–25% of cases in general have RHD, resulting in crippling valvular heart disease in many of them. Mitral valve is the most common valve involved in RHD. It may be regurgitant, stenotic or may have combined regurgitation and stenosis, combined mitral and aortic valve disease in the next common subset. Isolated aortic valve involvement is seen in only 2–8% of cases. Isolated rheumatic tricuspid valve disease does not occur. Tricuspid regurgitation—functional or organic is quiet common. Tricuspid stenosis occurs rarely and mostly with MS.

Clinical Features

In a child with MR, one should keep in mind other causes like mitral valve prolapse, cleft mitral valve, L-transposition with regurgitant left AV valve and connective tissue disorders.

Investigations lectrocardiogram: It reveals sinus rhythm and may be normal with mild MR. With increasing degree of MR, left atrial (LA) overload and LV volume overload is seen. Left atrial enlargement results in bifid P wave (P-mitrale). Left ventricular enlargement results in tall R waves in leftsided leads (I, aVL, V5, V6) with deep q waves indicating volume overload of left ventricle. In cases of severe MR, RV hypertrophy may be seen due to the development of pulmonary hypertension. • Chest -ray: Significant MR results in LA and LV enlargement with changes of pulmonary venous hypertension. • chocardiogram: It clearly shows the altered valve pathology, degree of LA and LV enlargement and function, and quantum of regurgitation. E

•

Management Symptomatic patients with moderate-to-severe MR will need decongestive therapy [including angiotensin converting enzyme (ACE) inhibitors]. The patients should be carefully evaluated if the mitral valve could be repaired surgically. Patients with severe MR with decreasing LV ejection fraction need early surgical intervention. If the valve can not be repaired, they will need mitral valve replacement.

itral tenosis S

heumatic

M

R



Exertional fatigue and palpitation are common features. Exertional dyspnea and features of right-sided cardiac failure are late manifestations of severe grades of MR and LV failure. There is no tachycardia, tachypnea or basal rales unless significant LV overload/failure occurs. The pulse pressure is wide with significant MR. The cardiac size increases with increasing severity of MR resulting in LV hyperdynamic impulse downwards and outwards. There may be a palpable LVS3. The S1 may be soft, normal or loud. It may be masked by the murmur. The S2 is normal with mild MR; with severe grades of MR, it becomes wide variable split due to early A2 as LV systole is shortened due to part of stroke volume regurgitating in the left atrium. With severe MR and CHF, P2 446 becomes loud due to high pulmonary arterial pressures. Left ventricular S3 is generally audible with significant MR due to

Differential Diagnosis

X

R

Chronic heumatic Heart Disease

increase in early diastolic filling. Moderate-to-severe degree of MR results in a low-pitched delayed diastolic murmur at the apex, which may be palpable as a diastolic thrill. A diastolic thrill is more commonly felt with MR than a systolic thrill. Systolic thrill is uncommonly felt at apex in MR as the blood is regurgitating in the left atrium, which is posteriorly placed. The diagnostic feature of MR is a pansystolic murmur best heard at the apex and radiating to the axilla. With significant MR, a low frequency delayed diastolic murmur is audible at the apex; the duration of murmur increases with increasing severity of MR. If MR is severe, the diastolic rumble is audible throughout diastole but has no presystolic accentuation in absence of MS. In patients with severe MR, there are also features of CHF like raised JVP and hepatomegaly.

E





d

c

extbook of Pe iatri s T

Its success depends on the awareness of parents regarding dangers of sore throat. Therefore, for primary prevention it is necessary to educate the community regarding the consequences of streptococcal sore throat. Logistically, it is difficult in our country since it requires (1) identification of sore throat, which is dependent on education of parents, (2) rapid laboratory confirmation of streptococcal infection of the throat and (3) medical help and availability of penicillin. Secondary prevention consists in giving long-acting benzathine penicillin intramuscularly. The dose is 1.2 mega units once every 3 weeks in patients above the age of 10 years or 0.6 mega units every 2 week below the age of 10 years. The injection is painful and some patient gets fever for 24–36 hours following the injection. As such, it is preferable to give the injection on a Saturday afternoon to avoid loss of studies for the child. In presence of carditis, ideally penicillin prophylaxis should continue life long. Less than ideal would be to continue it till the age of 35 years. If there is only polyarthritis but no carditis, usually the penicillin prophylaxis should be given for 5 years after the attack and subsequently all clinical attacks of streptococcal sore throat should be treated with penicillin. If there is allergy to penicillin (which is very rare in children), erythromycin can be used but this is not ideal.

Clinical Features In pediatric age group, male patients commonly have MS. The patient complains of shortness of breath on exertion initially. With increasing severity, this becomes more severe and the child becomes breathless on walking short distances. Subsequently, the child starts getting paroxysmal nocturnal dyspnea and breathlessness at rest. Later stages with development of severe pulmonary hypertension and TR features of CHF develop. The patient can also have

vip.persianss.ir

lectrocardiogram: The rhythm is usually sinus; LA overload is seen. With increasing pulmonary hypertension, increasing degree of right axis deviation, RV hypertrophy (tall R wave in right-sided leads) and RA overload (tall peaked “P” wave) are seen. • Chest -ray: Heart size is usually normal, with development of TR and CHF. Cardiomegaly develops due to RA and RV enlargement. The left atrium is enlarged. Features of varying grades of pulmonary venous and arterial hypertensions are also seen. Two-dimensional echocardiogram with color flow mapping clearly shows the stenotic mitral valve; the mitral

X

E

•

Differential Diagnosis

d

Cor triatriatum, supramitral ring, congenital MS and LA myxoma are rare entities that can sometimes mimic the presentation of MS.

Management

S

c

Salt restricted diet and diuretics are useful to reduce the pulmonary venous congestion. If there is evidence of cardiomegaly and CHF, digoxin is useful. Basically the mitral obstruction has to be relieved. Presently, balloon dilatation of mitral valve percutaneously is the method of choice. If it is not feasible, surgical closed mitral valvotomy is done. Both procedures have identical results. Both these procedures relieve the commissural fusion and to some extent the subvalvular fusion but chordae shortening and fusion are not relieved. If there is marked fibrosis of the valve and subvalvular apparatus, open surgical commissurotomy may be needed.

ortic egurgitation R

A



Investigations

valve area can be calculated. The gradient across the mitral valve is calculated by taking the velocity of the mitral inflow on Doppler. The peak velocity of TR gives the estimate of pulmonary arterial pressures.

Diseases of Car iovas ular ystem

hemoptysis or pulmonary edema due to severe pulmonary venous hypertension. On examination, there may be tachycardia and low pulse volume. Jugular venous pressure is normal with mild MS. With severe MS and TR, elevated JVP with prominent “V” wave (due to regurgitation of blood into right atrium) and rapid “Y” descent is seen (in the presence of TR without tricuspid stenosis). Enlarged and tender hepatomegaly, which may be pulsatile, if seen with significant TR, is seen. Respiratory rate with mild MS is normal; tachypnea develops with significant MS. Basal crepitations are also heard with significant MS. If the patient develops pulmonary edema severe respiratory distress, extensive rales and cyanosis are seen. Precordial examination reveals normal heart size in most cases. Cardiac enlargement is seen in the presence of CHF and TR. The apex beat is tapping; parasternal heave is present with the development of pulmonary hypertension. At this stage, P2 many also be palpable. Mild TR does not result in RV dilatation. Therefore, there is no parasternal pulsation. Significant TR results in RV dilatation resulting in parasternal lift. If the pressure in the right ventricle is elevated, parasternal pulsations can be seen and on palpation parasternal heave is felt. A systolic thrill is rarely felt along left or right sternal border. A diastolic thrill could be felt at the apex. On auscultation, the S1 is loud, S2 is normally split with loud pulmonary component, loudness depending on the degree of pulmonary hypertension. An opening snap—hallmark for the diagnosis of MS is audible at the apex at the beginning of diastole. The closure the opening snap to the A2, the severe is the MS. A low-pitched delayed diastolic rumble is audible at the apex with presystolic accentuation immediately after the opening snap. In the presence of significant TR, systolic pulsations can be felt over the liver, which enlarges with the development of CHF. A systolic murmur along the left sternal border increasing with inspiration or leg raising is the hallmark for the diagnosis of TR. With significant regurgitation, S3 can be heard in the tricuspid area; if the quantum of regurgitation is significant, a delayed diastolic murmur across tricuspid valve is heard. Rarely, an opening snap and presystolic accentuation of the diastolic murmur is heard if tricuspid stenosis is present.

Clinical Features Aortic regurgitation is common in male patients. The presenting symptom is usually palpitation and increased neck pulsations due to high stroke volume. Exertional fatigue and dyspnea develops in patient of significant AR, which become more prominent with the development of CHF. The pulse pressure is the best clinical parameter to asses the degree of AR. The wider the pulse pressure, severe is the regurgitation. The wide pulse pressure results in several signs (Table 7.3.4). It also results in prominent neck, suprasternal, peripheral and abdominal pulsations. The pulse shows a sharp rise and fall the so called water hammer pulse that becomes more evident on elevating the arm. Nodding of the head with cardiac cycle may be seen (de Musset’s sign). The increased pressure difference between the systolic pressure of upper and lower limbs is called Hill’s sign. Normally this difference is less than 20 mm Hg. With mild, moderate and severe regurgitation, it is 20–40 mm Hg, 40–60 mm Hg and greater than 60 mm Hg, respectively. Pistol shot sounds can be heard over large arteries with significant regurgitation. If slight pressure is applied over these arteries, a systolic murmur can be heard and if pressure is applied distally, a diastolic murmur is heard. This is called Durouzieu’s sign. Palpation of the precordium may be unremarkable with mild AR; with significant regurgitation, the cardiac impulse is displaced down and out and is of LV hyperdynamic type. The greater the amount of regurgitation, the bigger is the heart size. Rarely with severe regurgitation, the whole of

447

vip.persianss.ir

E

Differential Diagnosis In a case of isolated AR, one should look for Marfan’s syndrome, Hurler’s syndrome and nonspecific aortoarteritis. The other Table 7.3.4 Peripheral signs of aortic regurgitation (AR) Corrigan’s pulse

Dancing carotids

De Musset’s sign

Head nodding

Quinke’s sign

Capillary pulsations over nail bed

Traube’s sign

Pistal shot sounds over femorals

Hill’s sign

Lower extremity blood pressure > upper extremity blood pressure > 20 mm Hg—Mild AR > 40 mm Hg—Moderate AR > 60 mm Hg—Severe AR

Management Mild AR is well tolerated. Significant AR causing LV dilatation may benefit with ACE inhibitors. If the left ventricle is progressively dilating and the patient is symptomatic or there is evidence of CHF, decongestive therapy along with ACE inhibitor should be started and patient planned for surgery, aortic valve repair or replacement. In most of these patients, the aortic valve repair is not possible and they end up with valve replacement. The improvement with surgery is remarkable but with prosthetic valve, the child has to take life-long anticoagulant therapy with its own inherent hazards.

ricuspid egurgitation R

lectrocardiogram: The ECG may be normal with mild AR. If significant regurgitation is present, the ECG shows evidence volume overload of left ventricles—increased LV voltage with prominent “Q” waves and tall “T” waves. In patients with severe AR, particularly in presence of CHF, LA overload may also be seen. • -ray chest: In cases of mild AR, there is no cardiomegaly and aorta may be prominent. With increasing AR, heart size enlarges due to enlarged left ventricle. Changes of pulmonary venous hypertension develop with severe regurgitation and LV failure. • chocardiography: Two-dimensional and color Doppler echocardiography can confirm the diagnosis and also estimate the severity of AR, degree of LV enlargement and function. X

•

causes of AR like congenital aortic valve disease, rupture sinus of Valsalva or VSD with AR should be kept in mind.

T

Investigations E

d

c

extbook of Pe iatri s T

the precordium is pulsatile. The S3 may be felt over the LV apex. In some cases, a systolic thrill may be felt in second LICS. The S1 is soft, the S2 is normally split with mild-tomoderate regurgitation; it may be paradoxical with severe regurgitation particularly with LV failure. The murmur of AR is high pitched decrescendo diastolic starting with A2. A systolic ejection murmur is commonly heard in the aortic area conducted to the neck in cases with significant AR. Delayed diastolic rumble is heard in some cases of AR (Austin Flint murmur). Some of the other signs have been listed in Table 7.3.4.

Tricuspid regurgitation is commonly seen in patients of rheumatic mitral valve disease (20–50%). It can be functional or organic. If TR is present in absence of severe PAH, it is likely to be organic, but if PAH is present it can be either functional, organic or both.

Clinical Features There are no specific symptoms of TR except pain in right hypochondria and fatigue due to low cardiac output. The symptoms will depend on the lesion with which TR is associated. The signs diagnostic of TR are prominent “V” wave with rapid “Y” descent in JVP, systolic pulsations over liver, systolic murmur at lower left or right sternal border, increasing in severity with inspiration. It may sometimes be associated with a systolic thrill, enlarged right ventricle, S3, delayed diastolic rumble over tricuspid area. Tricuspid stenosis results in slow Y descent in JVP, diastolic rumble with presystolic accentuation in the tricuspid area and rarely an opening snap before the diastolic rumble. The other physical finding will be of the associated lesion; isolated tricuspid valve diseases of rheumatic etiology do not occur.

Investigations Electrocardiogram may reveal evidence of RA and RV overload pattern in addition to other changes due to the associated lesions. Significant TR results in cardiac enlargement due to dilatation of right atrium and right ventricle. Two-dimensional and color Doppler evaluation easily confirms tricuspid valve disease. The degree of regurgitation is estimated by color flow mapping.

Mueller’s sign

Systolic pulsation of uvula

Rosenbach’s sign

Pulsatile liver

Management

Gerhardt’s sign

Enlarged pulsatile spleen

Light house sign

Blanching and flushing of forehead

Landolfi’s sign

Alternating constriction and dilatation of pupil

Becker sign

Pulsations of retinal vessels

Presence of significant TR results in cardiomegaly and CHF, and the patients need decongestive therapy. The complete management will depend on the associated valvular lesions. Severe grades of TR results in dilatation of tricuspid annulus. If the tricuspid annulus is greater than 21 mm/m2,

448

vip.persianss.ir



d c S

























1. Grover A, Dhawan A, Lyengar SD, et al. Epidemiology of rheumatic fever and rheumatic heart disease in a rural community in northern India. Bull World Health Organ. 1993;71:59-66. 2. Gupta I, Gupta ML, Parihar A, et al. Epidemiology of rheumatic and congenital heart diseases in school children. J Indian Med Assoc. 1992;90:57-9. 3. Jones TD. The diagnosis of rheumatic fever. JAMA. 1994;126:481-4. 4. Kumar PK, Paul M, Francis TP. RHD in Indian: Are we ready to shift from secondary prophylaxis to vaccinating high-risk children? Curr Sci. 2009;97:397-404.

B

ibliography

5. Massell BF, Chute CG, Walker AM, et al. Penicillin and the marked decrease in morbidity and mortality from rheumatic fever in the United States. N Engl J Med. 1988;318:280-6. 6. Misra M, Mittal M, Singh R, et al. Prevalence of rheumatic heart disease in school-going children of Eastern Uttar Pradesh. Indian Heart J. 2007;59:42-3. 7. Padmawati S. Rheumatic fever and rheumatic heart disease in India at the turn of the century. Indian Heart J. 2001;53: 35-7. 8. Sanyal SK, Thapar MK, Ahmed AH, et al. The initial attack of acute rheumatic fever during childhood in North India: a prospective study of the clinical profile. Circulation. 1974;49:7-12. 9. Shrivastava S, Subramanyam K, Tandon R. Severity of rheumatic mitral regurgitation in children. Indian J Pediatr. 1992;59:579-83. 10. Shrivastava S, Tandon R. Severity of rheumatic mitral stenosis in children. Int J Cardiol. 1991;30:163-7.

Diseases of Car iovas ular ystem

it has to be repaired by annuloplasty in addition to the other valvular lesions at surgery.

449

vip.persianss.ir

D

F

C



ongestive Heart ailure: iagnosis and Management

I

F

auses of Heart ailure in nfants and hildren

Children can have diverse causes of HF depending on the age, geographical location and many other factors. The prominent causes of HF or ventricular dysfunction in children have been provided in Table 7.4.1. Table 7.4.2 enumerates the likely causes of HF by age at presentation. This is important as the symptoms and signs of HF can be confusing or fairly nonspecific in children. Heart failure presenting on the first day of life is commonly due to metabolic abnormalities and not CHD. Conditions producing fetal HF such as Ebstein’s anomaly or abnormal heart rate/rhythm are also causes of HF on day 1 of life. Obstructive and duct dependent lesions can present with HF or acute circulatory shock soon after birth. Heart failure due to left to right shunts occurs later (4–6 weeks) as the PVR falls, though large VSD, PDA, atrioventricular septal defect (AVSD) may present earlier. Children with isolated ASD are mostly asymptomatic; if an infant is diagnosed to -



Congestive heart failure is a complex syndrome resulting from multiple causes, which are different in children as compared to adults. This term is increasingly being replaced by heart failure, since peripheral congestion may not be present in all cases. Several definitions have been proposed for HF, which again reflects our less than complete understanding of this enigma. A commonly used definition is: “it is a pathophysiological state in which an abnormality of cardiac function is responsible for the failure of the heart to pump blood at a rate commensurate with the requirements of the metabolizing tissues, or does so only at elevated filling pressures”. In case of children, this requirement includes growth and development. Heart failure in infants and children is commonly secondary to congenital heart defects (shunts or obstructive lesions). Less common causes include cardiomyocyte dysfunction secondary to myocarditis/cardiomyopathies. Palliated CHD leading to HF is increasingly being recog­ nized.

C

C

ntroduction



I

Anita Saxena



t

t

Tex book of Pedia rics

7.4

Table 7.4.1 Causes of heart failure in children by underlying pathology Cause

Comment

Volume overload (relative or absolute)

CHD with increased pulmonary blood flow (VSD, PDA, AVSD, TGA, truncus, TAPVC, etc.)* arteriovenous fistula or malformations, anemia, thyrotoxicosis ***

Obstructive lesions/atretic valves or great vessels

AS, PS, mitral valve atresia or stenosis, coarctation of aorta, aortic interruption**

Regurgitant lesions (MR/TR)

Congenital (e.g. as part of AVSD, Ebstein’s), acquired (e.g. RF/RHD), postoperative*

Myocyte dysfunction Primary

Inborn errors of metabolism, muscular dystrophies, DCM, drug-induced, hemoglobinopathies***

Inflammatory

Myocarditis, Chagas, HIV*

Hemodynamic

Obstructive or regurgitant lesions, HT**

Abnormal rate/rhythms

Tachycardiomyopathy, bradycardia, AV dyssynchrony***

Abnormal morphology

SV physiology**, ALCAPA

Ischemic

CAD (including premature CAD)***

Postoperative

Postbypass, SV surgeries, post-TGA repair**

Abnormal homeostasis

Hypothermia, hypoxia, hypocalcemia, hypoglycemia, sepsis*** (peculiar to neonatal period)

*: common **: uncommon ***: rare (may be common in specific age group/settings)

Abbreviations: CHD, Congenital heart disease; VSD, Ventricular septal defect; PDA, Patent ductus arteriosus; AVSD, Atrioventricular septal defect; TGA, Transposition of great arteries; TAPVC, Total anomalous pulmonary venous connection; AS, Aortic stenosis; PS, Pulmonary stenosis; MR, Mitral regurgitation; TR, Tricuspid regurgitation; RF, Rheumatic fever; RHD, Rheumatic heart disease; DCM, Dilated cardiomyopathy; AV, Atrioventricular; CAD, Coronary artery disease; SV, Single ventricle; ALCAPA, Anomalous left coronary artery from the pulmonary artery

450

vip.persianss.ir

2–6 months

•  •  •  •  •  •  • 

•  Causes at 1–2 months •  Coarctation of aorta •  Aortic stenosis

Older children

•  •  •  •  • 

•  •  •  •  •  • 

Large VSD Large PDA AVSD Persistent truncus arteriosus Unobstructive TAPVC

RF/RHD CHD with complications Cardiomyopathies Severe PS with TR Palliated/postoperative CHD Tachycardiomyopathies

Abbreviations: VSD, Ventricular septal defect; PDA, Patent ductus arteriosus; AVSD, Atrioventricular septal defect; TAPVC, Total anomalous pulmonary venous connection; TGA, Transposition of great arteries; ALCAPA, Anomalous left coronary artery from the pulmonary artery; AS, Aortic stenosis; PS, Pulmonary stenosis; RF, Rheumatic fever; RHD, Rheumatic heart disease; CHD, Congenital heart disease; TR, Tricuspid regurgitation

-

Epidemiology of HF in children is a difficult science given the fact that symptoms, etiology, diagnostic criteria and outcomes are quite heterogeneous. The annual incidence of CHD is about 8 per 1,000 (0.8%) of live births, of which one third are severe enough to warrant attention. Half of these result in HF. Rheumatic fever/rheumatic heart disease is an important cause of HF in children in developing countries like India. While the incidence and prevalence of RF and RHD are well documented, there are no data on presentation with HF in this group, although a significant majority of acute rheumatic carditis and established juvenile MS will present with features of HF.

D

nvestigations

The cornerstones for rapid clinical diagnosis of HF in children are chest radiograph and an ECG.

C

pidemiology of Heart ailure in hildren F

E



have ASD and is in failure, the likely diagnosis is the much more malignant CHD, total anomalous pulmonary venous connection (TAPVC). Heart failure secondary to CHD is unusual after the first year of life unless complicated by IE, anemia, infections or arrhythmias. Thus, older children (usually beyond 2 years) are likely to have other causes for HF like acute/chronic RHD, myocarditis, cardiomyopathies and postoperative CHD.

hest adiograph R

Second week of life

C

Critical AS/PS Obstructive TAPVC Hypoplastic left heart Coarctation of aorta Adrenal insufficiency TGA with intact septum Day 1 causes

I



First week of life

The clinical features of HF in children vary according to the cause and age of the child. An important point to remember is that raised JVP, peripheral edema, effusions and chest crepitations are not seen in neonates and are unlikely in young children as a sign of HF. Chest crepitations, in fact, suggest the possibility of underlying chest infection, which so often accompanies HF in children especially in high pulmonary flow situations. Common clinical features of HF in children have been given in Table 7.4.3. These features are quite nonspecific in neonates and may resemble features of septicemia. Thus a high index of suspicion is required. Routine examination for lower limb pulses is very important otherwise coarctation of aorta will be missed. Coarctation of aorta is not a cause of HF beyond infancy and hence in such cases other diagnoses such as nonspecific aortoarteritis should be sought. One must also remember that neonates with coarctation of aorta or even interruption of aortic arch may have normal lower limb pulses due to PDA. Closure of PDA could be disastrous in these babies. Some of the other important clinical considerations are as follows: • Central cyanosis with HF should always be taken seriously in a neonate, especially if associated with soft or no murmur. • An ASD or VSD does not cause HF in first 2 weeks of life; prompt evaluation should be done for TAPVC or associated coarctation of aorta respectively. • A premature newborn with respiratory distress and a murmur is likely to have a PDA causing HF. • Rule out tachyarrhythmia as a cause of HF if heart rate is above 220 beats/min. • Associated extracardiac and chromosomal abnormalities may provide clues for diagnosis of CHD. • Older children with TOF or TOF physiology may develop HF due to complications such as anemia, IE, AR, large aortopulmonary collaterals.

C

VSD PDA AVSD Aortopulmonary window Unobstructed TAPVC TGA and malposition complexes ALCAPA

S

•  •  •  •  •  •  • 

Asphyxia Metabolic Systemic arteriovenous fistula Myocarditis Hematological

t

•  •  •  •  • 

linical eatures

m

1–2 months

F

C

 

Day 1 of life/fetal

iseases of ardiovascular ys e

Table 7.4.2 Common causes of heart failure by age at presentation

Chest radiograph should be done in all patients with suspected HF; an echocardiogram is not a substitute for radiograph. It enables diagnosis of cardiomegaly, quanti­ fication of pulmonary blood flow, presence of associated chest infection, pleural effusion, etc. as well as being pathognomonic in certain disease states. A cardiothoracic ratio of greater than 60% in neonates and greater than 55% in older children suggest cardiomegaly though expiratory films should be interpreted with caution. A large thymus can also give false impression of cardiomegaly in neonates and infants (Fig. 7.4.1). Cardiomegaly with increased pulmonary blood flow (pulmonary plethora), prominent main and 451

vip.persianss.ir

t

t

 

Tex book of Pedia rics

Table 7.4.3 Clinical features of heart failure by age and associated findings Newborn/Neonates •  •  •  •  •  •  •  •  • 

Tachypnea Tachycardia Hepatomegaly Cardiomegaly Feeding difficulties Excessive sweating Subcostal recession Cyanosis and wheeze Shock

•  •  •  •  •  •  • 

Bounding pulses in arteriovenous malformations, PDA, truncus Asymmetric upper and lower limb blood pressure in aortic arch anomalies Central cyanosis in TGA, TAPVC, truncus, tricuspid atresia with no PS Differential cyanosis in PPHN and R-L shunt through patent ductus Multiple heart sounds in Ebstein’s Ejection systolic murmur in AS/PS Syndromic anomalies (Down’s, Noonan)

HLHS, coarctation of aorta, Interrupted aortic arch, critical AS, tachyarrhythmias, myocarditis

Infants •  •  •  •  •  • 

Poor feeding Lethargy Excessive sweating Tachypnea, tachycardia Slow weight gain Hepatomegaly

•  Precordial bulge, signs of pulmonary hypertension and less impressive systolic murmurs suggest larger L-R shunts •  Crepitations should alert to possibility of chest infection •  Cyanosis in TAPVC, TGA with VSD, AVSD, truncus •  Findings of CHF and cyanosis in suspected ASD suggest TAPVC •  Later onset of HF in infancy can be due to certain forms of TAPVC and ALCAPA

Older children •  •  •  •  •  •  •  •  • 

Poor weight gain Effort intolerance, orthopnea Cardiomegaly Gallop rhythm, murmurs Peripheral edema Basilar crepitations Fatigue Hepatomegaly Raised JVP

•  •  •  • 

Diastolic murmur in a child with known VSD suggests associated AR Pericardial rub in appropriate settings suggests acute RF Hypertension and unequal pulses or bruits suggest nonspecific aortoarteritis Findings of raised JVP, ascites and anasarca should lead to suspicion of constrictive pericarditis or restrictive cardiomyopathy

Abbreviations: PDA, Patent ductus arteriosus; TGA, Transposition of great arteries; TAPVC, Total anomalous pulmonary venous connection; PS, Pulmonary stenosis; PPHN, Persistent pulmonary hypertension of the newborn; AS, Aortic stenosis; HLHS, Hypoplastic left heart syndrome; VSD, Ventricular septal defect; AVSD, Atrioventricular septal defect; CHF, Congestive heart failure; ASD, Atrial septal defect; HF, Heart failure; ALCAPA, Anomalous left coronary artery from the pulmonary artery; AR, Aortic regurgitation; RF, Rheumatic fever; JVP, Jugular venous pulse

E  

Figure 7.4.1 A large thymus may give false impression of cardiomegaly in neonates and infants

452

-

-

branch pulmonary arteries are features of significant left to right shunts (Fig. 7.4.2). Typical radiographs are rare, but if present are diagnostic of certain CHD, e.g. egg on side appearance in TGA (Fig. 7.4.3), normal heart size with ground glass lungs in obstructed TAPVC (Fig. 7.4.4), figure of 8 appearance in unobstructed TAPVC (Fig. 7.4.5) and so on.

lectrocardiogram

An ECG is a much underutilized investigation in children. It is often useful for diagnosing type of CHD and in tachyarrhythmia. Biventricular hypertrophy (Fig. 7.4.6) with volume overload of the left ventricle is seen in large VSD, the most common cause of HF in infants. Tachycardiomyopathy, a potentially reversible cause of HF, due to incessant supraventricular tachycardia (like ectopic atrial tachycardia) can only be picked up by ECG. Similarly bradyarrhythmias due to congenital complete heart block are detected on ECG (Fig. 7.4.7). Anomalous coronary artery from pulmonary artery (ALCAPA) produces a very specific pattern, pathologic q waves in anterolateral leads (Fig. 7.4.8). Left axis deviation in a child with large shunt lesion suggests

vip.persianss.ir

D

Figure 7.4.5 Figure of 8 appearance in unobstructed total anomalous pulmonary venous connection (TAPVC)

•

chocardiogram

An echocardiogram is invaluable in the diagnosis of HF. It confirms the presence of structural heart disease and aids in the management strategy. Echocardiogram is an operator dependent test and to avoid wrong diagnosis, it should always be interpreted in an integrated fashion with clinical, radiographic and ECG findings.

• •

I

ther nvestigations B type natriuretic peptide (BNP), a cardiac natriuretic hormone, secreted in escalating fashion in ventricular dysfunction and progressive HF, is increasingly used in acute settings for differentiation of HF from pulmonary causes of respiratory distress. Hemoglobin is important as anemia can cause decompensation in cases with underlying heart disease but no HF. Electrolytes like serum calcium, phosphorus and blood glucose should be routinely measured in all children 453 -

O

 

 

 

E

AVSD as a cause of HF (Fig. 7.4.9). Rarely, hypocalcemia may cause LV dysfunction; ECG shows prolonged QTc interval with terminal T wave inversion (Fig. 7.4.10).

m

Figure 7.4.3 Egg-on-side appearance in transposition of great arteries (TGA)

 

Figure 7.4.4 Normal heart size with ground glass lungs in obstructed total anomalous pulmonary venous connection (TAPVC)

t

S

C

iseases of ardiovascular ys e

Figure 7.4.2 Features of significant left to right shunts: cardiomegaly with increased pulmonary blood flow (pulmonary plethora), prominent main and branch pulmonary arteries

vip.persianss.ir

t

t

Tex book of Pedia rics

 

Figure 7.4.6 Biventricular hypertrophy with volume overload of the left ventricle seen in large ventricular septal defect (VSD)

 

Figure 7.4.7 Electrocardiogram detecting bradyarrhythmias due to congenital complete heart block

•

-

-

•

with HF, especially neonates, where their abnormalities are an uncommon but reversible cause of ventricular dysfunction. Tests to screen for hypoxia and sepsis also constitute evaluation of HF in a newborn. Anti streptolysin O and C reactive protein are invaluable in work up for diagnosis of RF.

Management Key concepts in the management of HF in children are listed below: • Treatment of HF in children, like in adults, should consist of treatment of the cause, precipitating factors (like anemia, IE, infections, acute RF, noncompliance with drug or diet, arrhythmias, etc.) and treatment of the 454 congested state.

•

Digoxin has a very narrow safety window in children and adults alike. It should be avoided in premature babies, those with renal compromised state and cases with acute myocarditis. Electrolytes (K+, Ca++, Mg++) should be carefully monitored to avoid potentiation of toxicity and development of arrhythmias (which are more often bradyarrhythmias in children). Generally initial total digitalization is not performed. One can start directly to oral maintenance dose at 10 µg/kg/day (the available digoxin elixir has 50 µg/mL, hence the dose is 0.1 mL/ kg twice daily). Continuous infusion of diuretics is recommended in cases of acute decompensated HF. Monitoring and supplementation of K+ is necessary at higher doses, as deficiency is associated with increased arrhythmic death. During early infancy, supplementation of potassium is usually not required up to 2 mg/kg of dose

vip.persianss.ir

D m

t

S

C

iseases of ardiovascular ys e

 

Figure 7.4.8 Anomalous coronary artery from pulmonary artery (ALCAPA) producing a very specific pattern, pathologic q waves in anterolateral leads

 

Figure 7.4.9 Left axis deviation in a child with large shunt lesion suggesting atrioventricular septal defect (AVSD) as a cause of heart failure

455

vip.persianss.ir

t

t

Tex book of Pedia rics

 

Figure 7.4.10 Electrocardiogram showing prolonged QTc interval with terminal T wave inversion in hypocalcemia

Short term medical treatment is required for stabilization and alleviation of symptoms. This is a very common group because most of CHDs causing HF require surgical intervention. These children present with HF and frequently have comorbidities like sepsis or chest infection.

Children Requiring Long-Term Medical Therapy Several causes of HF in children require prolonged medical therapy. Examples include moderate sized VSDs, PDA, myocarditis/cardiomyopathy, etc. Therapy is also required for long term in residual defects following surgery and in valvular diseases where the risk benefit ratio is not in favor of surgery. -

•

Infants with CHD Awaiting Surgery

-

•

aorta, interruption of aortic arch, critical AS and TGA with intact septum and restrictive interatrial communication. These disorders require maintenance of duct patency with prostaglandin infusion till the time more definitive treatment can be employed. Because these children are very sick, they should be transferred to tertiary centers with expertise in their care after initial resuscitation and prostaglandin infusion (if required).

-

-

•

for furosemide or equivalent. In cases requiring higher doses and in older children, a combination of loop diuretic and spironolactone (or other potassium sparing diuretics) may be preferred. Angiotensin converting enzyme inhibitors should be avoided in HF caused by lesions having pressure overload physiology, e.g. in AS, as they might interfere with compensatory hypertrophy. The incidence of ACE inhibitor induced cough is much less in children as compared to adults. One should avoid combining ACE inhibitors with spironolactone as there is a risk of hyperkalemia. Beta blockers should not be administered in acute decompensated HF. They should be started after stabilization and in low dose initially; the dose is uptitrated gradually. In neonates and infants, active fluid restriction is not recommended. Calorie supplementation, either by increasing the density of milk or giving commercially available high calorie formulas, is recommended. In older children, fluid and salt restriction are generally required. Children should be asked to avoid extra salt as is present in fries, chips, pizzas and other similar food items. S

456 These may present with HF and circulatory collapse due -

to ductus dependent CHD, such as tight coarctation of

rugs for the Treatment of Heart ailure F

D

Neonates

Advances in medical science have ensured a wide variety of evidence based and emerging therapies for the -

F

Management of Heart ailure in ubgroups

vip.persianss.ir

C

D

onclusion

iseases of ardiovascular ys e

Heart failure in children is a complex syndrome with heterogeneous etiology and presentation. Unlike adults, HF

­

treatment or palliation of HF (Table 7.4.4). There are several newer agents, the roles of which are still investigational, in adults and in children. These include natriuretic peptides (e.g. nesiritide), calcium sensitizers (e.g. levosimendan), vasopressin antagonists (e.g. tolvaptan), renin inhibitors (e.g. aliskiren), endothelin antagonists (e.g. sitoxentan) etc. Some like oral phosphodiesterase inhibitors, anti inflammatory molecules, nitric oxide agonists and neuropeptidase antagonists have not proven useful or found to have excess side effects. Tables 7.4.5 to 7.4.7 outline the treatment of HF in children and dosages of common drugs used for the treatment in acute and chronic settings.

ardiac Transplantation

C

Avoid hypothermia and hypoglycemia; check for hypocalcemia Maintenance of adequate oxygenation

Monitoring of blood gases if perfusion is poor Ventilate, if required, with modest PEEP to achieve PaO2 of 50–60 mm Hg and SaO2 of 75–85% to avoid pulmonary congestion

Adequate hydration

Stop oral feeds if severe tachypnea

IV access

IV and CVP lines (umbilical vein cannula)

IV inotropes for shock

Isoproterenol 0.5–2 µg/kg/min Dopamine 5–20 µg/kg/min Dobutamine 5–20 µg/kg/min Avoid digoxin or use cautiously

Milrinone

Inotrope and vasodilator Load with 25–50 µg/kg/min; maintain at 0.25–1 µg/kg/min

Diuretics

Furosemide 2–4 µg/kg PO/IV

Vasodilators

Captopril 0.1–1 mg/kg/day PO q 8 hourly Sodium nitroprusside 0.5–4 µg/kg/min IV Careful monitoring of blood pressure is necessary

Prostaglandin infusion for ductus-dependent lesions

Start at 0.1 µg/kg/min (up to 0.4 µg/kg/ min if no response), taper to lowest dose possible (0.005 µg/kg/min) Monitor for apnea; keep minimum required dose

tem ell Therapy C

S

-

-

m

t

-

Heart transplantation has been used for the treatment of end stage heart disease in children for nearly 4 decades with first infant transplant done in late 1960s. Around 350 pediatric cardiac transplantations are done annually, representing about 10% of total cardiac transplantations. Majority of the transplantations are carried out for end stage heart disease due to cardiomyopathies. Other causes include CHDs like hypoplastic heart syndrome and other complex CHDs, single ventricle, palliated heart disease, etc. One year survival has approached 90%. However, given the fact that the surgery is done in few centers globally and the available donor hearts have remained static over last many years to few hundreds, it is clear that heart transplantation can be a solution for a minority only.

Supportive measures

S

C



-

 

Table 7.4.5 Treatment algorithm for acute heart failure in neonates

A heightened interest has developed in stem cell therapy for HF. Several trials have been completed, or are ongoing in adults with HF, predominantly due to ischemic heart disease. Stem cell therapy is being investigated worldwide under experimental settings for children with refractory HF.

Abbreviations: PEEP, Positive end-expiratory pressure; IV, Intravenous; CVP, Central venous pressure; PO, Per os

 

Table 7.4.6 Oral dosages of common drugs used to treat chronic heart failure  

Table 7.4.4 Treatment options for chronic heart failure Established

Investigational

Pharmacotherapy •  ACE inhibitors •  Beta blockers •  Digoxin •  Diuretics •  Aldosterone antagonists •  Anticoagulants (with severe ventricular dysfunction) Cardiac transplantation Surgery Definite (for structural disease) Ventricular assist devices Extracorporeal membrane oxygenation Intermittent inotrope infusion (weekend pulsed dobutamine)

Pharmacotherapy •  Angiotensin receptor blocker •  Nesiritide •  Levosimendan Ventricular remodeling Cardiac resynchronization therapy Implantable cardiac defibrillator Stem cell therapy

Digoxin

10 µg/kg/day (in two divided doses for children < 5 years)

Furosemide

1–4 mg/kg/day (1–2 doses)

Spironolactone

2–4 mg/kg/day (two doses)

Captopril

Neonates: 0.4–1.6 mg/kg/day in three divided doses Infants and children: 0.5–4 mg/kg/day in three divided doses

Enalapril

0.1–0.5 mg/kg/day (two doses); avoid in neonates

Losartan

0.5 mg/kg/day once daily

Metoprolol

0.1–0.2 mg/kg/dose (two doses) and increase to 1 mg/kg/dose or maximally tolerated dose over weeks or months

Carvedilol

0.05 mg/kg/dose (twice daily) and increase to 0.4–0.5 mg/kg/dose (twice daily) or maximally tolerated dose

457

vip.persianss.ir

-

-

-

-

-



-

-

in children is commonly due to structural heart disease and reversible conditions, thus lending it amenable to definitive therapy or short term aggressive therapy. Thus, the overall outcome with HF is better in children than that in adults. Clinical presentation of HF in younger children can be nonspecific requiring heightened degree of suspicion. In particular, some conditions that can present with acute

-

B

Abbreviations: ACE, Angiotensin converting enzyme; ARB, Angiotensin receptor blocker

-

Cardiac transplantation Ventricular assist device as bridge in case of delays or even ? destination



Step 6



Once or twice weekly dobutamine therapy Consider stem cell coronary infusion

1. Burch M. Heart failure in the young. Heart. 2002;88:198 202. 2. Gazit AZ, Oren PP. Pharmaceutical management of decompensated heart failure syndrome in children: current state of the art and a new approach. Curr Treat Options Cardiovasc Med. 2009;11:403 9. 3. Kay JD, Colan SD, Graham TP. Congestive heart failure in pediatric patients. Am Heart J. 2001;42:923 8. 4. Moffett E, Chang AC. Future pharmacologic agents for treatment of heart failure in children. Pediatr Cardiol. 2006;27:533 51. 5. Ramakrishnan S, Kothari SS, Bahl VK. Heart failure—definition and diagnosis. Indian Heart J. 2005;57:13 20. 6. Saxena A. ‘Working Group on Management of Congenital Heart Diseases in India’. Consensus on timing of intervention for common congenital heart disease. Indian Pediatr. 2008;45:117 26. 7. Shaddy RE, Boucek MM, Hsu DT, et al. Carvedilol for children and adolescents with heart failure: a randomized controlled trial. JAMA. 2007;298:1171 9.

Step 5



Add carvedilol in compensated HF, especially in cases with tachycardia



Step 4

ibliography



Add ACE inhibitor. In case of ACE inhibitor-induced cough, switch to ARB like losartan Switch to nitrates if ACE inhibitor/ARB is not tolerated



Step 3



Start digoxin (not in myocarditis) Assess reversible and precipitating causes Assess the need for surgery/interventional procedure in case of structural heart disease



Step 2



In acute decompensation: bed rest, propped-up position, humidified oxygen Sodium and, if required, volume restriction

shock are important to recognize, as they can be effectively treated or palliated on an urgent basis. While the general principles of management are similar to those in adults, there is a dearth of evidence base in pediatric HF. It would require a judicious balance of extrapolation from adult medicine (thus avoiding generation of redundant evidence) and development of children specific treatments (thus recognizing the inherent differences in HF of children and adults) to optimize the outcomes in this challenging field.



 

Step 1

t

t

Tex book of Pedia rics

Table 7.4.7 Stepwise guide to management of heart failure

458

vip.persianss.ir

pidemiology

E



Infective endocarditis is more common in adults as compared to the children, and accounts for 0.8–3.3 cases for each 1,000 admissions to hospital. The incidence in children and neonates may be rising due to increasing use of newer devices, indwelling catheters, increasing sophistication of resuscitative techniques especially in sick babies, and also due to overall improved survival. In India or in other developing countries, RHD still forms a significant proportion of cases with IE. In general, cardiac lesions with steep pressure gradients (VSD, PDA, left-sided valvular regurgitation) are associated with increased risk of IE. It is extremely rare in patients with ASDs. Tetralogy of Fallot bicuspid aortic valve and children who had undergone repair or palliation for cyanotic CHD are also at higher risk of IE. In newborn, endocarditis often involves right-sided structures and, the diagnosis may be difficult with a very high mortality rate.

tiopathogenesis

Gram-positive cocci are the most likely organisms, although gram-negative rods and fungi can cause IE. Streptococcus viridans is the most common causative agent followed by Staphylococcus aureus in children beyond the age of 1 year. This has been reported in Indian studies as well. However, S. aureus and fungus are more common in children where vascular indwelling catheter or prosthetic valve is used and in immunocompromised patients. The cardiac lesions consist of vegetations of fibrin, leukocytes, platelets and bacteria. Many clinical manifestations are related to destructive aspects of the infection or to embolization of portions of the vegetation. Endocarditis, particularly from Staphylococcus, may cause valvular damage, such as perforation of the aortic cusps or ruptured chordae tendinae of the mitral valve. Embolization may occur into the pulmonary or the systemic circulations and cause infarction, abscess or inflammation of various tissues. Emboli to the lungs, kidneys, spleen or brain are reported most frequently because in each location there are major clinical or laboratory findings of the phenomenon.

d

The clinical presentation is insidious with prolonged fever, malaise, myalgia, anorexia, weight loss, pallor, arthralgia and headache. The classical signs like Janeway lesions, Osler’s node and Roth spot are rarely seen in children. The diagnosis should be suspected in any child with a significant cardiac murmur and a prolonged fever. Congestive cardiac failure may develop, especially if aortic or mitral valve regurgitation is created by the infection. Signs and symptoms of embolic phenomenon should be sought. Signs of recurrent pneumonia or a pleuritic type of pain may indicate embolization of infected material to the lungs. Signs of systemic embolization, such as splenomegaly, hematuria, splinter hemorrhages and central nervous system signs, should be sought in any febrile patient with a cardiac anomaly and, rarely the children may present with these signs acutely.

Diagnosis The diagnosis can be confirmed by obtaining the organisms from a blood culture. Usually three blood cultures should be taken aseptically within the first 12 or 24 hours and 2–3 mL of blood may be sufficient. Blood culture may be negative in nearly 7% of cases, although studies from India have shown that the percentage is much higher, which may be due to prior use of antibiotics, and insufficient sample of blood for culture specially in our set up. Nonspecific acute-phase reactants, such as ESR, C-reactive protein and rheumatoid factor are usually elevated; the tests may also be useful in following the progress of therapy. Echocardiography has become an important modality for the diagnosis of IE and is taken as a major criteria in the modified Duke’s classification (Tables 7.5.1 and 7.5.2). But it is important to remember that diagnostic yield of echocardiography is influenced by the image quality, size of vegetation (vegetation less than 2–3 mm may not be well seen by transthoracic echocardiography), location of vegetation and the experience of echocardiographer. The absence of valve changes or vegetations does not exclude endocarditis. Transthoracic echocardiography (TTE) is very sensitive in children due to better acoustic window but occasionally transesophageal echocardiography (TEE) is advised in situation when window is poor, in patients with prosthetic valve, grafts or conduits, rarely in aortic valve endocarditis or aortic root abscess. M

E

Infective endocarditis is defined as the “endovascular microbial infection of cardiovascular structures,” involves infection of the endocardium or of the endothelium of the great vessels. This condition usually occurs as a complication of CHD or RHD, but rarely develops without pre-existing heart disease. It is associated with 16–25% mortality, and 20% patients require emergency surgery.



Clinical eatures F

nfective ndocarditis E

I

Srikanta Basu

c

yocardium and Pericardium

S

M

E





Diseases of ndocardium,

Diseases of Car iovas ular ystem

7.5

anagement

The aim of the management is to treat infection with antibiotics, manage complications and surgical intervention 459

vip.persianss.ir

d

 

c

extbook of Pe iatri s T

Table 7.5.1 Duke’s classification with addition of the modified Duke’s criteria Major criteria A. Positive blood culture for IE •  Typical microorganism consistent with IE from two separate blood cultures as noted below: –  Viridans streptococci, Streptococcus bovis or HACEK group or Staphylococcus aureus –  Community-acquired enterococci in the absence of a primary focus •  Microorganisms consistent with IE from persistently positive blood cultures defined as: –  Two positive cultures of blood samples drawn > 12 hours apart, or –  All of three or a majority of four separate cultures of blood (with first and last sample drawn 1 hour apart) –  Single positive blood culture for Coxiella burnetii or anti-phase 1 IgG antibody titer greater than 1:800 B. Evidence of endocardial involvement Echocardiogram positive for IE (TEE recommended for patients with prosthetic valves rated at least “possible IE” by clinical criteria, or complicated IE paravalvular abscess; TTE as first test in other patients) •  Positive echocardiogram for IE is defined as: –  Oscillating intracardiac mass on valve or supporting structures, in the path of regurgitant jets, or on implanted material in the absence of an alternative anatomic explanation, or –  Abscess, or –  New partial dehiscence of prosthetic valve •  New valvular regurgitation (worsening or changing of pre-existing murmur not sufficient) Minor criteria

p

g

m

•  Predisposition: predisposing heart condition or intravenous drug use •  Fever: temperature > 38.0°C (100.4°F) •  Vascular phenomena: ajor arterial emboli, septic pulmonary infarcts, mycotic aneurysm, intracranial hemorrhage, conjunctival hemorrhages and Janeway lesions •  Immunologic phenomena: lomerulonephritis, Osler’s nodes, Roth spots and rheumatoid factor •  Microbiological evidence: ositive blood culture but does not meet a major criterion as noted above¹ or serological evidence of active infection with organism consistent with IE •  Echocardiographic minor criteria eliminated ¹Excludes single positive cultures for coagulase-negative staphylococci and organisms that do not cause endocarditis Abbreviations: IE, Infective endocarditis; HACEK, Haemophilus species, Actinobacilius actinomycetemcomitans, Cardiobacterium hominis, Eikenella corrodens, Kingella species; TEE, Transesophageal echocardiography; TTE, Transthoracic echocardiography

in selected cases. If the patient is very ill, antibiotic treatment can be initiated empirically after the cultures are taken. However, in all the other cases, initiation of therapy should await the results of blood cultures. Exact treatment depends upon the organism isolated and its antibiotic sensitivities. Usually, penicillin (or vancomycin if sensitive to penicillin) and gentamicin are the preferred antibiotics that are administered parenterally. The total duration of therapy is for 6 weeks. Following completion of therapy, blood cultures should be obtained to verify eradication of the infection. American Heart Association has listed various regimens for IE in adults and the same can be followed in children with dose modification. Fungal endocarditis may be difficult to treat and may require surgery along with antifungals like amphotericin B for a duration of 6–8 weeks followed by oral antifungals like fluconazole for prolonged duration of time (may take up to a year). The indications of surgery in the acute phase have been listed in Table 7.5.3. The aims of the surgery are to repair the cardiac defects, eradicate infection and to prevent complications. Despite the availability of antimicrobials, endocarditis can lead to major complications (Table 7.5.4). Complications are more 460 likely to occur in patients with prosthetic valve, age less than

2 years, left-sided IE, fungal and staphylococcal IE, symptom continuing for more than 3 months and cyanotic CHDs.

Prophylaxis Antibiotics are recommended for the prevention of IE prior to certain medical and dental procedures. The guidelines by the American Heart Association have undergone changes in 2007. The cardiac conditions for which prophylaxis is recommended have been listed in Table 7.5.5. Presently it is recommended for all dental procedures that involve treatment of gingival tissue or periapical region of the teeth or oral mucosal perforation, for invasive procedures that involve incision or biopsy of respiratory mucosa, such as tonsillectomy and adenoidectomy. It is no longer recommended for genitourinary or gastrointestinal tract procedures solely for bacterial endocarditis prophylaxis. The application of these guidelines in Indian context is debated by various pediatric cardiologists. The drugs recommended are oral amoxicillin (50 mg/Kg), intravenous ampicillin, and those allergic to penicillin can take cefazolin, ceftriaxone, clindamycin, cephalexin, azithromycin or clarithromycin. The antibiotic should be given before the procedure and up to 2 hours after the procedure in those who did not take it earlier.

vip.persianss.ir

 

•  •  •  •  •  •  •  • 

Pathologic criteria Microorganism demonstrated by culture or histologic examination of vegetation, emboli, intracardiac abscess; or Active endocardial lesions on pathology examination Clinical criteria Two major criteria, or one major criterion and three minor criteria, or five minor criteria Possible IE One major criterion and one minor criterion, or three minor criteria

Embolic events: cerebral, pulmonary, renal, coronary Congestive heart failure Persistent bacteremia or fungemia Periannular extension of abscess Metastatic infection Glomerulonephritis/renal failure Mycotic aneurysm Arrhythmia or development of new heart block

d

 

Table 7.5.4 Complications of infective endocarditis (IE)

Definite IE

c

 

Table 7.5.5 Cardiac conditions for which antibiotic prophylaxis is recommended for dental, respiratory tract, infected skin, skin structures or musculoskeletal tissue procedures

Rejected •  Firm alternative diagnosis explaining evidence of IE; or •  “IE syndrome” resolved within 4 days of antibiotic therapy; or •  No pathological evidence of IE at surgery or autopsy within 4 days of antibiotic therapy; or •  Does not meet criteria for “possible IE” as above

 

Myocardial or periannular abscess Continued bacteremia after 2 weeks of antibiotic therapy Worsening of heart failure due to valvular regurgitation Embolic events Fungal endocarditis Prosthetic valve dysfunction

S

•  Prosthetic cardiac valve or prosthetic material used for cardiac valve repair •  Previous IE •  Unrepaired cyanotic CHD, including palliative shunts and conduits •  Completely repaired congenital heart defect with prosthetic material or device, whether placed by surgery or by catheter intervention during the first 6 months after the procedure •  Repaired CHD with residual defects at the site or adjacent to the site of a prosthetic patch or prosthetic device (which inhibits endothelialization) •  Cardiac transplantation recipients who develop cardiac valvulopathy

Table 7.5.3 Indications for surgery in acute phase of infective endocarditis (IE)

Table 7.5.6 Etiology of myocardial damage  

•  •  •  •  •  • 

Diseases of Car iovas ular ystem

Table 7.5.2 Diagnosis of infective endocarditis (IE) according to the modified Duke’s criteria

Cardiomyopathy

­

Cardiomyopathy refers to a group of diseases of heart muscle that is not secondary to structural heart disease, hypertension or pulmonary vascular disease. These are categorized by WHO as dilated, hypertrophic, restrictive and arrhythmogenic right ventricular dysplasia—cardio myopathy. Factors identified as causes of myocardial damage have been shown in Table 7.5.6.

Dilated Cardiomyopathy Dilated cardiomyopathy is the most common type of heart muscle disease in children where both the ventricles are dilated with reduced contractility. The majority of cases of DCM are idiopathic.

Etiopathogenesis Three major factors, viz. preceding viral myocarditis, autoimmunity and underlying genetic predisposition have been implicated in the pathogenesis of myocardial damage in DCM. Injury to the myocardial cells is the initiating factor that leads to cell death. If considerable cell loss occurs, the myocardium fails to generate enough contractile force to produce adequate cardiac output and leads to

Infections

Metabolic, Endocrinal, Nutritional

Viral infections (myocarditis): Coxsackievirus B, human immunodeficiency virus, echovirus, rubella, varicella, mumps, EpsteinBarr virus, cytomegalovirus, measles, polio Others: Diphtheria, Mycoplasma, tuberculosis, Lyme disease, Psittacosis, Rocky Mountain spotted fever, Toxoplasma, Toxocara, Cysticercus, Histoplasma, coccidioidomycosis, Actinomyces

Pompe, Carnitine deficiency syndrome, hyperthyroidism, carcinoid, infant of diabetic mother, kwashiorkor, pellagra, thiamine deficiency, selenium deficiency

Familial—Hereditary

Coronary arteries

Mitochondrial myopathy syndrome, hypertrophic cardiomyopathy, Duchenne or Becker muscular dystrophies, Friedreich’s ataxia, Kearns-Sayre syndrome, other muscular dystrophies

Kawasaki disease, anomalous left coronary artery

Connective tissue— Granulomatous—Infiltrative Systemic lupus erythematosus, scleroderma, rheumatoid arthritis, sarcoidosis, amyloidosis, dermatomyositis, leukemia

Other Anemia, sickle cell disease, endomyocardial fibroelastosis, histiocytosis

Drugs—Toxins Doxorubicin, cyclophosphamide, chloroquine hemosiderosis

461

vip.persianss.ir

Children with DCM usually present typically after HF. Cough, poor feeding, irritability and difficulty in breathing are usually the initial presenting symptoms. Older children complain of easy fatigability, dyspnea and palpitation, and rarely may present with pulmonary edema. Clinical examination may reveal displaced apical impulse, muffled heart sound with a prominent S3 or S4. Murmurs are usually not audible but rarely the murmur of MR or TR may be heard. The liver may be enlarged and in older children, other signs of venous congestion like neck vein congestion and peripheral edema may be observed. A short duration of symptoms (2–4 weeks) may suggest myocarditis. The infant or young child with the disease is often tachypneic, tachycardic with weak peripheral pulses, and has cool extremities and hepatomegaly. Blood pressure is low with a decreased pulse pressure, accentuated P2, murmurs of mitral and tricuspid regurgitation; murmurs may be inconspicuous initially if the patient presents with acute HF and cardiomegaly.

mmunoglobulins: Intravenous immunoglobulin (IVIg) has been used as an immunomodulator, which neutralizes pathogenic antibodies and suppress the ongoing process. Pediatric Cardiac Society of India in a consensus review (2009) has recommended IVIg in cases where symptoms are preceded by viral illness, or when history is short (< 3 months) or cardiac enzymes are elevated. • urgical procedures: Reduction cardiomyoplasty or Batista operation, and assist device and artificial heart are some procedures, which are often used as option to “bridge to transplantation procedure” and are very expensive. Cardiac transplantation is the final treatment for DCM with severe LV dysfunction and offers a 5-year survival benefit of 50–70%. I

•

Prognosis In a small proportion of pediatric patients, DCM may resolve completely. In few pediatric case series, the mortality from DCM at 5 years after presentation varied from 33% to 66%. In a large case series from AIIMS on 128 children, it was reported that 54% improved on medication and of these 24% became symptom free without medication. Thirty-five percent deteriorated despite therapy and 19% died. Female sex was only multivariate predictor of death. Overall the prognosis of DCM will improve only if various etiologies are understood and etiology-specific therapy is instituted. Table 7.5.7 List of investigations in DCM  

Clinical Features

not possible and many a times, various treatment modalities (immunoglobulins or carnitine) are administered without good evidence. The treatment modalities have been enlisted in Table 7.5.8.

S



Findings and comments

Chest X-ray

Cardiomegaly and features of pulmonary venous congestion

Electrocardiogram

Low voltage waves, S-T segment depres sions with T wave inversion in the anterolateral precordial leads, prominent Q wave, changes suggestive of arrhythmias

Echocardiogram

Ejection and shortening fraction, quanti fication of MR, clots, thrombus and coronaries; rule out any structural heart lesion

creening of family members: With the advancement of molecular genetic techniques, prospective controlled studies on screening of family members have revealed a genetic transmission up to 20–30% cases with DCM and a prevalent autosomal trait. Due to limited awareness about the genetic predisposition, screening and echo of family members are not performed, but it is advisable in view of above findings.

Basic laboratory tests

Hemogram, ESR, glucose, urea, creatinine, LFT, electrolytes, calcium, phosphorous, ABGs

Metabolic screening

Blood lactate, serum ammonia, urinary ketones

Muscle enzymes

CPK, CPK-MB, cardiac troponin T

Treatment

Endomyocardial biopsy and viral culture

S

The main aim for diagnostic evaluation for a patient with DCM is to identify the underlying etiology so that an etiologyspecific therapy can be planned. An additional purpose is to determine the extent of myocardial dysfunction and its complication like clot formation in the heart. Table 7.5.7 provides a list of useful diagnostic tests.

Treatment involves supportive management of CHF 462 (discussed in detail in the chapter on congestive cardiac failure). Ideally, etiology-specific treatment is recommended, but it requires clear proof of association of etiology, which is

­

Diagnosis

­

Investigations

Screening and echocar Screening of first degree relatives of the case diogram of family members ­

d

c

extbook of Pe iatri s T

CHF. Overstretching of the ventricles causes myocardial thinning, cavity dilatation, secondary valvular regurgitation and compromised myocardial perfusion. The resulting subendocardial ischemia perpetuates myocyte damage. Myocardial remodeling is an important contributor to worsening HF. Lost myocyte cells are replaced with fibrous tissue, thereby decreasing the compliance of one or more ventricles and adversely affecting performance. Aldosterone, angiotensin II, catecholamines, endothelins and mechanical factors, such as excessive myocardial stretch and ischemia, have been identified as mediators of remodeling. Altered gene expressions resulting in calcium-handling abnormalities, down regulation of myosin or conversion to the less active beta isoform, and abnormal beta-receptor signal transduction have all been identified at the molecular level in the chronically failing heart.

Not advised routinely and not very specific for identifying etiology

Abbreviations: DCM, Dilated cardiomyopathy; MR, Mitral regurgitation; ESR, Erythrocyte sedimentation rate; LFT, Liver function test; ABGs, Arterial blood gases; CPK, Creatine phosphokinase

vip.persianss.ir

Diseases of Car iovas ular ystem

 

Table 7.5.8 Treatment options for DCM Treatment options

Comments

Digoxin

Can be given in myocarditis but loading dose is to be avoided

Furosemide + spironolactone

Helps in reducing systemic and pulmonary venous congestion

Beta blockers and carvedolol

Carvedolol is the most widely used and improves symptoms and LV failure. It should be considered in the absence of overt heart failure, particularly in the presence of tachycardia

Anticoagulation and aspirin

All children with DCM and severe LV dysfunction (EF < 30%) should ideally be anticoagulated and in the absence of monitoring INR, aspirin can be tried. Presence of LV thrombus is a strong indication for oral anticoagulation.

Carnitine

If metabolic screening suggest carnitine deficiency (elevated ammonia levels, high blood lactate or presence of urinary ketones), carnitine can be given.

c

Benefits by reducing systemic vascular resistance. Initiate treatment with captopril and can be changed to enalapril.

S

ACE inhibitors (Captopril, enalapril)

d

Intravenous inotropes and vasodilators Dobutamine is the first choice in the stage of decompensation (dobutamine, dopamine, amrinone, milrinone)

Abbreviations: DCM, Dilated cardiomyopathy; ACE, Angiotensin converting enzyme; LV, Left ventricular; EF, Ejection fraction; INR, International normalized ratio

Hypertrophic Cardiomyopathy

Investigations

Hypertrophic cardiomyopathy is a condition in which myocardial thickness is increased. In contrast to DCM, the LV cavity has a normal or decreased size. During systole, the hypertrophied myocardium bulges into the LV outflow tract and may result in subaortic obstruction.

Chest X-ray is not diagnostic and ECG shows evidence of left and frequently biventricular hypertrophy with or without ischemic changes. Septal changes are evidenced by abnormal q waves in V5 and V6. The echocardiogram may reveal asymmetric septal hypertrophy. The interventricular septum may be 2–3 cm thick compared with the normal, which is less than or equal to 1 cm. Systolic anterior motion of the mitral valve anterior leaflet is a classic twodimensional echocardiographic finding. It results from the high velocity flow occurring in the LV outflow tract. This creates low pressure, which “pulls” the valve leaflet toward the interventricular septum during systole. Cardiac catheterization and angiography is rarely required.



Patients with HCM usually come to medical attention in one or more of the following ways: • Detection of a murmur • An anginal kind of chest pain • An abnormal ECG • From family screening or a positive family history, or • From evaluation of syncope, palpitations, or out of hospital cardiac arrest. The physical findings can be variable. The peripheral pulse may be of bisferiens character with rapid upstroke, and palpation of the apex may reveal a double impulse. A long systolic ejection murmur is present along the left sternal border and faintly radiates to the base. The murmur varies in intensity with change in position; it is usually loudest with the patient standing in contrast to functional flow murmurs. Third and fourth sounds may be present.

The greatest risk with HCM is sudden death in spite of medical and surgical treatment. For symptomatic patients, beta blockers or verapamil can be tried, and for the control of arrhythmias, disopyramide and amiodarone are the drugs of choice. Digitalis, other inotropic drugs and nitrates, which increase the gradient, are contraindicated. It is necessary that patients with HCM should undergo a 24-hour holter to document for arrhythmias and should be restrained from strenuous games and exercises. Implantable automatic cardioverter/defibrillator (AICD) devices may abort poten tially lethal arrhythmia in some patients. For patients with significant LV outflow tract obstru ction, surgical myomectomy or alcohol ablation can be done. There is increasing evidence that elimination of the obstruction prolongs life and relieves symptoms. ­

Clinical Features

Treatment

­

The disease may be caused by one of many possible mutations of genes coding for various contractile proteins. This condition frequently occurs as an autosomal dominant with a variable penetrance. Multiple generations may be involved. Manifestations can begin at any age. Siblings of the proband may not be affected as children but may show evidence of the disease as they reach adolescence and adulthood. The natural history and prognosis are variable; sudden death is not uncommon even in patients who have no important obstruction or sentinel arrhythmia.



Etiology and Genetics

Prognosis Although ultimately fatal in majority, interval between discovery to death often goes in decades. The risk of sudden 463 death in recent population-based studies has shown to be nearly 1% per year. Following features may be associated with

vip.persianss.ir

­



Pericardial Disease Pericarditis is the inflammation of the visceral and parietal layer of pericardium. The causes are variable and are listed in Table 7.5.10. Pericardial effusion is the accumulation of excess fluid in pericardial sac. The symptoms that result from pericardial fluid depend upon the status of the myocardium and the volume and the speed at which the fluid accumulates. It may be exudative due to bacterial infections, serous to increased capillary pressures (e.g. CHF) or decreased plasma oncotic pressure (e.g. hypoproteinemia). Cardiac tamponade is a life threatening, slow or rapid compression of the heart due to accumulation of fluid, blood, clots or gas in the pericardial space leading to significant impairment of ventricular filling with reduction in stroke volume and cardiac output. It is most commonly associated with viral infections, neoplasm, uremia and acute hemorrhage. Constrictive pericarditis is due to adherent thick non-compliant pericardium that restricts ventricular filling. In children, tuberculosis is the most common cause in India. The clinical manifestations of pericarditis which may vary from a simple inflammatory response with no cardiovascular compromise to cardiac tamponade and constrictive pericarditis are noted in Table 7.5.11. The differentiation of the constrictive pericarditis and restrictive myocarditis can be challenging. The management of these conditions will depend on the cause and are listed in Table 7.5.11.



A comparison of the three variants of cardiomyopathy has been summarized in Table 7.5.9.



R

estrictive Cardiomyopathy This is the rarest form of cardiomyopathy characterized by poor ventricular compliance and limited filling. It may be idiopathic or may be associated with a systemic disease such as scleroderma, amyloidosis, sarcoidosis or inborn error of metabolism (mucopolysaccharidoses). The two common types are endocardial fibroelastosis (EFE) with normal or less than normal left ventricle and endomyocardial fibrosis (EMF). EMF is endemic in Kerela, but rare in North India. Symptoms are nonspecific and similar to those of CHF seen with DCM. In contrast to DCM, the left ventricle is of normal size and may have normal systolic function. This condition alters diastolic ventricular function, so the clinical manifestations are those of elevated left and right atrial pressures. Examination reveals hepatic and splenic enlargement and jugular venous distension. Electrocardiographic abnormalities are usually limited to atrial enlargement. Chest X-ray shows pulmonary vascular congestion with a relatively normal cardiac silhouette. The echocardiogram reveals striking dilatation of the atria and great veins but normal or small ventricles. Physiologically, the condition is similar to constrictive pericarditis and differentiating the two can be difficult. Overall the prognosis is poor in infants and children and cardiac transplantation is advised once the diagnosis is made.

d

c

extbook of Pe iatri s T

higher risk for sudden death: diagnosis in childhood, septal thickness exceeding 3 cm, nonsustained VT, failure of normal increase of systolic blood pressure during exercise, family history of premature death associated with HCM, significant LV outflow tract obstruction, and prior cardiac arrest.

 

Table 7.5.9 Overview of myopathies

464

Dilated

Hypertrophic

Restrictive

Prevalence

50/100,000

1/500

Unknown

Inheritance

25–50% AD, AR, X-L, Mt

50% AD

Unknown

Ventricular function

Systolic and diastolic dysfunction

Diastolic dysfunction

Severely reduced diastolic function

Arrhythmias

Atrial, ventricular and conduction disturbances

Atrial and ventricular Dynamic systolic outflow obstruction

Atrial fibrillation Normal to reduced systolic function

Echocardiographic findings

Dilated LV cavity with normal to thin wall thickness

Asymmetric, concentric or apical LV hypertrophy

Normal or small ventricular cavity size, marked biatrial enlargement

Medical management

ACE inhibitors Diuretics (furosemide, spironolactone) Digitalis β2-blocking agents

Propranolol Pacemaker Calcium antagonists (digitalis/catechols and nitrates contraindicated and diuretics may worsen symptoms)

Antiarrhythmic agents Careful use of diuretics, milrinone

Surgical/interventional

ICD, resynchronization biventricular pacing Transplantation

ICD, septal myomectomy Transplantation

ICD Transplantation

Sudden death

Yes

Depends on gene 0.7–11% per year, associated with exercise

1.5% per year

Abbreviations: AD, Autosomal dominant; AR, Autosomal recessive; X-L, X-linked; Mt, Mitochondrial; LV, Left ventricular; ACE, Angiotensin converting enzyme; ICD, Implantable cardioverter defibrillator

vip.persianss.ir

Diseases of Car iovas ular ystem

 

Table 7.5.10 Causes of pericardial diseases

c

d

Hematology-Oncology: •  Bleeding diathesis •  Malignancy (primary, metastatic) •  Radiotherapy-induced Post procedural: •  Post cardiac Catheterization •  Cardiac surgery •  Central line placement Drugs: •  Anticoagulants, antithrombolytics •  Hydralazine •  Phenytoin •  INH •  Rifampicin Other: •  Renal failure •  Hypothyroidism •  Chylopericardium

S





Congenital: •  Absence of pericardium •  Pericardial cyst Infectious: •  Viral (coxsackievirus A and B, Epstein-Barr virus, hepatitis, HIV, adenovirus) •  Bacterial (Staphylococcus, H influenzae type B, Streptococcus, Pneumococcus) •  Tuberculosis •  Fungal (Histoplasmosis, Actinomycosis) •  Parasitic (Toxoplasmosis, Echinococcosis) Connective Tissue Diseases: •  Rheumatic fever •  Rheumatoid arthritis •  Systemic lupus erythematosus •  Systemic sclerosis •  Kawasaki disease •  Wegener granulomatosis

 

Table 7.5.11 Manifestations and management of pericarditis Non-constrictive pericarditis

Pericardial effusion

Cardiac tamponade

Constrictive pericarditis

Symptoms

Retrosternal chest pain (occasionally radiating to back, aggravated by supine position, relieved by leaning forward, dyspnea

May be asymptomatic, dull ache, and other features of cardiac tamponade

Dyspnea, fatigue, anorexia, cough, cold extremities

Exercise intolerance, syncope with exertion, dyspnea, fatigue, anorexia

Signs

Fever, tachypnea, pericardial rub, distant heart sound

Muffled heart sound, Ewart sign (dullness of posterior left chest wall); tachycardia

Raised JVP, hypotension, tachycardia, pulsus paradoxus (decrease in systolic blood pressure > 10 mm Hg with each inspiration) decreased capillary refill time, silent precordium, distant heart sound, hepatomegaly

Tachycardia, pedal edema, raised JVP, hepatomegaly, ascitis, pericardial knock and signs of low cardiac output

EKG

S-T segment elevation in all leads; PR segment depression

Decreased voltage QRS complex, electrical alternans

Electrical alternans sinus tachycardia, low voltage waves, electrical alternans

Low voltage waves, intraventricular conduction delay

Chest X-ray

Normal

Globular, symmetrical cardiomegaly

Globular, symmetrical cardiomegaly

Normal, pericardial calcification or effusion

Echocardiography

Usually normal

An echo-free space (suggesting fluid) around the heart

Global fluid collection, compressed and collapsing chambers with hyperdynamic cardiac function

Flattening of the left ventricular posterior wall endocardium, abnormal septal motion, dilated SVC, IVC, atria

Treatment

Treat the cause; symptomatic treatment with rest, analgesic and rarely antibiotics

Treat the underlying condition. Pericardiocentesis if sudden accumulation of fluid, NSAIDs or steroids are required occasionally

Needle pericardiocentesis or surgical drainage, pericardial stripping or window in recurrent condition

Analgesic, corticosteroids and antibiotics. Pericardiectomy is definitive treatment but mostly unwarranted as many cases including TB resolve spontaneously

465

vip.persianss.ir











4. Towbin JA, Lowe LM, Colan SD, et al. Incidence, causes, and outcomes of dilated cardiomyopathy in children. JAMA. 2006;296:1867-76. 5. Wilson W, et al. Prevention of Infective Endocarditis Guidelines From the American Heart Association. A Guideline from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. Circulation. 2007;116:1745-54. 6. Working group on management of congenital heart diseases in India. Drug Therapy of Cardiac Diseases in Children. Indian Pediatr. 2009;46:310-38.



1. Baddour LM, Wilson WR, Bayer AS, et al. Infective endocarditis. Diagnosis, antimicrobial therapy, and management of complications. A statement for healthcare professionals from the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, and the Councils on Clinical Cardiology, Stroke, and Cardiovascular Surgery and Anesthesia, American Heart Association. Circulation. 2005;111:e394-433. 2. Kumar RK, Prabhu SS, Ahamed Z. IAP Specialty Textbook of Pediatric Cardiology. New Delhi, India: Jaypee Brothers; 2008. 3. Saxena A. ‘Working Group on Management of Congenital Heart Diseases in India’. Consensus on timing of intervention for common congenital heart disease. Indian Pediatr. 2008;45:117-26.

B



d

c

extbook of Pe iatri s T

ibliography

466

vip.persianss.ir

T

inus Bradycardia and inus achycardia

Treatment Drug treatment is not essential as simple vagal maneuvers like carotid sinus massage can effectively terminate SVT in majority of cases. Adenosine with its short half-life is the most preferred drug and verapamil is the next drug of choice. Verapamil is not to be given in children below 1 year of age. Beta blockers, disopyramide and digitalis are less preferred alternatives, and digoxin is not helpful in acute case. Prevention of AV nodal re-entry is achieved by the use of digitalis, beta blockers or calcium channel blockers which act primarily on the antegrade slow pathway. In an emergency, DC cardioversion terminates the attack. Temporary percutaneous venous, atrial or ventricular pacing terminates the attack when drugs fail to do so or in recurrent cases. Catheter radiofrequency ablation of the re-entrant 467 circuit is frequently being resorted to in refractory cases.

achyarrhythmias

Supraventricular Tachyarrhythmia (SVT, Paroxysmal Atrial Tachycardia, AV Nodal Re-entrant Tachycardia). Supraventricular tachyarrhythmia is the most common of the rapid rhythm abnormalities encountered in children, the greatest frequency being recorded in infancy. It is known to occur in fetus as early as in middle fetal life.



T







Sinus bradycardia (heart rate less than 60/min) occurs in older children who are athletic. It also occurs following therapy with drugs, such as beta blockers, digitalis, verapamil; and conditions like hypothyroidism, hypothermia and raised intracranial pressure, etc. Sinus tachycardia (heart rate > 120/min) occurs with sympathetic stimulation after exercise, fever, anxiety, anemia, HF, pregnancy, thyrotoxicosis and administration of drugs which stimulate sympathetics. Sinus arrhythmia is a manifestation of normal automatic nervous activity with increase in sinus heart rate during inspiration and a decrease during expiration. This is seen in young children.



d







The mechanism of supraventricular tachycardia is commonly a re-entry of the electrical impulse back into the conducting system either through an accessory pathway (Wolff-Parkinson-White syndrome) or within the AV node. It may originate in a localized focus of enhanced automaticity in the atria or AV junction. In the absence of antegrade conduction by the bypass accessory pathway, re-entry through the AV node or through a concealed bypass tract is responsible for more than 90% of all SVTs. Atrioventricular nodal re-entrant tachycardia, being the most common of all SVTs, has no specific predisposing factors. It most often occurs in children who are otherwise normal. In infancy, it is more common in children below 6 months of age. The heart rate is regular and often ranges between 200/min and 320/min and all impulses are conducted to the ventricles. These children are often asymptomatic. In infants, if it persists for more than 24 hours, symptoms and signs of CHF occur besides pallor, fatigue and tachypnea. The onset of the paroxysm is as sudden and abrupt as its termination. An older child may sense palpitation with sudden onset. Syncope and hypotension may be the presenting symptoms. Acute pulmonary edema can ensue. In the fetus, when arrhythmia is prolonged, heart failure and nonimmune hydrops fetalis are known to occur. Polyuria may occur due to release of atrial natriuretic peptide during such paroxysms. Electrocardiogram shows marked tachycardia (> 200/ min) with regular narrow QRS complexes and the P waves may be absent, buried in the QRS complex or appear as distortions at the terminal part of the QRS complexes. QRS complexes become wider in presence of associated bundle branch block.

R

inus hythms S

S

S









The sinoatrial (SA) node located at the junction of superior vena cava and right atrium acts as an electrical impulse generator or pacemaker with its own intrinsic rate, controlled by the autonomic nervous system. The impulse from SA node passes through AV node and Bundle of His to depolarize the atria and ventricles in sequence determined by the slower conductivity of AV node. If the sinus rate slows down considerably, shift in pacemaker activity occurs and the AV node (nodal rhythm) or the ventricle (idioventricular rhythm) takes over the function of impulse generation and propagation. An arrhythmia is a disturbance in the electrical activity of the heart, which may be episodic or continuous. When heart rate slows down to less than 60/min, it is termed as bradycardia and when the same is more than 120/min, it is called tachycardia. On the standard surface ECG, P wave corresponds to SA node depolarization, the P-R segment to the conduction time in the AV node and the purkinje system, the QRS complex to the ventricular depolarization and the T wave to the ventricular repolarization. The two basic mechanisms that initiate tachycardia are: (1) increased automaticity resulting in ectopic impulse formation and (2) re-entry loop or closed circuit propagation of ectopic impulse generated.

c

S Srinivasan

S

A





Cardiac rrhythmias in Children

Diseases of Car iovas ular ystem

7.6

vip.persianss.ir

S

F

trial lutter

In atrial flutter, the characteristic feature is a very rapid atrial activity (250–400 beats/min) with the ventricles responding to every second to fourth atrial beat resulting in a regular or regularly irregular tachycardia.

S

­



Congenital heart diseases resulting in a grossly enlarged atria, e.g. mitral or tricuspid insufficiency, tricuspid atresia, Ebstein’s anomaly, acquired rheumatic mitral valvular heart disease, acute viral myocarditis, pericarditis and intra-atrial surgery.

Pathophysiology An electrically active (irritable), abnormal focus in atria produces abnormal impulse, which gets repeatedly propagated by a circus rhythm resulting in extremely rapid atrial rate. All of these rapid atrial beats cannot get transmitted through AV node. Varying degrees of AV block results in, anywhere from 2:1 to 8:1 atrial ventricular rate ratios.

Symptoms Symptoms depend upon the ventricular rate. No symptoms occur in atrial flutter with reasonable ventricular rate. Prolonged episodes of atrial flutter with very rapid ventri cular rate precipitate CHF. Electrocardiogram is characteristic showing the rapid and regular atrial saw-toothed flutter “F” waves.

Treatment Direct current cardioversion is the most effective method of reverting back to sinus rhythm. If the clinical status is stable, the ventricular rate is first slowed by administration of AV node blocking drugs like beta blockers, calcium channel antagonist (verapamil) or digitalis. Once the ventricular rate is slowed, attempt is made to convert the flutter into normal sinus rhythm by the use of Class 1A drugs (quinidine, procainamide or disopyramide), Class 1C drugs (flecainide) or amiodarone. The same drugs also prevent recurrences of atrial flutter and fibrillation.

E

trial ctopic Beats/ xtrasystoles

F

A

trial lutter and trial ibrillation F

A

Premature atrial beats are recognized by abnormally shaped premature “P” waves followed by near normal P-R interval with normal QRS complex. Rarely, QRS complex may be aberrant. They may occur in normal newborns and disappear with age and do not cause symptoms. Multiple atrial premature beats may sometimes result in transient atrial fibrillation. These two types of atrial arrhythmias are less common in

468 children than in adults.

F

trial ibrillation

Atrial fibrillation is characterized by totally distorted, chaotic, rapid and ineffective atrial contractions with irregular and erratic ventricular response resulting in the diagnostic “irregularly irregular” radial arterial pulse with pulse deficit. It may be paroxysmal or persistent. The same causes, mentioned above in atrial flutter, may also result in atrial fibrillation. It may represent the tachycardiac phase of the sick sinus syndrome.

T

trial achyarrhythmias E

A

A

A





Causes

­

An abnormal band of specialized electrically conductible atrial tissue acts as an accessory pathway bypassing the junctional tissue. This occurs in association with some congenital heart diseases and most commonly with Ebstein’s anomaly. Wolff-Parkinson-White syndrome refers to antegrade conduction by AV bypass tract resulting in a short P-R interval, a slurred upstroke of the QRS complex termed delta wave and a wide QRS complex. Differential conducting speeds of accessory and normal pathways with differing refractory periods cause re-entry phenomenon to occur resulting in paroxysmal SVT. Atrial flutter and atrial fibrillation also occur commonly in this condition. This in turn, may lead to very rapid ventricular rates, even resulting in VT and ventricular fibrillation (VF) because of the lack of decremental conducting properties in the bypass tract as in AV node. Supraventricular tachyarrhythmia is treated as men tioned earlier. Class 1A drug (quinidine, procainamide, disopyramide) or Class 1C (flecainide) may be used to slow conduction and increase refractoriness primarily in the bypass tract. Flecainide is to be limited to be used in otherwise normal heart. Digitalis and verapamil can precipitate VF by shortening the refractory period of the bypass tract. Radiofrequency ablation of an accessory pathway is another treatment option commonly used in patients with re-entrant rhythm or atrial ectopic tachycardia. It is often used electively in children and teenagers, as well as in patients who require multiple agents or find drug side effects intolerable or for whom arrhythmia control is poor. The overall initial success rate ranges approximately from 80%–95%, depending on the location of bypass tract. Surgical ablation of bypass tract may also be successful in related patients. Flecainide is to be limited to be used in otherwise normal heart.



E

A



Wolff-Parkinson-White yndrome (Pre- xcitation yndrome)



b

d

c

ext ook of Pe iatri s T

In AV re-entrant tachycardia, retrograde conduction occurs from the ventricles back to the atrium by a concealed bypass tract.

Symptoms Symptoms occur with rapid ventricular rate—fatigue, palpitation, giddiness or syncope, symptoms of heart failure and symptoms of systemic embolization in children with mitral valvular disease.

vip.persianss.ir

entricular achyarrhythmias

d S

c



F

entricular ibrillation

Ventricular fibrillation is a chaotic ventricular tachyarrhythmia with no effective ventricular contraction. The ECG record shows a wavy line without any discernible QRS complexes; P waves may be discernible. Ventricular fibrillation may be a preterminal event in many illnesses. Hypokalemia, digitalis or quinidine toxicity, myocardial inflammation or damage, catecholamines, aminophylline, anesthetic drugs and plant toxins may precipitate VF. Uncontrolled VT or multifocal ventricular ectopic beats or long Q-T interval syndrome may result in VF. If not terminated instantaneously, death ensues. A thump in the chest may restore sinus rhythm. External cardiac massage with artificial ventilation and DC defibrillation are immediate measures to be undertaken. The precipitating factors are corrected without delay. After defibrillatory conversion, VT is treated with drugs. Refractory cases are treated by implantable automatic cardioverterdefibrillator.

Bradyarrhythmias inus rrest and inoatrial Block

Failure of impulse formation within the sinus node is termed as sinus arrest, and blockade of the generated sinus impulse from reaching the atrium is sinoatrial arrest. Though rare in children, these disturbances may occur secondary to digitalis toxicity and extensive atrial surgery. A





­

­

Myocarditis, ischemic damage, anomalous origin of the coronary artery, cardiomyopathy, mitral valve prolapse, prolonged Q-T interval (congenital or acquired), proarrhy thmogenic drugs, Wolff-Parkinson-White syndrome, drug abuse with cocaine or amphetamine, hypokalemia, hypomag nesemia, hypoxia and severe acidosis are all known predisposing factors. In a significant number of children with SVT, an underlying cause may not be found. Syncope, chest pain and dyspnea are the common presenting symptoms. Ventricular tachycardia must be differentiated from SVT and all broad QRS tachycardias should be considered as VT until proved otherwise. The electrocardiographic features, which are helpful in the diagnosis of VT are the AV dissociation, capture fusion beats, extreme left axis deviation, no response to carotid sinus massage or intravenous administration of adenosine besides the very broad QRS complexes.

S

Cause

A

Ventricular tachycardia is defined as occurrence of at least three or more ectopic ventricular beats in sequence. Sustained VC means a run of ventricular premature contractions (VPCs) in succession for a period of 30 seconds or more. It is less common in children, and it indicates the presence of a serious underlying structural or functional cardiac problem. The prognosis is poor and carries a great risk of mortality unless corrected immediately.

S

Ventricular Tachycardia





T

V



The therapeutic goal is to immediately slow down the ventricular rate by using either beta blockers (propranolol) or calcium channel antagonists (verapamil). Quinidine or other Class 1A drugs as mentioned above or Class 1C drugs like flecainide may then correct the condition to sinus rhythm. If no response occurs within 24 hours, electrical DC conversion is resorted to. The causative factors have to be immediately attended. Anticoagulation 2 weeks prior and 2 weeks after any attempt at cardioversion is indicated to prevent the dreaded thromboembolic complications in situations where atrial fibrillation has been persistent for more than 48–72 hours. In exceptional circumstances of refractory atrial fibrillation, surgical or transvenous catheter radiofrequency ablation may be resorted to deliberately induce complete heart block with simultaneous permanent pacemaker implantation.

Treatment is immediately initiated. DC conversion (1–2 Watt/sec/kg) is the treatment of choice; if it is not available or if VT is relatively well tolerated, bolus dose of lignocaine (1 mg/kg) is intravenously administered with continuing intravenous infusion at a rate of 10–50 mg/kg/min. Bretylium is an alternative drug in lignocaine refractory cases. Mexilitine, flecainide, disopyramide and amiodarone are suitable alternatives. Phenytoin is effective in VT, especially when it occurs following digitalis toxicity. The precipitating factors— hypokalemia, hypomagnesemia and others have to be identified and immediately corrected. Myocardial tumor, anomalous origin of the coronary artery and similar surgical problems are appropriately handled. Failure of drug therapy necessitates alternative treatment strategies—implantation of automatic cardioverter, defibrillator or surgical resection of the diseased myocardial area. V



Treatment

Treatment

Diseases of Car iovas ular ystem

The ECG is characteristic with no organized discernible “P” waves, except for irregular, fibrillatory “f” waves in the baseline with irregular but normal QRS complexes.

trioventricular Block

Atrioventricular block occurs when interference occurs in normal conduction of the electrical impulses from the atria to the ventricles through the AV node.

469

vip.persianss.ir

ext ook of Pe iatri s

 

c

Table 7.6.1 Intravenous antiarrhythmic agents Dosage

Comments

Monitoring

Verapamil

0.1–0.2 mg/kg IV (5–10 mg max)

Contraindicated in children < 12 months of age

For hypotension

Propranolol

0.02 mg/kg IV every 5 minutes to maximum 0.1 mg/kg

Contraindicated in children with asthma, congestive heart failure. Not to be given with verapamil

Pulse, BP

Procainamide

10–15 mg/kg IV over 30 minutes

Continuous monitoring

Dizziness, hypotension

Lidocaine

1–2 mg/kg IV over 15 minutes cont, infusion: 30– 50 µg/kg/min

Causes respiratory depression, hypotension

BP

Adenosine

0.05 mg/kg through a central line; double the dose until effect is seen to a maximum of 0.4 mg/kg

Caution in asthmatics, contraindicated in pre-existing type 2 and 3. Atrioventricular block without pacemaker

T

b

d

Drug

b

econd Degree

AV

S

lock







Some of the atrial impulses are blocked and hence, not conducted to the ventricles. In Mobitz type I (Wenckebach phenomenon), while P-P interval remains constant, progressive increase in P-R interval occurs with successive beats until an atrial impulse seen as P wave is not conducted to the ventricle (absent QRS complex). The P-R interval is again shorter in the cycle following the dropped ventricular complex. It will then progressively increase to result in another blocked ventricular impulse. In Mobitz type II, atrial conduction is blocked at intervals without a change in P-R interval, once every three, four or five beats. It is less often noticed in individuals with normal hearts. The same predisposing factors mentioned in first degree

Congenital/ cquired Complete Heart Block A

P-R interval prolonged beyond what is normal for that age and heart rate without blockage of the conduction of any of the atrial impulses to the ventricles is defined as first degree heart block. No evidence of heart disease is seen in majority of such cases. However, it may be seen in children with CHD like ASD, corrected transposition of great vessels, Ebstein’s anomaly, primary myocardial disease, rheumatic carditis, diphtheria and in children receiving drugs like digitalis and quinidine. Children with first degree block are asymptomatic and need no treatment except for the treatment of primary cause.

AV block also play a role. No treatment is necessary. If Stokes-Adams syndrome occurs, although rare, pacemaker insertion is undertaken. Table 7.6.1 lists intravenous antiarrhythmic agents commonly employed.

AV

lock

Autoimmune injury of the fetal conduction tissue by IgG antibodies transferred from mother with active or inactive systemic lupus erythematosus (SLE) is one of well-known causes of this condition. Other autoimmune diseases such as rheumatoid arthritis are reported to cause congenital heart block. Myocarditis and postsurgical repair involving ventricles are other known causes of acquired complete heart block. In utero, it may result in hydrops fetalis. It may also result in fetal wastage. In some children, it may occur at 3–6 months of age. Older children are asymptomatic. Syncope, fatigue, irritability and night terrors may be some of the symptoms. Slow but bounding pulse less than 60/min not increasing by more than 10–20 beats/min after exercise or atropine administration, cannon a waves, varying intensity of the S1 are diagnostic. The diagnosis is confirmed by ECG. The prognosis of this condition is usually good. In symptomatic children with Stokes-Adams syndrome, insertion of artificial pacemaker is imperative to prevent sudden death. All cardiac depressants should be avoided. Cardiac pacing is recommended in neonates with low ventricular

b

irst Degree

AV

F

Abbreviations: IV, Intravenous; BP, Blood pressure

470

vip.persianss.ir

6.







d

1. Campbell RM, Dick M, Rosenthal A. Cardiac arrhythmias in children. Annu Rev Med. 1984;35:397-410. 2. Losek JD, Endom E, Dietrich A, et al. Adenosine and pediatric supraventricular tachycardia in the emergency department:





Bibliography

c

5.

S







4.





3.

multicenter study and review. Ann Emerg Med. 1999;33:18591. Sacchetti A, Moyer V, Baricella R, et al. Primary cardiac arrhythmias in children. Pediatr Emerg Care. 1999;15:95-7. Walsh EP, Saul JP, Triedman JK (Eds). Cardiac arrhythmias in the pediatric patient. Philadelphia: Lippincott Williams and Wilkins; 2001. Wolff G, Gelband H, Deal BJ. Arrhythmias in infants and children: Current concepts in diagnosis and management of atrial arrhythmias in infants and children. New Your Futura Publishing Inc; 1998. Ziegler VL, Gillette PC (Eds). Practical Management of Pediatric Cardiac Arrhythmias. New York Futura Publishing Inc; 2001.

Diseases of Car iovas ular ystem

rate (50/min), evidence of heart failure, wide complex rhythms or CHD. Isoproterenol, atropine or epinephrine may be used to increase the heart rate temporarily until pacemaker placement can be arranged. Transthoracic epicardial implants have traditionally been used in infants. Transvenous placement of pacemaker lead is available for young children.

471

vip.persianss.ir

Section

8 Diseases of Respiratory System



















8.1 8.2 8.3 8.4 8.5 8.6 8.7 8.8 8.9 8.10























Section Editor : TU Sukumaran Examination of the Respiratory System: TU Sukumaran Diagnostic Procedures and Investigations in Respiratory Diseases: D Vijayasekaran Respiratory Distress: Madhumati Otiv Upper Respiratory Tract Infections: Sushil K Kabra Croup: Deepak Ugra Pneumonia: Rohit C Agrawal Bronchiolitis: A Balachandran Empyema: Varinder Singh Suppurative Lung Disease: L Subramanyam Bronchial Asthma: H Paramesh

vip.persianss.ir

Examination of the Respiratory System

iseases





D

8.1

TU Sukumaran

These include abnormal breathing patterns, altered sensorium, convulsions, cyanosis, and inability to drink, sweating, hypotonia, repeated vomiting, dehydration and paralysis. Altered sensorium and convulsions indicate hypoxemia.

Vital Signs Tachypnea (Fast Breathing) This usually occurs in pneumonia, but can also occur in anxiety, asthma, collapsed lung cardiac failure, pulmonary edema, pneumothorax and pleural effusion. The rate of breathing should be assessed by counting it for 1 minute; it should be repeated in case of any doubt. Rapid, shallow breathing denotes respiratory muscle paralysis. Metabolic acidosis of any etiology is characterized by an increased rate and depth of breathing.

Tachycardia Sinus tachycardia may be a manifestation of respiratory tract infection. Anxiety, cardiac failure, respiratory failure, simultaneous intake of sympathomimetic drugs and so on should be considered.

Temperature Temperature of 102°F indicates upper (sinusitis, otitis media, tonsillopharyngitis and mastoiditis) as well as lower respiratory tract bacterial infection. Common causes for high fever in lower respiratory illness are pneumonia, empyema, lung abscess and bronchiectasis. Low grade fever occurs in mycoplasma infections.

Blood Pressure Blood pressure should always be recorded. Hypertension and pulsus paradoxus indicate serious respiratory impairment.

espirator

Apnea which is cessation of breathing for more than 20 seconds is associated with bradycardia or neurologic impairment. A duration of less than 20 seconds which is associated with pallor, limpness, cyanosis or convulsions, should be a cause for alarm • Cheyne-stokes breathing is characterized by rhythmic waxing and waning of the depth of respiration, with regularly recurring periods of apnea. It is seen in congestive heart failure (CCF) and raised intracranial pressure with cerebral cortical impairment • Biot breathing also called ataxic respiration is characterized by irregular periods of apnea, alternating with periods of four or five breaths of identical depth, and indicates severe brainstem damage • Kussmaul breathing is rapid deep breathing of metabolic acidosis • Gasping or jaw breathing is of slow rate with rapid inspiration, prominent jaw movement and slow expiration. It indicates severe hypoxia due to any cause.

ste

Work of Breathing This includes flaring of alae nasi, head nodding (sternomastoids and scalene over-activity) and chest retractions (intercostals, subcostal, suprasternal and infrasternal).

Chest and Abdominal Movements Observe the chest movements from the side in supine and standing positions to confirm that both sides are moving equally and to assess the relative contributions of chest and abdomen. In normal inspirations, the lower chest flares out and the abdomen moves forward by the actions of the lower intercostal muscles and diaphragm, respectively. When the intercostals are paralyzed, as in spinal muscular atrophy, inspiration causes the lower chest to be drawn inward by the diaphragm, and the outward movement of the abdominal wall is prominent. Such movements can occur also in upper airway occlusion and are caused by the violent action of the diaphragm. The reverse movement is seen in diaphragm weakness, in which the abdomen is drawn inward during inspiration (paradoxical breathing). In unilateral phrenic nerve paralysis, the abdomen is drawn in on the paralyzed side during inspiration, but is normal (i.e. moves out) on the non-paralyzed side. Cyanosis in respiratory diseases indicates a serious degree of hypoxia and can be identified by the hyperoxia test. Associated circulatory failure or underlying cardiac lesions should be ruled out.



Look for Danger Signs

•

m

General Assessment

Examination of the Respiratory System

y Sy

Respiratory disorders are as common in children as it is in adults. A systematic history taking coupled with a physical examination, is necessary for arriving at a diagnosis. In this chapter, author has discussed on the examination of the respiratory system. Physical examination consists of general assessment of the patient, vital signs and examination of upper and lower respiratory tract.

Of R

Introduction

473

vip.persianss.ir

Indicators of Serious Chronic Respiratory Illness These include persistent fever, limitation of physical activity, chronic purulent sputum, cyanosis, clubbing, persistent tachypnea, labored breathing, growth retardation, persistent chest hyperinflation, and a family history of heritable lung diseases.

Examination of the Upper Respiratory Tract Use a bright torch to examine the nose, ears and throat.

symptoms of pharyngeal infection are redness and mottling over the soft palate and uvula. In a retropharyngeal abscess, the neck is hyper extended and respiration becomes noisy with gurgling sounds and pooling of secretions. In lateral pharyngeal abscess, the features are torticollis toward the same side, trismus, and bulging in of the lateral pharyngeal wall. Peritonsillar abscess also is characterized by trismus and torticollis. Adenoidal tissue hypertrophy on the posterior pharyngeal wall may reveal cobble stoning. The integrity of the palate should be investigated by palpation to exclude a submucous cleft. A bifid uvula is a clue to an occult submucous cleft palate.



ediatrics o

k fP tboo e

T x

Clubbing in respiratory diseases is seen early in cystic fibrosis (CF), bronchiectasis (of at least 1 year duration) lung abscess, empyema and malignancies.

Examination of the Lower Respiratory Tract

Signs of Allergic Problems

Neck

People with allergic rhinitis frequently develop a transverse nasal crease resulting from repeated rubbing of the nose to relieve the itching (Darriers line). Another feature of nasobronchial allergy is dark circles under the eyes (allergic shiners).

The important aspects to be assessed in relation to the respiratory tract are the trachea, neck vein and the presence of surgical emphysema.

Changes in the Anatomical Structures ­

The nasal passages may be narrow, as in midface hypo plasia associated with various syndromes. Congenital abnormalities, such as a deviated nasal septum, should be looked for. Extensive nasal polyps cause widening of the nasal bridge. Signs, such as the presence of ulceration, crusting, purulent or a blood-stained discharge, presence of foreign bodies, trauma and tumors (vascular and nonvascular) should be noted.

Examination of Sinuses Sinus tenderness can be elicited on the affected sinus.



Examination of the Ears The ears should be examined for congenital anomalies, infections, foreign bodies and impacted wax. Gently move the pinna and tragus; tragal pain suggests otitis externa. Examine the mastoids and retroauricular areas for eczema, tenderness and lymph node enlargement. For better visualization of the external ear canal and tympanic membrane, pull the pinna up and posteriorly with the thumb and index finger. Otoscopic evaluation is a must for patients with fever of unknown or known origin, as well as those with ear symptoms. In the latter situation, the healthy side should be evaluated first. The otoscope should have a strong light and the speculum should be of the largest size that can fit comfortably into the ear canal. Gently place the otoscope over the external auditory meatus and inspect the external ear canal. Carefully advance it to observe the tympanic membrane. Absence of the normal light reflex, dull appearance, bulging, retraction, perforation, and so on, should be noted. Examination of the Throat 474 The tonsils should be examined for size (hypoplastic in

X-linked agammaglobulinemia; enlarged in infections and tumors), congestion, follicles or membranes. Other

Trachea This is inspected and palpated for deviation (in the standing or sitting position), with the examiner facing the patient. Tracheal deviation causes the clavicular head of the sternomastoid muscle on that side to appear prominent (Trail sign). In severe respiratory distress, “tracheal tug” (i.e. pulling in of the trachea) occurs because of the vigorous contraction of the diaphragm pulling down the mediastinum. Gently palpate the trachea with the middle finger at the suprasternal notch. Should there be massive pleural effusion, pneumothorax and large intrathoracic cysts and tumors; the finger will slip to the opposite side? It is pulled to the same side in upper lobe collapse, fibrosis and pleural adhesions.

Signs of Superior Mediastinal Obstruction Signs of superior mediastinal obstruction are edema of the head and neck, cyanosis, proptosis, Horner’s syndrome and distended non-pulsatile neck veins. The common cause is mediastinal mass, notably because of a lymphoma.

Examination of the Chest Inspection This should be performed with the chest as maximally exposed as the custom permits, with the eyes at the level of the chest, from head and foot ends and from front and lateral aspects. Evaluate the overall shape and appearance of the chest. Assess the shoulders for drooping (indicates painful conditions of the chest wall on the same side, pleurisy, pneumonia, collapse and fibrosis), position of the cardiac apical impulse, and precordial shape. The anteroposterior (AP) diameter of the chest is increased in hyperinflation. A localized bulge indicates parietal wall inflammatory swellings, hematoma or tumors. In pleural effusion or pneumothorax, intercostal spaces (ICS) appear bulging. Look for chest movements. Localized paucity of movements occurs in trauma and in painful conditions of the chest wall and pleura, pleural effusion, pneumothorax, pneumonia, collapse and tumors of the lung.

vip.persianss.ir

iseases espirator ste

­



Auscultatory Percussion It is helpful to detect mediastinal masses. The patient sits with arms resting on both thighs while the examiner sits facing the patient. With the tip of the terminal phalanx of the middle finger of the dominant side, light percussion is directly applied over the sternum from the center to either side, while the note is auscultated from behind over the corresponding areas. The area of impaired resonance reveals the size of the mediastinal mass.

Auscultation Developing an expertise in interpreting auscultatory findings is an experimental process and, as such, there is no substitute for the experience of having listened to a large number of patients, both with and without lung disease. Follow a consistent pattern for auscultation to avoid missing any area. Start from the infraclavicular area; proceed to mammary, inframammary, axillary and infra-axillary areas, each time comparing with the opposite side. Segmental auscultation is the technique of comparing breath sounds of homologous segments. The examiner should sit facing the patient when auscultating the anterior aspect of the chest. The lateral side of the chest can be auscultated from front as well as from behind the patient. Posteriorly, auscultation is performed in the same way as in the order of percussion, with the examiner sitting behind the child. The parameters to be assessed are the breath sounds, added sounds and vocal resonance. Assess the intensity and quality of the breath sounds. Intensity may be normal (when the lung tissue is inflating normally), reduced or increased. It is reduced in airway obstruction, lung damage, pleural thickness, effusion, and pneumothorax. The normal breath sounds are vesicular. The inspiration is loud and highpitched followed by harsh low-pitched expiration (without 475 a pause in between), which is of short duration, i.e. almost up to half of inspiration. The cycle is followed by a pause.

­





m

The examiner should master the technique of percussion to feel, rather than hear, the normal and abnormal lung resonance. The patient should be in a comfortable position, i.e. sitting or lying down. The left middle finger (the pleximeter) is placed parallel to the long axis over the area to be percussed; back of the middle phalanx of the pleximeter finger is struck, as a tapping movement arising from the wrist. The plexor finger should immediately be raised after the blow. The terminal phalanx of the plexor finger should be at right angles to the metacarpal bone and the pleximeter finger while the blow is delivered. Proceed down the ICS in mammary line, with each blow being compared with that on the other side. The upper border of liver dullness reaches the 5th ICS on the right side in the mammary line and will shift to one space lower down on deep inspiration (tidal percussion). On the left side, the normal resonance is replaced by the tympanitic note of the stomach at the same level. The cardiac dullness should be verified on the left side and will be more resonant in emphysema. To percuss the lateral side of chest, patients’ limbs should be raised above the shoulders with the hands resting over the vertex – one over the other. Each ICS is percussed and compared with the opposite side, with the examiner standing or sitting on the side to be percussed. The normal resonance on the right side is replaced by liver dullness in the 7th space in the mid-axillary line, and on the left side by the tympanitic note of the stomach almost at the same level. Percussion of the back should start from the supra clavicular area, i.e. from the medial to the lateral side, the pleximeter finger being placed across the area with the tip directed forward. A band of lung resonance that normally presents in the central area (Kronig isthmus) will be replaced by a dull note in massive pleural effusion, consolidation, neoplasia, or collapse of the apical part of lung. Subsequently, the upper and lower interscapular and the infrascapular spaces should be percussed. The normal resonance is replaced by the dullness at the 9th and 10th ICS in the infrascapular area on the right (liver) and left side (spleen) respectively. Resonance is the normal feel and sound caused by air being set into vibration. Lung resonance is increased

y Sy

Percussion

Of R

This includes measurement of the expansion of the chest at the level of the nipples after deep inspiration and expiration. Expansion of identical sides of the chest should be assessed. Chest movements are assessed over supraclavicular, upper interscapular and lower interscapular areas from the back, and infraclavicular and inframammary areas from the front. To do this, encircle the chest from both sides with four fingers and palm, with the thumbs meeting in the midline but not touching the chest wall. On inspiration, if one thumb remains closer to the midline it indicates reduced chest movements on that side. Tactile vocal fremitus is compared for spoken words over identical areas on both sides of the chest with the medial margin of the right palm.

in airway obstruction and pneumothorax; in the latter situation it reaches up to a tympanitic note. Localized obstruction occurs in congenital lobar emphysema or in partial obstruction by a foreign body in the bronchus (check valve or ball valve). Overall, hyperinflation occurs in obstructive airway disease, as in asthma pushing down the liver dullness. Percussion note is impaired or dull when the lung becomes more solid (pneumonia, consolidation, collapse, fibrosis, sequestration, abscess, infraction), or the pleura becomes thickened or contains fluid (pleural effusion, empyema, hemothorax) or solid (mesothelioma). A feel of resistance below the pleximeter finger is perceived in pleural effusion, as if percussed against a wall, and is described as a “stony dullness”. In hydropneumothorax, a shifting dullness can be identi fied by percussing the chest (from anterior to posterior) in the supine, lying position of the patient. As the fluid shifts to the posterior part of the chest in the lying position, the dull lower chest (in the sitting position) becomes resonant anteriorly and a definite level below which there is complete dullness can be demonstrated.

D

Palpation

vip.persianss.ir

ediatrics

Parameter

Acute asthma

Consolidation

Collapse

Pleural effusion

Pneumothorax

Respiratory rate

↑

↑↑

↑↑

↑↑↑

↑↑↑

Chest in-drawing

+++ Overall

++ Spares affected region

++ Spares affected region ++ Spares affected region

++ Spares affected region

Chest expansion

Overall reduction

Reduced over affected area

Reduced over affected area

Reduced over affected area

Reduced over affected area

Tracheal and mediastinal shift

Nil

Nil

Toward the affected side

Toward the opposite side

Toward the opposite side

Lung resonance

Hyper-resonant; liver Dull over affected area dullness pushed down

Dull over affected area

Stony dullness over affected area

Tympanitic over affected area

Breath sound intensity

N/↓

↑

↓/ -over affected area

↓ / -over affected area

↓/ -over affected area

Breath sound quality

Bronchovesicular

Bronchial

Vesicular/Bronchial

Vesicular

Vesicular

Normal

↑↑

↓↓

Imperceptible

Imperceptible



476









and expiration; unlike crackles, it does not change after coughing. The intensity increases as the chest piece is pressed firmly; it can be felt also with the palpating hand. Mediastinal crunch (Haman sign) is heard in emphysema and left-sided pneumothorax. A crunching sound will be heard over the left sternal border during a systole. Peristaltic sounds are gurgling sounds occasionally heard over the lower part of the left side. Frequent peristaltic sounds with other clinical evidences indicate diaphragmatic hernia. In addition to peristaltic and diaphragmatic sounds crepitus sounds may occur over the region of a broken rib or surgical emphysema. Additional false sounds arise by the movement of the stethoscope on the patient’s skin, and should be differentiated from the symptomatic sounds. Vocal resonance is the sound perceived by the chest piece as the voice is transmitted through the lungs. The child is auscultated while saying one or his name in the same order of auscultation, and both sides are compared. The intensity of resonance will be decreased in airway obstruction, collapse, pleural effusion or pneumothorax (except when bronchial breathing is present). Intensity is increased over consolidation, cavity, infraction and collapse consolidation. If the patient is asked to whisper the same words, they will be heard clearly in the examiners ears (whispering pectoriloquy) in massive consolidation and bronchopleural fistula (Table 8.1.1).

Bibliography

1. Empey D. The respiratory system. In: Swash M (Ed). Hutchison’s Clinical Methods, 10th edition. London: WB Saunders and Company Ltd. 1995.pp.60-8. 2. Kuruvilla VJ, Thulasi Prasad K. Clinical Evaluation of Newborns, Infants and Children. 2nd edition. New Delhi: Jaypee Brothers Medical Publishers (P) Ltd. 2009.pp.112-20. 3. Silverman M, Callaghan Cl. In: Practical Pediatric Respiratory Medicine, 1st edition. New Delhi: Viva Books Pvt Ltd, 2005.











In bronchovesicular breathing, the expiration is prolonged up to or more than the inspiration, and there is no pause in between. This is typical of asthma. Bronchial breathing is louder in intensity. Expiration is high-pitched, loud and prolonged (equal to or more than the duration of inspiration). There is a pause in between. The normal vesicular breathing is replaced by bronchial breathing when the lung becomes more solid (as in consolidation, collapse consolidation, fibrosis, abscess lung, massive pleural effusion and neoplastic growth) or contains a cavity. In these situations the sounds generated in large airways are transmitted more efficiently and resemble those obtained by listening over the trachea. Adventitious sounds are wheezes, crackles, pleural rub, mediastinal crunch, peristaltic sounds and crepitus. Wheezes arise from narrowed intrathoracic airways. Monophonic wheeze is due to localized narrowing of a single bronchus, as in foreign body aspiration or bronchial adenoma. Widespread polyphonic wheezes (of differing intensity); usually heard in expiration indicate diffuse airway obstruction as in asthma. Crackles or crepitations are short bubbling or crackling noises arising from large airways with secretions (coarse), or from small airways (fine), and from alveoli containing exudates (fine). These sounds are produced by sudden changes in gas pressure from the rapid opening of previously closed small airways or alveoli. Crackles are heard in obstructive airway disease, pneumonia and pulmonary edema. Localized loud and coarse crackles may indicate bronchiectasis. The added sounds should be differentiated from conducted sounds from the throat and pharynx. Conducted sounds disappear after suction and changes in position and are heard with equal intensity over equidistant parts of the chest, and better by auscultation in front of the mouth and neck. Pleural rub has a rubbing, superficial, leathery character, and is heard during an identical phase of inspiration



Vocal resonance



e

T x

tboo

o

k fP

Table 8.1.1 Salient features of common lower respiratory diseases in children

vip.persianss.ir

Complete blood count may give information about the current infection. Leukocytosis [white blood cell (WBC) above 16,000/µL] and leukopenia (WBC below 4,000/µL) may suggest acute infection. Raised ESR suggests chronic infection and inflammatory disorders. The CRP is useful in monitoring the disease.

Optional

Hematological • Complete blood count • Erythrocyte sedimentation rate, C-reactive protein

Pulmonary function tests • Spirometry • Peak expiratory flow rate

Imaging studies • Chest X-ray • Contrast radiography • Fluoroscopy • Computed tomography and magnetic resonance imaging • Ultrasound chest • Radionuclide lung scans

Endoscopy • Fiberoptic bronchoscopy • Bronchoalveolar lavage • Rigid bronchoscopy • Laryngoscopy

Microbiological • Gastric lavage • Induced sputum • Sputum, blood culture

Monitoring • Pulse oximetry • Capnography • Polysomnography

Miscellaneous • Immunoglobulin assay • Radioallergosorbent assay • Mantoux test • Allergic skin tests • Sweat chloride

Procedures • Thoracocentesis • Intercostal drainage • Video assisted thoracoscopy 

M

r

agnetic

The CT provides more information than the plain skiagram and can reconstruct three dimensional views. With the use of intravascular contrast, vascular rings can be studied. In high resolution CT (HRCT), the cuts are taken with the collimator distance at about 1–2 mm. Bronchiectasis (cylindrical) and interstitial lung disease are identified early with HRCT. Since MRI does not involve radiation, it is preferred in young infants for the same indications as CT. F

Common

Computed omography and Resonance maging T

Table 8.2.1 Investigations and procedures in respiratory diseases

Barium swallow gives information about the upper gastrointestinal (GI) tract especially in the evaluation of gastroesophageal reflux, achalasia and hiatus hernia. The demonstration of “pulled up cecum” in barium (meal) follow-through suggests abdominal TB.

I

T

M

One tuberculin unit (TU) purified protein derivative with Tween 80 is injected intradermally and the reaction read between 48–72 hours. Mantoux test is considered positive if the induration is 10 mm or more. Similar to Mantoux, Interferon Gamma Release Assays also detects



The standard chest radiograph is the posteroanterior (PA) but in younger children AP view is taken for convenience. Chest X-ray delineates four densities namely air, fluid, soft tissue and bone density (Figs 8.2.1A to D). Before viewing the skiagram, it is assumed that technical qualities (projection, orientation, rotation, penetration) are fulfilled. Pneumonia, collapse, obstructive emphysema, pneumothorax and pleural effusion can be diagnosed with CXR.

Contrast Radiography

antoux est



S

 

T

Hematological ests

Chest Radiography

y ystem

Respiratory diseases are a major cause of mortality and morbidity in children. Detailed history taking and methodical clinical examination help to arrive to a closer diagnosis but to confirm, investigations are required. Children with chronic lung problems require many investigations (Table 8.2.1).

tuberculosis (TB) infection. Since it uses selective antigens namely early secretion antigen target 6 (ESAT 6) and culture filtrate protein 10 (CFP 10), it is not affected by prior bacille Calmette-Guerin (BCG) and infection due to nontuberculous mycobacterium.



ntroduction

or

I

P



I

D Vijayasekaran

Diseases Of Respi at

Diagnostic rocedures and nvestigations in Respiratory Diseases

8.2

luoroscopy

The combination of fluoroscopy along with barium swallow forms a useful investigation in the evaluation of children with gastroesophageal reflux disease [GERD (eosinophils for late phase reaction in general)]. To study the diaphragmatic movements and suspected foreign body fluoroscopy plays a significant role.

Ultrasound Chest In the evaluation of eventration of the diaphragm, ultrasound may arrive at diagnosis. It helps to differentiate 477 loculated pleural fluid from lung abscess.

vip.persianss.ir

o

P

d

r

c

k f e iat i s oo

extb

B

T

A

C

D

nduced putum S

I

The patient is nebulized with 3% hypertonic saline to induce sputum. Secretions are then collected from the throat or 478 nasopharynx. Recent studies report that induced sputum yield higher results than GL.

eak xpiratory low Rate F

Pediatric TB is a paucibacillary disease. The GL is done in the early morning to isolate the acid fast bacilli. Mycobacterium growth indicator system (MGIT) and battle area clearance and training equipment consultants (BACTEC) detect much faster (< 2 weeks) than Lowenstein Jensen medium. The GL can also be done as an ambulatory procedure after 4–6 hours fasting.

E

Gastric Lavage

Spirometry differentiates lung diseases (functionally) into obstructive and restrictive diseases but does not give any information about the etiology. Spirometry measures the forced vital capacity (FVC) and not the individual lung volumes. The other indices like forced expiratory volume in 1 second (FEV1), the ratio of FEV1 with FVC (FEV1/FVC), forced expiratory flow 25–75% of FVC [forced expiratory flow (FEF) 25–75%] are derived from FV C by the machine (Fig. 8.2.2). Achieving a good forced vital capacity curve is the most important aspect of spirometry. For this a child (after appropriate coaching) should take a deep breath to full inhalation followed by a brief hold and then a sustained exhalation (at least 3 seconds) with maximum effort to produce a good curve (flow volume curve or loop). Forced vital capacity is diminished in both obstructive and restrictive diseases. The FEV1 is decreased in obstructive diseases. The FEV1/FVC ratio may be normal or even increased in restrictive diseases.

Microbiological tests in children pose many disadvantages like sputum collection (children < 7 years don’t expectorate), contamination from upper airway flora and difficulty in differentiating infection from colonization.

pirometry



icrobiological tudies S

M

Ventilation perfusion studies are indicated in the evaluation of pulmonary embolism or anomalies of lung. Perfusion studies are done with radioactive technetium (99mTc). Radioactive xenon133Xe scan study both perfusion and ventilation in a single sitting.

P

S

Radionuclide Lung cans

S

 

Figures 8.2.1A to D X-ray chest showing four densities namely air, fluid, soft tissue and bone density

Peak flow meter records peak expiratory flow rate, the greatest flow obtained on forced expiration after full inspiration. Peak expiratory flow rate is effort dependent and measures mostly large airway function. It plays a major role in monitoring asthma therapy (correlates with FEV1) and sudden fall may be an indicator of impending asthma attack.

vip.persianss.ir

S

y ystem

or

r

Diseases Of Respi at

B

F

iberoptic ronchoscopy

Fiberoptic bronchoscopy is an important diagnostic technique, done under local anesthesia. The diagnostic indications and therapeutic utility are increasing (Table 8.2.2).

B

ronchoalveolar Lavage

Bronchoalveolar lavage (BAL) is a diagnostic procedure, allows the recovery of both cellular and non-cellular components from the epithelial surface of the lower respiratory tract. Bronchoalveolar lavage is rightly called as the “liquid biopsy of the lung”. After wedging the scope into the desired sub-segmental bronchus, BAL is performed by instilling 2 mL/kg of sterile, normal saline. Bronchoalveolar lavage is helpful in diagnosing opportunistic infections like Pneumocystis jiroveci, cytomegalovirus, Aspergillus fumigatus and Mycobacterium tuberculosis. T







Arterial blood gas (ABG) analysis reveals oxygenation status, adequacy of ventilation and acid-base balance. Many serious acid-base disturbances can coexist without significant clinical manifestations. The radial artery at the wrist is the most preferred site of ABG specimen collection as it has adequate collaterals. Heparinized syringe with 22 gauge needle should be held like a pen with 45º angles (with beveled edge of the tip facing downward) and the artery should be punctured just 2 cm above the wrist crease and the sample should be analyzed immediately. The normal pH of our body is 7.4 (range 7.36–7.44), depends upon the levels of PaCO2 and HCO3 and their disturbance results in four simple acid-base disorders namely metabolic acidosis (↓ HCO3), metabolic alkalosis (raised ↑ HCO3), respiratory acidosis (↑ PaCO2) and respiratory alkalosis (↓ PaCO2). Metabolic acidosis results due to loss of HCO3 from the body (diarrhea) or due to addition of acids (lactic acid and keto acids) and the anion gap calculation is useful to differentiate the above two. Metabolic alkalosis results due to loss of acids (H+ ions) from the GI tract (vomiting) or through renal mechanisms (Bartter syndrome). Mild respiratory disorders cause hypoxemia resulting in respiratory alkalosis due to CO2 washout. Advanced respiratory diseases and neuromuscular dysfunction leads to CO2 retention (PaCO2 > 45 mm Hg) resulting in respiratory acidosis.

After lignocaine gel is applied into the nostril, lignocaine solution is instilled through the suction channel of bronchoscope as “spray and precede technique”. Dynamic lesions like vocal cord pathology, laryngomalacia, tracheomalacia and foreign body are better identified with FBS (Figs 8.2.3A to D).

horacocentesis

Thoracocentesis or pleural tap is done to diagnose pleural fluid (preferably after ultrasonographic confirmation). Nature of the fluid and biochemistry may be useful to differentiate transudate versus exudate (Table 8.2.3). Table 8.2.2 Fiberoptic bronchoscopy - indications  

A

rterial lood Gas nalysis B

A

 

Figure 8.2.2 Spirometry recordings of lung volumes and flow volume loop

Diagnostic

Therapeutic

Persistent pneumonia, atelectasis Prolonged stridor and wheezing Suspected foreign body, endobronchial TB Suspected airway anomaly Evaluation of hemoptysis and hemosiderosis Bronchoalveolar lavage

Removal of mucus plug/ bronchial cast Removal of retained secretions Drug instillation Bronchoscopy guided endotracheal tube intubation Tracheostomy evaluation Endobronchial biopsy

479

vip.persianss.ir

o

P

d

r

c

k f e iat i s oo

extb

B

C

D

T

A

 

Figures 8.2.3A to D Bronchoscopic view of laryngomalacia, laryngeal web, tracheomalacia and central foreign body

Feature

Transudate

Exudate

Cause

↑ Hydrostatic and ↓ oncotic pressure

Inflammation Infiltration of pleura

Appearance

Clear

Turbid pus, straw color

Protein

< 3 g/dL

> 3 g/dL

Lactate dehydrogenase

< 200 IU/L

> 200 IU/L

pH

> 7.2

< 7.2

Glucose

> 40 mg/dL

< 40 mg/dL

Pleural fluid/serum protein

< 0.5

> 0.5

Pleural fluid/serum lactate dehydrogenase < 0.6

> 0.6

Possible diagnosis

Complicated parapneumonic effusion, empyema

Congestive heart failure, nephrotic syndrome, cirrhosis, hypoproteinemia

ntercostal Drainage

Intercostal drainage (ICD) is the procedure to remove pus from the pleural cavity by inserting a chest tube. Fifth intercostal space at mid-axillary line is the ideal site.

S

I

 

Table 8.2.3 Differentiation between transudate and exudate

weat Chloride

Sweat is collected by pilocarpine induced iontophoresis. Electric current (3 mA) is passed for 30 minutes and about 100 mg of sweat is collected. Chloride estimation is done by titration. Sweat chloride more than 60 mEq/L on two occasions confirms CF.

480

vip.persianss.ir

The main functions of respiratory system are oxygenation and ventilation. This happens appropriately when almost all of the cardiac output (perfusion) returns to the heart only after taking part in gas (ventilation) exchange. Ventilation is poor and perfusion remains relatively normal in cases of pneumonia, non-cardiogenic pulmonary edema and asthma (Low V/Q). On the other hand in cyanotic congenital heart diseases and pulmonary embolism ventilation is normal while perfusion is decreased (High V/Q).

< 2 months

> 60 breaths/min

2–12 months

> 50 breaths/min

1–5 years

> 40 breaths/min

> 5 years

> 20 breaths/min



Disturbance in oxygenation or CO2 removal when sensed by the carotid bodies and the central (medullary) carotid receptors results into increased work of breathing (tachypnea/dyspnea). Also normal respiration requires intact central nervous system (CNS) control and respiratory apparatus therefore loss of central control can also result into abnormal pattern of respiration such as periodic or irregular respiration. Additionally increased metabolic demands such as sepsis can result into respiratory distress. Thus, respiratory distress can be the result of number of diseases which may be respiratory or nonrespiratory. Localization of respiratory distress is usually not so difficult if detailed history and physical examination is carefully performed. Table 8.3.2 shows etiological classification of respiratory distress. There are some conditions such as epiglottitis, anaphylaxis, foreign body, tension pneumothorax, chest trauma (flail chest), cardiac tamponade, which need to be handled very urgently.

r

 

tiology and athogenesis P

E



E

Clinical eatures and valuation F

Goals of Clinical xamination E

Tachypnea is increased in the respiratory rate beyond the age specific physiological limit (Table 8.3.1) while dyspnea or respiratory distress is characterized by signs of increased work of breathing (characterized by stridor, wheeze, tachypnea or hyperpnea, use of accessory muscles, and/ or retractions). A patient with inadequate respiratory effort may or may not have signs of increased work of breathing such as retractions, but usually has an abnormal pattern of respiration or a respiratory rate that is inappropriately slow for the clinical condition. Respiratory failure is defined as inability of the respiratory system to fulfill the gas exchange needs of the patient. Most of the times physical examination alone is sufficient to realize that the blood gas abnormalities are present or imminent and should never delay the institution of life saving measures. It is characterized by altered sensorium, dusky color, poor tone, weak or absent cry, poor pulses or capillary refill time (N = less than 2 seconds).

Breaths/min

• •

To identify respiratory failure To localize the origin of respiratory distress/respiratory failure. As mentioned earlier in this chapter identification of respiratory failure can be done by simple clinical examination. If child is in respiratory failure, urgent resuscitation as per pediatric advanced life support (PALS) guidelines is the priority. Once respiratory failure is excluded, localization of respiratory distress by physical signs should be done which will help in designing treatment to prevent the child from going into respiratory failure (Table 8.3.3). While examining it is important to provide oxygen in the most comfortable way that the child can tolerate as far as possible in mother’s lap. Respiratory distress worsens in a crying child. A conscious child finds the most comfortable position such as head extension in case of upper airway obstruction. 481 Although this is an emergency, there is no substitute to a detailed history taking after initial stabilization. Availability of pulse oximetry is highly desirable with every

Definitions

Age

y ystem

Respiratory distress and respiratory failure together account for nearly 50% of pediatric intensive care unit (PICU) admissions. Infants and young children become fatigued and/or decompensate more quickly than older children and adolescents due to smaller airways, increased metabolic demands, decreased respiratory reserves, transverse placement of ribs with poor mechanical advantage, easy fatigability of intercostal muscles thus inadequate compensatory mechanisms. With supportive care and aggressive treatment of the underlying cause, most children with respiratory distress recover uneventfully. However, outcomes are poor for patients who develop cardiopulmonary arrest as the result of respiratory deterioration. This chapter describes definitions of respiratory distress and failure, diagnosis, pathophysiology, clinical features with emphasis of early diagnosis of respiratory failure and some life-saving maneuvers. Management of individual causes of respiratory distress is discussed elsewhere in this book.



Table 8.3.1 World Health Organization definition of tachypnea in children

or

ntroduction



I

Madhumati Otiv

S





Respiratory Distress

Diseases Of Respi at

8.3

vip.persianss.ir

o

P

d

r

 

c

k f e iat i s oo

extb

Respiratory causes of respiratory distress

Nonrespiratory causes of respiratory distress

Respiratory tract Infection • Epiglottitis* • Retropharyngeal abscess • Peritonsillar abscess • Croup • Tracheitis • Bronchiolitis • Pneumonia Asthma Anaphylaxis* Foreign body* • Upper airway • Lower airway • Esophageal Biologic or chemical weapons Chest wall/thoracic • Air leak (e.g. tension pneumothorax*) • Chest wall deformity (e.g. thoracic dystrophy, flail chest*) • Mass lesion (e.g. pulmonary sequestration, malignancy)

Cardiovascular • Heart failure • Cyanotic heart disease • Pericarditis • Cardiac tamponade* • Myocarditis Trauma • Blunt or penetrating (e.g. pneumothorax, lung contusion) • Inhalational injury (e.g. airway burn, smoke inhalation) Nervous system • Depressed ventilation (from ingestion, injury, or infection) • Hypotonia (poor pharyngeal tone, ineffective respiratory effort) • Loss of airway protective reflexes Metabolic/Endocrine • Acidosis (e.g. diabetic ketoacidosis, severe dehydration, sepsis) • Hyperthyroidism/hypothyroidism Hematologic • Decreased oxygen carrying capacity (e.g. severe anemia, methemoglobinemia) Gastrointestinal • Splinting from abdominal pain, abdominal distension, aspiration as a gastroesophageal reflux 

T

Table 8.3.2 Etiological classification of acute respiratory distress in children

* Extremely life-threatening, commonly occurring diseases

 

Table 8.3.3 Localization of respiratory distress by physical findings Upper airway obstruction

Lower airway disease

Sniffing position: neck is flexed with head extended to open airway

Retractions: intercostal, subcostal

Nasal flaring: also seen with lower airway disease

Nasal flaring: also seen with upper airway obstruction

Prolonged inspiration

Prolonged expiration: lower airway obstruction

Retractions: supraclavicular, suprasternal

Wheezing: intrathoracic airway obstruction

Abnormal voice: hoarseness, stridor, barking cough

Grunting: expiratory sound heard in young children with severe hypoxia or severe pain from an intra-abdominal process

Transmitted upper airway sounds (stridor)

Crackles (rales) Pleural rub

Cardiac disease

Bronchophony

Gallop or other murmur

Pulsus paradoxus: caused by lower airway obstruction. May also be seen with cardiac tamponade

Jugular venous distention Metabolic disease

Pulsus paradoxus: caused by cardiac tamponade. May also be seen with lower airway obstruction

Kussmaul respirations

Hepatomegaly

or failure suggests CNS cause. Child with trauma requires a very close observation as especially because the chest and abdominal injuries can suddenly decompensate in a subject who could be conscious and apparently stable at the time of admission. I

clinician who treats children. There can be many clues in the history and examination which point toward etiology for example, presence of fever suggests infective cause, tachycardia disproportionate to tachypnea associated with hepatomegaly suggests cardiac cause, a well-child suddenly becoming tachypneic suggests foreign body, 482 a child who is a known wheezer suggests exacerbation of asthma, exposure to some allergen or drug suggests anaphylaxis, altered sensorium or CNS symptoms that started even before there was a marked respiratory distress

nvestigations

After initial clinical evaluation based on the suspected cause further investigation should be done (Table 8.3.4). vip.persianss.ir

A

M

S

able 8.3.4 Indications for investigations in respiratory distress Indication

Bedside: pulse oximetry electrocardiogram

All children with respiratory distress Suspected cardiac disease

Arterial blood gas Electrolytes, glucose, and ammonia

Gas exchange evaluation in respiratory failure, a rapidly deteriorating patient

Toxicology screen (e.g. organophosphate poisoning, salicylate, paracetamol) Carbon monoxide/methemoglobin

Only when suspected based on history (Carbon monoxide poisoning patients may be pink, and methemoglobin may be cyanosed)

Lateral neck radiograph

Useful for evaluation of upper airway obstruction

Chest radiograph

Should be performed for all children with persistent asymmetric breath sounds and for most children with significant respiratory distress

Forced expiratory or bilateral decubitus chest radiograph

Indicated for suspected foreign body aspiration

Unilateral decubitus chest radiograph

This study can distinguish a pulmonary infiltrate from an effusion

Abdominal radiographs (supine and upright, or cross-table lateral)

May demonstrate signs of abdominal obstruction or perforation

Computed tomography of the head

May demonstrate mass, injury, or hydrocephalus

Computed tomography pulmonary angiography

May indicate a pulmonary embolism

Abdominal CT

May be useful to evaluate abdominal causes of respiratory distress

Ventilation-perfusion scan (V/Q scan)

Indicated for possible pulmonary embolism

y ystem

or

r

Test

S

T

A child with suspected nasal or airway foreign body if breathing and maintaining saturation above 93%, may best be handed over to a center where anesthesia back-up is

available. This is because there is always a risk of bronchial foreign body getting dislodged and blocking airway at carina. The foreign body removal maneuvers should be used only in children who are unable to phonate. These are summarized in Table 8.3.5.

Diseases Of Respi at

Life- aving aneuvers to Relieve cute Respiratory Distress

Table 8.3.5 Life-saving maneuvers to relieve acute respiratory distress Condition

Maneuver

Comments

Complete upper airway obstruction

Needle cricothyrotomy

Temporary measure, can provide oxygenation, not ventilation

Foreign body

Back blows/chest thrusts (< 1 year of age)

Maneuvers should only be used for patients who are unable to phonate

Abdominal thrusts (≥ 1 year of age) Manual removal with finger sweep

Perform this maneuver only when foreign body is visible in the oropharynx

Laryngoscopy and removal with Magill forceps Laryngospasm

Positive pressure with a ventilation bag and tight fitting mask

Soft tissue upper airway obstruction

Head tilt/chin lift Jaw thrust

Use for patients who may have cervical spine injury

Nasopharyngeal airway

May be tolerated by a conscious patient

Oropharyngeal airway

Use only in an unconscious patient

Bag-mask ventilation

Consider upper airway obstruction if unable to ventilate with proper size equipment and technique

Endotracheal intubation

Use for patient who requires more than a few minutes of assisted ventilation. Consider for patient with complete subglottic upper airway obstruction

Tension pneumothorax

Needle thoracocentesis

Most patients will require chest tube placement following emergent decompression

Cardiac tamponade

Pericardiocentesis

Respiratory failure

483

vip.persianss.ir

I

R





Upper espiratory Tract nfections

ntroduction

Acute respiratory infections are a major cause of morbidity and mortality in children and of particular significance in developing countries like India. Outpatient attendance attributed to acute respiratory infections is as high as 20– 40% of all outpatients and 12–35% of in-patients. The overall incidence of acute respiratory infection in the under-5 may be between 3 and 8 episodes/child/year. Of the majority are upper respiratory tract infections (URTI). Upper respiratory tract infection is a loose term which includes infection of nasal cavity, throat, nasopharynx, ears and sinuses. Upper respiratory tract infections are common causes of morbidity in children.

be given 4–6 hourly. For nasal block normal saline can be instilled in nostrils every 4–6 hourly and specially before giving feeds. Child may be given warm drinks with plenty of liquids. There is no role of antibiotics, antihistaminics, local decongestive drops or steroids. Home remedies for cough and cold such as tulsi, ginger or honey may be beneficial in common cold. However, mother should be told to bring the child to hospital immediately, if there is rapid respiration, lower chest indrawing or poor feeding.

tiology

linical eatures F

C

Acute nasopharyngitis is caused by viruses. The common viruses include rhinovirus and corona viruses. The other viruses include adenoviruses, influenza, parainfluenza or respiratory syncytial viruses (RSVs). These are spread by droplet infection. Predisposing factors include chilling, sudden exposure to cold air, and overcrowding. Rhinitis could also be due to allergy.

Clinical features of common cold are due to congestion, swelling and increased secretion of nasopharyngeal mucosa. Clinical manifestations are more distressing in infant and young children. The common manifestation includes nasal discharge, initially watery than thick white to yellowish, nasal block, cough and conjunctival congestion. Nasal block causes difficulty in feeding, irritability, excessive crying and breathing from mouth. Occasionally may be complicated by secondary bacterial sinusitis and otitis media. Otitis media should be suspected in a child with no relief in crying, even after treatment for nasal block. If a course of common cold is prolonged beyond 7–10 days, then sinusitis should be considered in a school going child.

Treatment

484

Acute nasopharyngitis is caused by virus and self-limiting requires no specific treatment. For fever paracetamol can

A E

tiology

Commonly caused by viruses such as rhino, corona, influenza, parainfluenza and adenoviruses. The 10–20% of sore throats caused by bacteria. The important bacterial pathogen is group A beta hemolytic Streptococcus. Rarely Corynebacterium diphtheria may present with acute pharyngitis. C

E

Infection of nasopharynx is also called common cold. It is probably the most common infection in children. In young children 3–8 episodes of common cold may occur in 1 year.

Acute pharyngitis includes infection of pharynx and tonsils. This is also called acute tonsillopharyngitis. Most of the times, it is associated with rhinitis, sinusitis and occasionally laryngitis.

linical eatures

Children with acute pharyngitis may have fever, sore throat, pain during deglutition, nasal discharge, conjunctival congestion and discomfort in throat. There may be enlargement of tonsils and soreness in throat. Sore throat may lead to dysphagia and drooling of saliva cervical lymph nodes may be enlarged and tender. Examination may reveal grayish-white pseudomembrane specifically in infection with C. diphtheria and Epstein-barr virus. Infection due to group A streptococcus may show pus points over tonsillar surface, palate or pharyngeal wall. Viral pharyngitis is self-limiting and recovers in 5–7 days. Pharyngitis caused by group A beta hemolytic streptococcus, may lead to suppurative complication such as retropharyngeal and peritonsillar abscess. Presence of these complications may be indicated by high-grade fever severe dysphagia and bulge in the posterior wall of pharynx or around tonsils. The non-suppurative complications due to streptococcal pharyngitis include acute rheumatic fever and acute glomerulonephritis. These complications can be prevented by administration of antibiotics. It is very difficult to differentiate viral from bacterial pharyngitis. Presence of exudates/pus points on pharynx with enlarged tender cervical nodes and absence of nasal discharge suggests bacterial pharyngitis and may be used to start antibiotics.

A

cute Nasopharyngitis

cute Pharyngitis

F

I

Sushil K Kabra



t

t

Tex book of Pedia rics

8.4

vip.persianss.ir

linical eatures F

C

Acute suppurative otitis media in children is commonly caused by Streptococcus pneumoniae, Hemophilus influenzae and Moraxella catarrhalis. Very rarely, it may be caused due to Staphylococcus and Gram-negative organisms. The latter is more common in immunocompromised hosts.

Acute suppurative otitis media presents with fever, ear pain, ear discharge and restlessness. In young children, this is common cause of excessive crying. Once the tympanic membrane perforates, the child may get relief in pain but could develop pus discharge from ear. Acute suppurative otitis media may cause infection of mastoids in older children. The intracranial extension may be in form of pyogenic brain abscess. Sometimes, ASOM may cause lower motor neuron facial palsy. Children may develop middle ear effusion even after treatment with antibiotics, it is self-limiting and resolve in majority in 12 weeks’ time. If it

D

D O R p t S t

cute inusitis

In children, ethmoid and maxillary sinuses are present in infancy. Sphenoid sinuses are well-developed by 3–5 years and frontal sinus develop between 6 and 11 years of age. Infection of sinuses is common and associated with nasopharyngitis and pharyngitis.

tiology

Commonly, the viruses causing pharyngitis and naso pharyngitis are responsible for sinusitis. Sometimes, they may be invaded by bacterial pathogens. The common bacterial pathogens include Streptococcus pneumoniae, Hemophilus influenzae and Moraxella catarrhalis. Gram- 485 negative bacteria and fungi may invade paranasal sinuses in immunocompromised patients. ­

tiology

S

E

Acute suppurative otitis media (ASOM) is a common cause of morbidity in children. It is defined as inflammation of mucoperiosteal lining of the middle ear. If the duration of illness is more than 2 weeks, it is termed as chronic suppurative otitis media. Children are more prone for ASOM because, the eustachian tubes communicating throat with ears are straight and short. Acute suppurative otitis media may be one of the complications of other respiratory infections.

Acute suppurative otitis media is a bacterial infection and should be treated with antibiotics. The antibacterial useful in ASOM includes ampicillin, amoxicillin, oral cephalosporins or macrolide. Children below 2 years of age may be treated with antibiotics from the time of diagnosis. However, in children above 2 years of age with mild disease one can wait for 2–3 days for improvement in clinical symptoms without antibiotics. In severe disease as indicated by presence of high fever (explosive onset, severe otalgia and toxic appearance and high-grade fever more than 102°F) and children with mild disease in beginning but deterioration in 48–72 hours one should consider starting antibiotics. The antibiotic of choice is amoxicillin. The antibiotic is continued for 10 days to prevent recurrence and development of chronicity. If the child is not improving by 3–4 days, an alternative antibiotic like injectable third generation cephalosporin or amoxicillin clavulanic acid combination may be started. In severe cases injectable third generation cephalosporin (cefotaxime or ceftriaxone) may be started. For relief of pain, paracetamol or one of the nonsteroidal anti-inflammatory, i.e. ibuprofen may be given, round the clock. Occasionally, tympanocentesis may be required to relieve pain. There is no role of local antibiotic drugs in ASOM. For treatment of chronic otitis media (COM), it is recommended to keep the ears dry by cotton wick. One course of oral antibiotics sometimes may be useful.

edia

Treatment



M

O

cute uppurative titis S

A



The major consideration in treatment of acute pharyngitis is to prevent acute rheumatic fever. If a clinical diagnosis of streptococcal pharyngitis is made, a throat swab should be taken or rapid diagnostic test performed to demonstrate streptococci and penicillin should be administered. Penicillin can be given orally or by intramuscular route. The duration of oral penicillin is for 10–14 days. If compliance is a problem, single injection of Benzathine penicillin can be given. The other alternative antibiotics are ampicillin, amoxicillin or oral cephalosporins. If an individual is sensitive to penicillin, he or she may be treated with erythromycin. The newer macrolide antibiotics such as roxithromycin, clarithromycin and azithromycin are alternative to erythromycin.

A

Treatment

iagnosis

In a setting of URTI if a child is crying excessively, his ears should be examined by otoscope. The eardrum may be inflamed, and bulging with loss of normal anatomy with fluid in middle ear. Otoscopic examination should be part of routine examination in young children, presenting with fever without localization.

E

D

Acute pharyngitis is a clinical diagnosis. At times, it is very difficult to differentiate nasopharyngitis from pharyngitis. Diagnosis of streptococcal pharyngitis can be made with presence of exudates, enlarged tonsils and absence of nasal discharge. The diagnosis can be confirmed by throat swab culture. Now, a rapid diagnostic test based on latex agglutination is also available for diagnosis of streptococcal pharyngitis and can be carried out in office practice.

persists, these children should be sent to otolaryngologists for consideration of grommet insertion. Chronic middle ear effusion may lead to hearing impairment.

iseases f es ira ory ys em

iagnosis

vip.persianss.ir

The clinical significance of sinusitis is due to bacterial invasion. A course of amoxicillin, ampicillin or oral cephalosporin should

B









Treatment

1. Aroll B. Antibiotics for upper respiratory tract infections: an overview of Cochrane reviews. Respir Med. 2005;99(3):255-61. 2. Casey JR, Pichichero ME. Meta-analysis of short course antibiotic treatment for group a streptococcal tonsillopharyngitis. Pediatr Infect Dis J. 2005;24(10):909-17. 3. Kabra SK, Lodha R. Acute respiratory mortality. In Child Survival and Development: Recommendations of National Consultation Meeting on Child Survival and Development 20-21 Nov 2004, Held at All India Institute of Medical Sciences. New Delhi: Indian Academy of Pediatrics. 2004.pp.19-29. 4. Mirza A, Wludyka P, Chiu TT, et al. Throat culture is necessary after negative rapid antigen detection tests. Clin Pediatr (Phila). 2007;46(3):241-6. 5. Simasek M, Blandino DA. Treatment of the common cold. Am Fam Physician. 2007;75(4):515-20. 6. Tiewsoh K, Kaur J, Lodha R, et al. Management of Upper respiratory tract infection. Indian J Pediatr. 2008;75:S28-S32.



Usually prolonged course of nasopharyngitis in form of persistence of fever and nasal discharge could be due to sinusitis. If tenderness over sinuses can be demonstrated or periorbital puffiness is present in young children, a clinical diagnosis of sinusitis can be made. It can be confirmed by demonstrating opacity or mucosal thickening or fluid level in paranasal sinuses on X-ray films. Other imaging such as CT or MRI scan of sinuses may be done in immunocompromised host or complicated cases.



iagnosis

ibliography



F

C D

Common presentation of sinusitis includes non-resolving rhinitis or common cold even after 7 days, thick purulent nasal discharge, fever and tenderness over sinuses. In young infants there may be swelling around eyes. There may be headache-unilateral, bilateral, temporal or occipital depending on sinus involvement.

be given to the child. The supportive care includes normal saline in nostrils and paracetamol for fever and pain relief. There is no role of routine administration of antihistamines.



t

t

Tex book of Pedia rics

linical eatures

486

vip.persianss.ir

Croup





Diseases Of Respi at

8.5



r or S

Croup is a clinical diagnosis and does not require any investigation. Radiographs are used only if diagnosis is uncertain. The X-ray of the neck may show the typical subglottic narrowing or steeple sign on the PA view (Fig. 8.5.1). However, the steeple sign may be absent in patients with croup. Radiograph does not reflect the severity of airway obstruction.

Differential Diagnosis The differential diagnosis includes conditions that cause obstruction in the region of larynx: • piglottitis: It is very rare in India • Laryngeal foreign body: Sudden onset of choking and coughing without prodromal signs of infection • cute angioedema: Usually presents with swelling of the face and neck and other manifestations of allergic reactions • Retropharyngeal and peritonsillar abscess: A peri tonsillar abscess is often a clinical diagnosis whereas radiograph or CT scan of upper airway helps in diagnosis of retropharyngeal abscess • Bacterial tracheitis: Although, it is very rare, it is impor tant differential diagnosis as it may have fulminant course and needs antibiotic • Laryngeal diphtheria: Early symptoms of diphtheria include malaise, sore throat, anorexia, and low-grade fever. Within 2–3 days a typical gray-white membrane ­

­



The parainfluenza viruses (types 1, 2 and 3) account for 75% of cases; other viruses associated with croup include influenza A and B, adenovirus, rhinovirus, RSV and measles. Influenza A has been associated with severe laryngotracheobronchitis. Rarely, Mycoplasma pneumoniae has been isolated from children with croup. Viral infection of the upper airway causes inflammation of pharynx, larynx, trachea and bronchi. The subglottic inflammation compromises the airway in croup.

A

E

P

tiology and athogenesis

nvestigations

y ystem

Croup is the most common infectious cause of upper respiratory obstruction amongst children, with peak incidence ranging from 6 months to 6 years of age with the peak incidence at around 2 years of age. It is an acute and infectious process and is also termed as acute laryngotracheitis or laryngotracheobronchitis. It affects boys more often than girls. Traditionally, spasmodic croup and recurrent croup are considered as separate diagnoses, but, many believe that they are a part of the spectrum of a single disease. Croup occurs most commonly in winter but can occur throughout the year. Recurrences are frequent from 3–6 years of age and decrease with age. Approximately 15% of patients have a strong family history of croup. Most children with croup have uncomplicated course, managed with symptomatic treatment and have complete recovery. Only a small number of patients require hospitalization. E

In severe cases the child appears tired and exhausted due to labored breathing, has significant tachypnea and tachycardia and is restless and agitated. Some of the children may have altered sensorium, hypotonia, cyanosis and marked pallor due to severe airway obstruction and resultant hypoxia.

ntroduction

I

I

Deepak Ugra

F

Clinical eatures





The diagnosis of croup is usually clinical. It presents with barking cough, hoarse voice and high-pitched inspiratory stridor and respiratory distress. These symptoms usually follow a prodrome of mild fever, rhinorrhea and sore throat for 2–3 days. The symptoms are characteristically worse at night. The breath sounds are normal with no other adventitious sounds in chest. Occasionally, there may be wheezing. Immunity to viral infection is transient and repeat infections are common; however, in older children, symptoms are less severe. The disease can be of varying severity from mild to severe. In most children the illness is mild. The child appears happy, playful and feeds well. The stridor appears on coughing and crying. There may be mild respiratory distress. In moderate illness the stridor is audible at rest and gets worse on crying. Tachypnea, respiratory distress and sometimes tachycardia are present. The child may be irritable but is alert and comforted by parents.

487  

Figure 8.5.1 Radiograph of an airway of a child with croup, showing typical subglottic narrowing (steeple sign)

vip.persianss.ir

T

ext T

Children with mild croup do not need any specific treatment. Steam inhalation at home or saline nebulization in a clinic is often effective in humidifying the airway and providing symptomatic relief. Children with moderate to severe croup need definitive therapy (Table 8.5.1). The components of definitive therapy are: • Oxygen • Corticosteroids • Nebulized adrenaline • Intubation.

Oxygen Oxygen is the most important component of the definitive therapy of moderate to severe croup. Humidified oxygen is given to children with significant upper airway obstruction and SpO2 less than 92%.

Corticosteroids Steroids are useful in moderate to severe croup. Nebulized Budesonide 2 mg 12 hourly for up to 48 hours shows improvement in croup symptoms. The onset of action is seen within 30 minutes. Oral prednisolone (1–2 mg/kg),

Nebulized drenaline A

M

reatment

oral or IM dexamethasone (0.15–0.3 mg/kg) are equally efficacious. Steroid therapy causes significant decrease in the need for nebulized adrenaline, need and duration of intubation and also average stay in hospital.

It should be given immediately to children with severe croup. The doses are 0.5 mL/kg of body weight of 1:1,000 dilutions to a maximum of 5 mL. The onset of action is seen within 30 minutes and the effect lasts for about 2 hours. In case of persistent stridor the dose can be repeated every 2–4 hours. I

P

d

•

boo

o

•

S

r

c

k f e iat i s

•

on tonsils and/or soft palate is seen on pharyngeal examination. The membrane is adherent to the tissue, and forcible attempts to remove it cause bleeding easles croup: It almost always has full manifestations of systemic disease and the course may be fulminant Bronchial asthma: A croupy cough may be an early sign of asthma ubglottic stenosis: It presents from early infancy and is not associated with prodromal symptoms.

ntubation

Children with signs of impending respiratory failure should be intubated for a better control on airway. An endotracheal tube (ET) of 0.5 mm smaller than expected for the child’s size is selected. Intubation is maintained till an air leak develops to a maximum of 5 days.

Outcome Croup is a common infectious condition amongst small children. Most children have mild symptoms and are treated at home and the illness resolves in 3–4 days. It is observed that if the child’s symptoms are minimal at discharge, return within 24 hours is unlikely. Only about 5% of children discharged from the emergency department after corticosteroid therapy need to return because of worsening of symptoms. Intubation is rarely required. In Canada, of all children with croup, about 4% have been estimated to require hospitalization, and intubation was required for only 1 of the 170 hospitalized children or 1 in 4,500 of all children with croup.

Table 8.5.1 Evaluation and management of children with croup  

Croup severity (Westley score) Mild (≤2)

Moderate (3–7)

Severe (≥8)

Barking cough, hoarseness; no stridor, no or minimal chest wall retractions at rest

Stridor and chest wall retractions at rest; no agitation

Stridor, sternal contractions at rest, accompanied by agitation or fatigue

Decongestants, cough Not recommended suppressants, antibiotics

Not recommended

Not recommended

Humidification

Not proven beneficial

Not effective

Not effective

Corticosteroids

Dexamethasone (0.6 mg/kg, 1 dose PO)

Dexamethasone (0.6 mg/kg, 1 dose PO or IM)

Dexamethasone (0.6 mg/kg, 1 dose PO or IM)

Nebulized epinephrine

Not recommended

Not recommended

Nebulized racemic epinephrine (2.25%, 0.5 mL in 2.5 mL of saline or l-epinephrine (1:1,000 dilution in 5 mL of saline)

Discharge home

Discharge to home if no stridor, no retractions at rest. If no improvement in 4 hours, consider hospitalization

Observe 2 hours Good response: no recurrence, no stridor and no retractions at rest. Discharge to home possible Poor response: stridor, retractions at rest after 2 epinephrine doses. Hospitalize

  Therapy

Disposition

488

Source: Mandell, Douglas and Bennett’s Principles and Practice of Infectious Disease. 5th edition.

vip.persianss.ir

neumonia

P



Diseases Of Respi at

8.6

E

pidemiology

r or S

tiology

Definition Pneumonia is defined as an inflammatory process involving lung parenchyma usually due to microorganisms. It is





It depends on various factors like age, immune status, underlying comorbidity and various risk factors as shown in Flow chart 8.6.1. The causative agents may be viral, bacterial or atypical pathogens. They are usually age specific as shown in Table 8.6.1. Most common bacterial pathogens are Streptococcus pneumoniae and Hemophilus influenzae which together are responsible for 60–70% of total pneumonia cases, followed by viruses which account for 30–35% of pneumonia cases. Mycoplasma pneumoniae and chlamydia are most common causes of “atypical pneumonia” in school-going





­

Approximately 150 million episodes of childhood pneu monia are reported every year from the world out of which 95% are from developing countries. Fifteen countries account for nearly 75% and 6 countries including India account for 50%. India alone bears the brunt of 25% disease burden. Out of the 7.6 million under-5 childhood mortality world over 16%, i.e. 12 million deaths are due to pneumonia. More than 90% of deaths due to pneumonia among young children occur in 68 poor nations, mostly from Africa and Asia. In India the disease burden is huge. 45 million episodes are estimated annually with 6.6 million hospitalizations, which contribute to 24% national disease burden and 0.37 million deaths annually.

y ystem

Pneumonia probably is one of the oldest diseases, as old as antiquity known to human kind and has always remained a subject of challenge to medical science, despite extensive research. Pneumonia is number one cause of under-5 childhood mortality across the globe particularly in developing countries. Unfortunately, over the years the mortality remained almost the same and hence it is also been called as “forgotten killer” or “silent killer”.

referred as “Pneumonitis” when the cause is non-infective. Pneumonia’s are mainly classified as: • Community acquired—pneumonia acquired outside the hospital environment in a previously healthy immune competent subject. The patient should not have been hospitalized within 14 days prior to the onset of symptoms. • Nosocomial pneumonia—pneumonia acquired within hospital setting more than 48 hours after hospitalization [hospital-acquired pneumonia (HAP)] or more than 48 to 72 hours after endotracheal intubation [ventilatorassociated pneumonia (VAP)]. This classification does not include “Recurrent Pneumonia” which is defined as two episodes of pneumonia in 1 year or 3 episodes in any time frame, and “Aspiration Pneumonia” which occurs due to aspiration of foreign materials in the lower airways.

ntroduction

E

I

Rohit C Agrawal

 

Flow chart 8.6.1 Predisposing factors for community acquired pneumonia (CAP)

(Source: Study by Burman et al. Epidemiology of ARI in children of developing countries. Rev Inf Dis. 1991)

489

vip.persianss.ir

P o oo

extb

Streptococcus pneumoniae Viruses (35%) Hemophilus influenzae Staphylococcus Mycoplasma pneumoniae

>5 years

Streptococcus pneumoniae Mycoplasma pneumoniae (24–30%) Viruses Staphylococcus Staphylococcus pyogenes

T

3 months to 5 years

P

children older than 5 years of age accounting for 11–30% cases. Legionella is another rare and frequent cause of atypical pneumonia. Significant proportion of pneumonia is due to mixed infection (8–40%) and in 20–60% of cases pathogens are not identified.

athogenesis

linical eatures F

C

Pneumonia is usually preceded by respiratory viral infection which disturbs the defense mechanism of the lungs and also disrupts the normal epithelial layer of respiratory tract and as a result there is dysfunction of ciliary brush border clearing mechanism. There is inhibition of phagocytosis by alveolar macrophages. Thus, bacteria and other organisms invade the lung parenchyma and produce a pneumonic lesion. The invasion could be either direct spread from nasopharyngeal tract by respiratory droplet infection or could be by invasion through hematogenous dissemination within the lung parenchyma. When the spread is hematogenous it is called “invasive or bacteremic pneumonia” and when the spread is direct it is called “non-bacteremic pneumonia”. However, the pathogenesis is still ill understood till date.

Diagnosis of pneumonia is essentially clinical and seldom requires lab support. Absence of past history of recurrent cough and presence of fever with fast breathing is a hallmark presentation in clinical diagnosis of pneumonia. It should always be remembered that there are no definite differentiating markers between viral, bacterial and atypical pneumonia. However, there are certain clinical clues which can help to nail down on etiological diagnosis (Table 8.6.3).

Characteristics of Viral Pneumonia • • • • •

Acute – sudden onset Younger age Preceding upper respiratory catarrh Wheeze with crackles Clinical evidence of hyperinflation with scattered exudates on radiology due to segmental atelectasis. For optimum antimicrobial management of pneumonia it is prudent to differentiate between bacterial, viral and atypical pneumonia clinically, as it is often very difficult to isolate the offending pathogen (Table 8.6.4).

Laboratory Diagnosis Acute phase reactants, like CBC, CRP, ESR, have poor sensitivity and specificity. They do not distinguish between viral and bacterial etiology, nor they help in making decision of antibiotic choice; however, they may be useful tools for monitoring the course of the disease. Radiology is not routinely required in non-severe pneumonia to confirm the diagnosis. At times it may not correlate with the clinical signs; there is also wide variation in the interpretation by radiologists. Moreover, reliability in predicting the etiology is poor. However, CXR may be indicated in very severe disease, ambiguous picture, no Table 8.6.2 The WHO grading of pneumonia Pneumonia

Fever less than 38.5°C, no feeding difficulties, no dehydration, cough and tachypnea

Severe pneumonia

High-grade fever more than 39°C, difficulty in feeding, tachypnea, respiratory distress with intercostal retraction (ICR) or subcostal retraction (SCR), dehydration, grunt, bronchial breath sounds on auscultation with or without crackles, SpO2 > 92 at room air, radiological opacity on CXR +/–

Very severe pneumonia

Inability to feed, altered sensorium, intermittent apneic spells, cyanosis, excessive diaphoresis, narrow pulse pressure, acidemia, SpO2 < 92 at room air



Constellation of symptoms triad of fever, cough, rapid breathing and or difficult breathing is classical clinical manifestations of pneumonia. Diagnosis of pneumonia is essentially clinical. Tachypnea is single most sensitive and specific sign to diagnose pneumonia, as sensitive as 66–88% of auscultation (Table 8.3.1 for grading of tachypnea.) Clinical features may differ from neonate to older child. In the neonate there could be absence of cough and fever, and common presenting features are excessive irritability or lethargy, difficulty in feeding, intermittent apneic 490 spells, cyanosis +/–, progressive air hunger, rapid clinical deterioration with or without evidence of sepsis. In severely

Diagnosis

 

Gram-negative Enterobacteriaceae Enterococci Chlamydia trachomatis Group B streptococci Hemophilus influenzae Streptococcus pneumoniae Listeria monocytogenes



0–3 months



 

r

Etiology

d

Age group

malnourished children, the breathing efforts will be poor. The WHO has graded pneumonia as given in Table 8.6.2 based on symptoms. Assessment and grading of severity is most important for optimum and successful management.



k f e iat ics

Table 8.6.1 Etiology of pneumonia – relation to age

vip.persianss.ir

Diseases Of Respi at

 

Table 8.6.3 Clinical clues which help in diagnosis of etiological diagnosis of pneumonia Organisms (apart from usual ones)

Pyoderma, measles, pneumatoceles

Staphylococcus

Human immunodeficiency virus (HIV)

Pneumocystis

Neutropenia

Gram-negative, Aspergillus

CF

Pseudomonas, Staphylococcus

Severe protein-energy malnutrition (PEM)

Gram-negative, Staphylococcus

Preceding coryza, wheeze

Viral

Young afebrile infant with neonatal conjunctivitis

Chlamydia

Multisystem involvement (rash, anemia, hepatitis, encephalitis)

Mycoplasma

Marked leukocytosis

Bacterial

Acute otitis media

Streptococcus pneumoniae/Hemophilus influenzae

S

y ystem

or

r

Predisposing factors

 

Table 8.6.4 Differential diagnosis between typical and atypical pneumonia Features

Typical pneumonia

Atypical pneumonia

Age

More common in young infants, children and also in older children

School going children, adolescents and adults

Onset

Acute/sudden

Gradual/insidious

Facies

Toxic

Well

Rigors

Shaking chills

Chilliness

Wheeze

Rare or nil

Common

Cough

Productive

Non-productive/paroxysmal

Sputum

Purulent/bloody

Mucoid

Temperature

High 102–104°F

Mild-Moderate 15,000 /cu mm; shift to left

>15,000 /cu mm; no shift

Chest radiography

Defined density

Non-defined infiltrates

Differential Diagnosis Though symptom complex of fever, cough and rapid/difficult breathing is classical presentation of pneumonia; it is prudent to differentiate pneumonia from other masqueraders which may mimic with same symptomatology.



M

anagement

The mainstays of management are antibiotics and supportive treatment. It is imperative to understand that all pneumonias deserve antibiotics as differentiation between viral and bacterial is difficult—“Empirical antibiotics are prudent and rational in pneumonia”. Non-severe pneumonia above the age of 3 months can be managed at domiciliary level with oral antibiotics.







improvement/worsening more than 48–72 hours of therapy, suspected complications and known immunocompromised child (Figs 8.6.1A to C). Microbiology—sputum culture or blood culture though may be more specific, but the yield is very poor (10–15%). There is also a risk of growing normal nasopharyngeal flora. Serology—serology, urinary antigens, rapid antigen detection test (RADT) and cold agglutinins for mycoplasma are not easily available, expensive with time lagging and have poor sensitivity. Invasive procedures, like bronchoscopy, BAL and lung aspiration, have high sensitivity and specificity; however, they are too invasive to be advised in office practice. Pulse oximetry is a mandatory tool for monitoring the course of the disease in all the hospitalized children.

491

vip.persianss.ir

o

P

d

r

k f e iat ics oo





B

X-Rays – Bacterial lobar consolidation

C

Bronchopneumonia

Interstitial pneumonia



extb T

a

 

Figures 8.6.1A to C Radiologic findings in bacterial lobar consolidation, bronchopneumonia and interstitial pneumonia

 

Table 8.6.5 Choice of antibiotics Age

anagement

All non-severe pneumonias more than 3 months of age, when the child is not toxic, accepting oral feeds, not dehydrated, maintaining normal saturation and fever less than 38.5ºC can be managed at domiciliary level with supportive care like maintenance of hydration, nutrition and antipyretics and adjuvants if needed. The choice of oral antibiotic is as shown in Table 8.6.5. First line oral antibiotics should be given minimum for 5 days and second line for 7 days. Child should be followed-up after 48 hours and if there is clinical improvement the child should be continued with the same management. If the condition clinically deteriorates after 48 hours, one should revise the diagnosis, look for associated complication and co-morbidities, changing the antibiotic to second line and if need be, the child should be hospitalized (Table 8.6.6).



Macrolideb/Co-amoxiclav/ Cefuroxime

Amoxicillina

Standard doses: 40–45 mg/kg/day in two or three divided doses Erythromycin: 30–40 mg/kg/day in three divided doses; Clarithromycin: 15 mg/kg/day in two divided doses; Azithromycin: 10 mg/kg/day in OD dose

 

Table 8.6.6 Indications for hospitalization •  •  •  •  •  •  •  •  •  • 

Infants less than 3 months Severe malnutrition More than two risk factors Comorbidities Associated complications Respiratory rate more than 70/min in infants and more than 50/min in older children Respiratory distress – grunting, alae nasi flare, ICR or SCR Cyanosis or SpO2 less than 92% in room air Poor oral intake/dehydration Inappropriate observation or supervision at home

divided doses) and should never be cefixime as it has no activity against Pneumococcus and poor activity against community pathogens responsible for pneumonia. Despite rational choice of antibiotics in right dose and for optimal duration, if there is failure in clinical improvement, one needs to:

M

> 5 years

b

anagement

Management comprises of specific antimicrobial along with supportive care of nutrition, hydration, oxygen if needed, antipyretics (paracetamol) and bronchodilators along with chest physiotherapy if needed (Table 8.6.7). In case of methicillin-sensitive Staphylococcus aureus (MSSA), the duration should be for 2 weeks and in case of Methicillin-resistant Staphylococcus aureus (MRSA), it should be 4–6 weeks. The duration for IV antibiotics should be for 5–7 days in uncomplicated cases, however, switch over to oral antibiotics may be considered if accepted orally. The switch over therapy for injections of third generation cephalosporins should be either cefpodoxime (10 mg/kg/ day in two divided doses) or cefdinir (15 mg/kg/day in two

Second line Co-amoxiclav/Cefuroxime/ Chloramphenicol

a

I

npatient

First line

3 months to 5 years Amoxicillina



Outpatient

M

However, any pneumonia below the age of 3 months should be hospitalized and treated with parenteral antibiotics. The choice of antibiotics though empirical should be determined by age, severity, pre-disposing conditions if any and local epidemiology and drug resistance pattern.

• •

•

Check the diagnosis and rule out foreign body, aspiration pneumonia and interstitial lung disease Look for underlying comorbidity like lung abscess, empyema, bronchiectasis, left to right shunts, GERD asthma, CF and ciliary dyskinesia Evaluate immunosuppression in the host like HIV and hypogammaglobulinemia

492

vip.persianss.ir

Diseases Of Respi at

 

Table 8.6.7 Specific antimicrobial therapy in pneumonia Age

First line

Second line

5 years

Ampicillin/Penicillin G Co-amoxiclav/Macrolide (if mycoplasma suspected)

Ceftriaxoneb/Cefotaximec and Macrolides

Suspected staph

Cefuroximed or Co-amoxiclav or IV 3rd generation Cephalosporins + Cloxacillin

Ceftriaxoneb/Cefotaximec and Vancomycin/ Teicoplanin/Linezolid

e

r

b

y ystem

or

c

Co-amoxiclav: 30–40 mg/kg/day Ceftriaxone: 50–100 mg/kg/day c Cefotaxime: 100–200 mg/kg/day d Cefuroxime: 20–30 mg/kg/day e Aminoglycosides: 15 mg/kg/day in single or two divided doses

S

a

b

20% cases of pneumonia receive proper and adequate antibiotics, which is the main reason for high infant mortality due to pneumonias particularly in developing countries.

P

Test phagocytic dysfunctions for chronic granulomatous disease (CGD) • Look for drug resistance, particularly if child is from day care center, has received multiple courses of betalactams and corticosteroids • Search for the possibility of polymicrobial etiology. There is no need to chase for follow-up X-rays since total radiological resolution may take 4–12 weeks’ time depending on offending organisms.

revention

C



•

omplicationsv

P

These include empyema, pneumothorax, bronchogenic dissemination, septicemia, osteomyelitis, multiple systemic abscesses, septic arthritis and meningitis.

rognosis

Prognosis is fairly good provided there is appropriate recognition and proper referral by the health care provider and early initiation of antibiotics. Unfortunately only 15–

A multifaceted approach is needed to prevent and control childhood pneumonia. These include: • • • • •

Exclusive breast feeding for first 6 months of life Weaning to solid foods after 6 months of age, preferably with home-made foods Avoidance of risk factors like overcrowded environment, exposure to pollution and bottle feeding Protection from malnutrition and supplementation of vitamin A and D Optimum immunization with DPT, measles, Hemophilus influenzae b (Hib), pneumococcal vaccines at appropriate ages.

493

vip.persianss.ir

B



ronchiolitis

implicated occasionally in children with lower respiratory tract infection (LRTI) and wheezing.

isk actors F

R

Bronchiolitis is a clinical syndrome characterized by the acute onset of respiratory symptoms in a child younger than 2 years of age. Typically, the initial symptoms of upper respiratory tract viral infection, such as fever and coryza, progress within 4–6 days to include evidence of lower respiratory tract involvement with the onset of cough and wheezing.

E

­

­



In bronchiolitis, an increased risk for hospitalization has been seen among infants attending day care, in those exposed to passive smoking and overcrowding in the household. Environmental and genetic factors may also contribute to the severity of disease. High-risk group includes age less than 3 months of age, premature delivery, low birth weight, congenital heart disease, chronic lung disease (CF, recurrent aspiration pneumonitis, bronchopulmonary dysplasia (BPD), congenital lung malformations, trachea-esophageal fistula, and neurogenic disorders interfering with pulmonary toilet), immunodeficiency and malnourished infants. The high-risk groups are vulnerable for rapid deterio ration and more severe disease. Pulmonary patch (consoli dation or atelectasis) on radiograph, hypoxia, repeat episodes of apnea, extreme tachypnea at admission are at higher risk of admission to the intensive care unit (ICU) and may need mechanical ventilation.

tiology

Bronchiolitis is usually a result of a viral respiratory tract infection. Respiratory syncytial virus is the most common underlying viral infection. Other viral pathogens such as 494 influenza, parainfluenza, adenovirus, coronaviruses and rhinoviruses can also cause bronchiolitis. Mycoplasma is also

Respiratory syncytial virus infection, results in loss of epithelial cilia and sloughing of epithelial cells in the airways. This leads to collection of desquamated airway epithelial cells, polymorphonuclear cells and lymphocytes within the airway. There may also be cellular infiltration and airway mucosal edema with very little or no alveolar infiltration. In acute bronchiolitis, sloughed epithelial cells, neutrophils, and lymphocytes appear to be the major contributors to airway obstruction. The complete plugging of some airways and partial plugging of others may lead to localized atelectasis of some units of lung parenchyma and over distention of other units. These results in ventilationperfusion mismatch causing hypoxemia, which is generally relieved by the administration of oxygen.

linical eatures F

efinition

Pathology

Clinical features are quite variable. Initially, they present like any other viral URTI with cough or cold with or without fever. Later disturbing cough, tachypnea, respiratory distress and poor feeding may develop depending on the severity of illness. Fever is present in almost 50% of infants. In patients with adenovirus or influenza associated bronchiolitis, fever is often higher than 39°C. On examination, infants typically may have tachypnea, respiratory distress, audible wheezing, rales or rhonchi, crackles, poor air movement and the expiratory phase is usually prolonged. Other concomitant findings like conjunctivitis, rhinitis and otitis media may be present. Once hypoxia sets in, there can be lethargy, seizure and death.

Grading of ronchiolitis B

Bronchiolitis is a common viral respiratory illness in children. It accounts for a substantial portion of the pediatric burden of illness all over the world; it is the most common and serious lower respiratory tract syndrome that results in hospital admission among infants with associated considerable morbidity. It is generally a selflimiting condition and is most commonly associated with RSV infection. Babies with chronic lung disease are at risk for more severe RSV-associated illness. Bronchiolitis occurs most frequently among children younger than 12 months of age. Most cases occur between late autumn and early spring, with sporadic cases any time.

C

ntroduction

Based on the ability to feed, the respiratory effort and oxygen saturation observed at admission each infant’s condition can be clinically graded as mild, moderate, and severe bronchiolitis (Table 8.7.1). I

I

A Balachandran

D

t

t

Tex book of Pedia rics

8.7

nvestigations

Bronchiolitis is a clinical diagnosis. The specific and supportive investigations which can be done in bronchiolitis include: • Nasopharyngeal aspirate for RSV and viral culture • Full blood count • Electrolytes, to look for evidence of syndrome of inappropriate antidiuretic hormone secretion (SIADH), especially when intravenous (IV) fluids are needed

vip.persianss.ir

D

Ability to feed normally

Appear short of breath during feeds

May be reluctant or unable to feed

Respiratory effort

Little or no respiratory distress

Moderate distress with some chest wall retractions and nasal flaring

Severe distress with marked chest wall retractions, nasal flaring and grunting May have frequent or prolonged apneic episodes

Oxygen saturation

Saturations SaO2 more than 92%

Saturations less than 92%, correctable with oxygen

Saturations less than 92%, may or may not be correctable with oxygen

O

Ability to feed

R

Severe

t

Moderate

p

 

Mild

iseases f es ira ory ys em

Table 8.7.1 Grading of bronchiolitis

D

ifferential iagnosis

anagement

Supportive care is the mainstay of therapy for infants with bronchiolitis. Children with mild bronchiolitis can be managed on outpatient basis. They need to be followed and reviewed earlier if there is clinical worsening. Infants in high-risk group are better hospitalized and managed even if they are graded as mild in view of their propensity to deteriorate rapidly. Whereas moderate and severe cases have to be definitely admitted and monitored. Considerable, unexplained variation exists in inpatient management of bronchiolitis (Flow chart 8.7.1).

Noncontroversial ssues I

•

Blood culture if temperature more than 38.5°C Arterial blood gas analysis to look for CO2 retention when there is evidence of respiratory failure The CXR need not be done routinely and is indicated only in children with severe respiratory distress or when there is diagnostic dilemma. The radiographic findings of bronchiolitis include hyperinflation, patchy infiltrates that are typically migratory and attributable to postobstructive atelectasis, and peribronchial cuffing. Because bronchiolitis is not a disease of the alveolar spaces, a secondary bacterial pneumonitis should be suspected if a true alveolar infiltrate is seen on chest radiograph. D

• •

(* Source: Respiratory Tract Infection – Group Education Module, IAP Action Plan, 2006.)

M

 

t

S

 

Flow chart 8.7.1 Management algorithm*

Congenital anomalies, such as vascular ring, congenital heart disease, gastroesophageal reflux, aspiration pneumonia or foreign body aspiration can mimic the symptoms of bronchiolitis.

Oxygen The drug of choice in bronchiolitis is oxygen. Oxygen is used when the SaO2 is less than 94% or in a combination 495 of clinically significant respiratory distress, a respiratory

vip.persianss.ir

If infant tolerates well, oral feeds are continued as is the case with mild to moderate severity. Intravenous fluids are needed to maintain hydration in moderate-to-severe or severe respiratory distress (marked retractions, nasal flaring), marked tachypnea (> 60/min), coughing and choking with feeds, vomiting, decreased intake, apneic episodes and tiring during feeds. Normal general maintenance fluids with normal maintenance volumes are used with N/2 or N/4 dextrose saline.

Nasogastric Feeding Nasogastric (NG) feeding is avoided during acute phase and is reserved for recovery phase. During acute phase the NG tube blocks one nostril and increases upper airway resistance which in turn further increases the work of breathing.

Symptomatic Management

ontroversial ssues I

C

Symptomatic management like antipyretics is needed to relieve fever.

Bronchodilators

Sedation should be avoided in an irritable child as this may be a sign of hypoxia and sedation further compromises the respiratory drive of the child.

Antibiotics There is no role for routine or prophylactic antibiotics in bronchiolitis. Since secondary bacterial infection of the lower respiratory tract are unusual in children with bronchiolitis.

Aerosolized Ribavirin Respiratory syncytial virus is the most common cause of bronchiolitis but specific antiviral therapy for symptomatic infants has been of limited value. Aerosolized ribavirin, used to treat mild to moderately ill infants with laboratory confirmed RSV bronchiolitis neither prevents the need for mechanical ventilation nor reduces the length of hospital stay.

Noninvasive Ventilation Continuous positive airway pressure (CPAP) may potentially benefit infants with bronchiolitis, by stenting open the smaller airways during all phases of respiration, preventing air trapping and obstructive disease and by serving as a constant or ipratropium stimulus in infants with a propensity to experience apnea.

Role of Steroids There is no role for systemic (parenteral/oral) or inhaled steroids or ipratropium bromide in the management of bronchiolitis.

Intensive Care Unit Management It will be needed in the following category if there is: • Progression to severe respiratory distress, especially in high-risk group • Any significant apneic episodes (more than 15 seconds or associated with desaturation) or frequent recurrent brief episodes • Persistent desaturation despite oxygen where ABG shows evidence of respiratory failure, i.e. PO2 less than 80 mm Hg; PCO2 more than 50 mm Hg; pH less than 7.25.

ther Nonstandard Therapies

Anti-Respiratory Syncytial Virus preparation The use of intravenous immunoglobulin with a neutra lizing activity against RSV [RSV-immune globulin intra venous (IGIV)] or RSV-specific humanized monoclonal antibody (palivizumab) has failed to improve outcomes in infants with or without risk factors, hospitalized with RSV infection. ­



The role of bronchodilators (salbutamol and epinephrine) in acute bronchiolitis is controversial. Though there is no absolute contraindication, bronchodilators neither treats hypoxemia nor affects the duration or progress of bronchiolitis. Inhaled beta agonist therapy when used should be on a case-by-case basis, by critically evaluating its effectiveness in the individual patient. They might be of benefit in intermediate conditions like early wheezers or children with atopy. Since mucosal edema is an important component of airway obstruction in infants with bronchiolitis, a logical approach to therapy might be to use a combined (alpha-adrenergic and beta-adrenergic agonist, such as epinephrine (0.3 mL/kg of 1:1,000 solution). Till date there is no definitive evidence to support the use of nebulized epinephrine in all children with moderate or severe bronchiolitis. When the effect of epinephrine was compared with placebo, the improvement in respiratory symptoms across studies has been inconsistent and potentially shortlived. It may therefore be appropriate, for clinicians to use nebulized epinephrine as a potential rescue medication for patients who are to be admitted.

Sedation

­

Fluid Therapy

bronchiolitis. Several trials have indicated the potential benefit of hypertonic saline (3% NaCl) in acute bronchiolitis with conflicting results.

O

t

t

Tex book of Pedia rics

rate above 60/min, and difficulty in feeding. The aim of humidified oxygen supplementation is to maintain oxygen saturation above 95% using suitable device, preferably in a non-threatening fashion. During recovery, SaO2 of 90–92% in room air is acceptable if baby is feeding well and not distressed except in children with chronic lung disease infants.

Hypertonic Saline

496 Hypertonic saline has the potential to reduce airway edema and mucus plugging the predominant feature of acute

vip.persianss.ir

Prognosis The need for supplemental oxygen (based on SaO2 in room air) at admission is highly predictive of the length of the hospital stay. The case fatality rate for bronchiolitis is highest among infants less than 3 months age.

Prevention Respiratory syncytial virus is highly contagious and spreads via inhalation of small particle aerosols or direct transfer via hand-to-hand contact. Respiratory syncytial virus crossinfection is common, but is largely preventable by: • Simple hand washing by nursing, medical, other staff and parents • Isolation of RSV infected cases will minimize this problem.

D R S

1. Balachandran A, Gowrishankar NC. Bronchiolitis. In: Subramanyam L, Shivbalan So, Gowrishankar NC, Vijayasekaran D, Balachandran A (Eds). Essentials of Pediatric Pulmonology, 3rd edition. New Delhi: Jaypee Brothers Medical Publishers (P) Ltd. 2008.pp.91-5. 2. Christakis DA, Cowan CA, Garrison MM, et al. Variation in inpatient diagnostic testing and management of bronchiolitis. Pediatrics. 2005;115(4):878-84. 3. Coffin SE. Bronchiolitis: in-patient focus. Pediatr Clin North Am. 2005;52(4):1047-57. 4. Dayan SP, Roskind GC, Levine AD, et al. Controversies in the management of children with bronchiolitis. Clin Pediatr Emerg Med. 2004;5:41-53. 5. Grimaldi M, Gouyon B, Michaut F, et al. Severe respiratory syncytial virus bronchiolitis: epidemiologic variations associated with the initiation of palivizumab in severely premature infants with bronchopulmonary dysplasia. Pediatr Infect Dis J. 2004;23(12):1081-5. 6. King VJ, Viswanathan M, Bordley WC, et al. Pharmacologic treatment of bronchiolitis in infants and children: a systematic review. Arch Pediatr Adolesc Med. 2004;158(2):127-37. 7. Respiratory Tract Infection – Group Education Module. IAP Consensus Protocol for the Management of Respiratory Tract Infection for Children. IAP Action Plan, 2006. 8. Wainwright C, Altamirano L, Cheney M, et al. A Multicenter Randomized Double-blind controlled trial of nebulized epinephrine in infants with acute bronchiolitis. N Engl J Med. 2003;349(1):27-35.

t

Complication rates are higher in former premature infants, infants with congenital heart disease and infants with other congenital abnormalities compared to infants without risk factors. Serious respiratory complications, includes respiratory failure, apnea and pneumothorax. Others include secondary infection, cardiovascular involvement and electrolyte imbalance.

ibliography



omplications



C

B

t

Infant is considered ready for discharge if the child had not received supplemental oxygen for 10 hours, had minimal or no chest recession, and is feeding adequately, without the need for intravenous fluids.

Generally a self-limiting condition Commonly of viral etiology Diagnosis is clinical Oxygen (humidified) is the drug of choice Little support for use of beta-2 agonist and steroids

p

C

ischarge riteria

ey Points

O

K

• • • • •

iseases f es ira ory ys em

It is a mixture of helium and oxygen (80:20%). It can flow through airways with less turbulence and resistance than oxygen. Only very small benefit is observed in a limited group of children who were administered heliox, hence not recommended for routine use. D

Palivizumab (RSV Ig) prophylaxis in BPD may be beneficial and has to be decided on case by case basis.

Heliox

497

vip.persianss.ir

E



mpyema

tiopathogenesis

498

linical eatures F

C

Staphylococcus aureus, Streptococcus pneumoniae and Streptococcus pyogenes are the organisms most commonly implicated in empyema thoracis. While S. pneumoniae is the most common organism implicated in most western and developed countries, the prevalence of Hemophilus influenzae pneumonia with empyema has decreased following Hib vaccination. In India, S. aureus continues to be the commonest organism. S. pneumoniae, H. influenzae and gram-negative bacilli form rest of the burden. Frank tuberculous empyema is very uncommon.

Clinical signs vary depending on the age of the patient, stage of the effusion and type of prior antibiotic therapy.



iagnosis F

hest kiagram - rontal Views

Chest skiagram - frontal views form the most important and primary investigation for patients with pleural disease. There is no role for a routine lateral or decubitus views. The earliest sign to be seen is the obliteration of the costophrenic angle. White opacity of the lateral part or whole of hemithorax is usually seen. Skiagrams, however, cannot differentiate empyema from a parapneumonic effusion. Scoliosis may also be seen.

onography of hest C

E

The reported global incidence of parapneumonic effusion and empyema is 3.3 per 10,000 children but this varies with different populace and economic level. Some of the developed nations are showing an increased prevalence lately. It is more common in infants and young children under 5 years of age.

S

B

isease urden

D

Involvement of the pleural space with pulmonary infections has been recognized since ancient times. Bacterial pneumonia with associated pleural empyema is the most common cause of pleural effusion found in the pediatric population. They need to be understood differently from parapneumonic effusions which are exudative thin effusions which can be seen in a significant proportion of those admitted with a severe and complicated pneumonia. Parapneumonic effusion and empyema lie on a continuum: the stage of the effusion is best assessed using chest ultrasound. While simple effusions have free flowing pleural fluid; more complex effusions have fibrin strands and loculations, whereas an empyema has multiple loculations and often a thick pleural rind. Importantly, a clinician must know that while most parapneumonic effusions resolve with adequate and appropriate antimicrobial therapy but antibiotics alone are almost never sufficient to treat empyema (frank pus in the pleural cavity).

A high index of suspicion and appreciation of factors that predispose to development of empyema facilitates its recognition. Children usually present with a severe pneumonia, or a pneumonia that does not respond well to initial therapy. Lack of response to antibiotics after 48 hours of treatment should raise the suspicion of an effusion. However, some may also present with a fever without clear focus, or with chest or abdominal pain. Usual features of pneumonia like fever, tachypnea, hypoxia, respiratory distress are present. The patient often has rapid and shallow breathing due to pleural pain and the child may prefer to lie on the affected side. Few children may also be septicemic, dehydrated and/or in respiratory failure. Fever can be absent in immunocompromised or debilitated patients. On physical examination, chest-asymmetry with swelling/splinting of the chest on the affected side, decreased chest expansion, decreased or bronchial breath sounds, dullness to percussion and contralateral mediastinal shift are common signs. Few children can have pleural friction rub, bronchophony or egophony above pleural effusion. Oxygen saturation levels below 92% may indicate severe disease. Some children may have other stigmata of staphylococcal infection like skin pustules or boils, infected scabies, pyomyositis, septic arthritis, pyopericardium, etc.

C

ntroduction

S

I

Varinder Singh

D

t

t

Tex book of Pedia rics

8.8

It is a very useful tool for diagnosis, guidance of thoracocentesis, or pleural catheter placement. It is especially helpful when the radiograph shows a white out. Sonography can distinguish solid from liquid pleural abnormalities with much higher accuracy compared to skiagrams. Sonography gives valuable information regarding size of effusion, presence of adhesions or loculations and the echogenicity of the pleural fluid. Sonographic appearance of pleural fluid varies according to the stage of effusion; ranging from an anechoic completely echo-free or sonolucent parapneumonic effu-

vip.persianss.ir

A

Pus/ spirate

M

Most important investigation is the aspiration of the pleural fluid to confirm that it is pus. Fluid/pus aspirated should be sent for gram stain and culture to guide therapy. In case non-pus fluid is aspirated, it should also be subjected to cytological and biochemical examination to differentiate between transudative and exudative effusions.

anagement

upportive

Oxygen (in cases with SaO2 < 92%) and fluid therapy, if child is dehydrated or unable to drink should be provided. Adequate analgesia should be given especially if the child has a chest tube. It helps prevent scoliosis and aids mobilization.

Chest tube can be placed following marking done by ultrasound. If the initial aspiration fails, the drainage may be done under ultrasound. Conscious sedation can be used for the chest tube insertion with all the personnel and equipment necessary for resuscitation.

O R p t S

Antipyretics

A

Antipyretics should be judiciously used as fever is one of the parameters used to assess response.

ntimicrobial Therapy

The choice of empirical antibiotics is based on the likeliest causative organisms. Anti-staphylococcal penicillin (cloxacillin 100–200 mg/kg/day) along with 3rd generation cephalosporin like ceftriaxone may be used as first line drug. Aminoglycoside may be used instead of ceftriaxone. Coamoxiclav is another good alternative drug. The preferred route of therapy is intravenous. R

D

rainage

M

• • • • •

S

Oxygen

All children with empyema should be admitted to hospital and given intravenous antibiotics and tubethoracotomy should be done. Small collections (< 10 mm in a child below 2 years and < 20 mm in a child above 2 years) with no significant symptoms or distress may respond to antibiotic therapy alone, otherwise all symptomatic cases need drainage. Repeated thoracocentesis is not recommended except in a few with very thin pus. If the aspirate is in the form of thick pus, chest tube must be inserted at the outset.

t

anagement

The treatment options for management of empyema thoracis are antibiotics, with any of the following: Insertion of chest drain alone Intercostal drainage with addition of fibrinolytics Open decortication Video assisted thoracic surgery Rib resection/thoracoplasty/lobectomy.

D



Empyema appears well-defined, smooth, round or elliptical on CT scan. The parietal and visceral layers are separated by interposed empyema fluid, giving rise to “split pleura sign” of empyema. CT scans should not be performed routinely as it exposes the child to a very high radiation dose while most information for diagnosis and for guiding treatment decisions can be received from a good sonogram. It may have a limited role in the cases which do not respond to the initial medical management or before surgery to delineate the anatomy and to rule out a lung abscess.



S

C

T can

The chest tube is connected to an underwater seal bottle which must always be kept below the level of the patient’s chest. The drain should be clamped for 1 hour once around 10 mL/kg of initial removal of pleural pus. In larger children and adolescents no more than 1.5 L should be drained at one time. A chest radiograph is repeated after chest drain insertion to check the placement of tube and lung expansion. The chest tube can be removed when the drainage decreases to less than 30–50 mL/day and there is no air leak from any accompanying bronchopleural fistula and the lung has expanded. A non-functional drain (no air column movement) should also be removed and the need for replacement has to be assessed by repeat imaging. Loculation should be ruled out. The chest tube should be removed during expiration or a Valsalva manoeuver. Chest X-ray is repeated after 24 hours to check for any recollection. The agents such as urokinase, streptokinase and alteplase have been used to facilitate drainage. Their use is recommended for complicated parapneumonic effusion (thick fluid with loculations) or empyema (frank pus) as it increases the drainage and obviates the need for more invasive procedures, shortens hospital stay. The treatment however is expensive and has side effects which can be serious.

iseases f es ira ory ys em

sion to very echogenic fluid with septa as seen in frank empyema. Ultrasonography shows limiting membranes suggesting the presence of loculated collections even when they are not so well seen on CT scan.

esponse to Therapy

Children may continue to be febrile for 5–7 days after starting antibiotic therapy in the case of S. pneumoniae and

499

vip.persianss.ir



t

t

K

Tex book of Pedia rics

H. influenzae and a little longer in the case of Staphylococcus aureus infection. The clinical response to therapy should be assessed with parameters such as decrease in fever, normalization of lab parameters such as WBC count, CRP, decrease in drain volume, clearing in CXR, improvement in the overall condition of the patient. In case of total non-response after 96 hours of treatment, as evidenced by high spiking fever and persistent drainage, second line treatment may be instituted. A combination of vancomycin with ceftazidime is suggested. Parenteral therapy is continued till the child becomes afebrile or at least till the chest tube is removed and subsequently can be started on oral antibiotics to finish 4–6 weeks of therapy. Failure of medical therapy, persisting sepsis and large pleural collection beyond 10 days should prompt surgical referral. Cases of chronic empyema with a symptomatic child should be referred for open thoracotomy and decortication. Cases with persistent bronchopleural fistula will also benefit from surgical intervention. A persistent radiological abnormality in a symptom free child is not an indication for surgery.

ey Points

• There is no “one size fits all” treatment for parapneumonic effusions and empyema • The pathogenicity of empyema is a dynamic process: it is not feasible to manage all stages of with a single therapeutic strategy • Management, therefore, needs to be tailor made using the broad principals described above on a case-by-case basis and requires clinical experience • The outcome is usually good • Key steps in treatment are diagnostic thoracocentesis and preference for percutaneous drain • The use of fibrinolysis in the early empyema stage may be guided by the user’s experience, affordability and cost vs. benefit expected • Non-resolving cases may benefit from surgical intervention. However, efforts to achieve complete radiological clearance are largely non-productive and inconsequential • In the long term, most children will eventually show a complete expansion of the lung, if appropriately treated in the early phase.

500

vip.persianss.ir

athophysiology



The continued cycle of infection, inflammation, and airway injury with impaired mucociliary clearance results in loss of the airway muscular and elastic components with dilation and distortion of the airways and increased mucus production. The airways become collapsible, limiting airflow, especially with forced expiration. The lung parenchyma is often involved, developing areas of atelectasis, emphysema and fibrosis. In addition, there is marked hypertrophy of the bronchial vasculature, which is prone to rupture. Morphologically bronchiectasis is classified as cylindrical (fusiform), varicose and saccular (cystic). Cylindrical is mildly enlarged bronchi that fails to taper distally, this is an early feature after an infection and can be reversed on appropriate management. Varicose type has a beaded appearance due to areas of constriction and dilation. Saccular is the most severe form, i.e. irreversible ballooning of airways.

M

Etiological factors in bronchiectasis are traditionally classified as congenital and acquired. Congenital causes of bronchiectasis are unusual either due to abnormal tracheobronchial development or inherited disorders. Acquired bronchiectasis is always due to two mechanisms, obstruction and infection. It is usually the net result of the classic triad of bronchial obstruction, infection and inflammation causing progressive irreversible airway damage resulting in bronchiectasis.

r

anifestations

The most common symptom in children with bronchiectasis is persistent cough, which is present in 80–90% of children with bronchiectasis, and is typically “wet” or productive. About 50–70% of children also produce purulent sputum. The absence of sputum production does not exclude bronchiectasis, because children younger than 6 or 7 years old may not be able to expectorate sputum. Some patients present with episodic exacerbations of infection, characterized by increased cough and sputum production that may be associated with fever, pleuritic chest pain and dyspnea. These exacerbations typically respond to antibiotic therapy. Hemoptysis is caused by erosion of inflamed airway tissue adjacent to pulmonary vessels. The amount of bleeding can range from mild, with blood streaked sputum, to profuse amounts of fresh bleeding if larger pulmonary vessels rupture. Dyspnea and exercise intolerance are uncommon at presentation but may develop as the disease progresses, or may occur during an acute exacerbation of the disease due to intercurrent infection. Children with severe bronchiectatic lung disease may have cyanosis, indicating severe hypoxemia due to mismatched pulmonary ventilation and perfusion. If the hypoxemia is prolonged and profound, it may lead to pulmonary hypertension and cor pulmonale. The underlying disorder responsible for the bron­ chiectasis may also cause other symptoms at presentation, and provide clues to the diagnosis. As example, failure to thrive is frequently seen in diseases such as CF and immunodeficiency disorders. Chronic sinusitis is common among children with CF, ciliary dysfunction disorders, and immunodeficiencies. The presence of congenital anomalies should alert the clinician to the possibility of associated anomalies that predispose to bronchiectasis (e.g. congenital heart disease and anomalies of the tracheobronchial tree). 501

Causes

Clinical



P



Bronchiectasis is a structural abnormality characterized by abnormal dilation and distortion of the bronchial tree, resulting in chronic obstructive lung disease. This condition is typically the end result of a variety of pathophysiologic processes that render the bronchial walls weakened, easily collapsible, chronically inflamed, and plugged with mucus secretions. The prevalence of bronchiectasis in developed nations has gradually declined in recent years, probably because of improvements in sanitation and housing, immunizations against respiratory illnesses (e.g. measles and pertussis) and antibiotic use.



Bronchiectasis

y ystem



Suppurative disease of the lung includes bronchiectasis, lung abscess and empyema. Bronchiectasis and lung abscess have been discussed here. Empyema is discussed separately in Chapter “Empyema”.

The types of disorders that cause bronchiectasis vary among populations and age groups. As examples, infections and acquired causes of bronchiectasis predominate in developing nations, whereas congenital anomalies of the airways or immune system are more prominent in children of developed nations. The conditions that predispose to bronchiectasis can be classified into the following categories (Table 8.9.1): • Congenital anatomic defects • Immunodeficiency states • Altered pulmonary host defences • Acquired bronchial obstruction • Infection • Miscellaneous disorders.

ntroduction

or

I

L Subramanyam

S

S



uppurative Lung Disease

Diseases Of Respi at

8.9

vip.persianss.ir















d P































Infections – Childhood infections - Pertussis - Measles – Persistent bacterial bronchitis - Staphylococcus aureus - Klebsiella pneumoniae - Pseudomonas aeruginosa – Post-viral – adenovirus – Other infections - Mycobacterium tuberculosis - Fungal – Mycoplasma pneumoniae

-

















Acquired bronchial obstruction – Intraluminal obstruction - Airway foreign-body - Granulation tissue – Extraluminal compression - Lymphadenopathy (TB) - Vascular compression – Right middle lobe syndrome - Chronic or recurrent atelectasis usually seen in asthmatics









Altered pulmonary host defences – Cystic fibrosis (mucoviscidosis) – Primary ciliary dyskinesia - Kartagener syndrome – Impaired cough (e.g. neuromuscular weakness condition)













Miscellaneous disorders – Allergic bronchopulmonary aspergillosis – Recurrent aspiration - Tracheoesophageal fistula - Neurological disorders - GERD – Bronchiolitis obliterans

valuation

The aims of evaluating children with suspected bronchiec­ tasis are: • To confirm the diagnosis • To define the distribution and severity of airway involve­ ment • To characterize extrapulmonary organ involvement associated with bronchiectasis (e.g. cor pulmonale) 502

Figure 8.9.1 Bronchiectasis of left lower lobe  

E













•



























•



















•









•















T





ext





boo





o

• Immunodeficiency states – Primary abnormality - Congenital (Bruton’s type) agammaglobulinemia - Selective immunoglobulin A, immunoglobulin G sub-class deficiency - Severe Combined T and B cell immunodeficiency (SCID) – Secondary abnormality - HIV infection - Immunosuppressive agents

To identify familial and treatable underlying causes of bronchiectasis. The evaluation includes a complete medical history and physical examination, as well as laboratory testing, radiographic imaging and pulmonary function test. A complete medical history, including past medical, family, travel and environmental history, is a crucial part of the evaluation and can be helpful in identifying the underlying cause for bronchiectasis. Certain features of the history should raise concern for specific underlying disorders (e.g. history of choking suggests foreign body aspiration; chronic aspiration should be considered in patients with recurrent pneumonia particularly those with neurologic dysfunction). The general physical examination of the patient with suspected bronchiectasis may also identify the features described above that point to an underlying etiology, including failure to thrive, sinus and ear infections, neurologic dysfunction, and the presence of congenital anomalies. In addition, the pulmonary examination may reveal the following features: crackles and rhonchi, which are often heard over the area of bronchiectasis; wheezing, which is less common; clubbing of the nail bed, which is a basic clinical sign of bronchiectasis; and chest wall deformity, which can be seen in obstructive lung diseases (e.g. CF), in which hyperinflation of the lungs results in increased anterior to posterior chest diameter. On evaluation chest radiograph, findings that are suspicious for bronchiectasis include recurrent/persistent infiltrates or atelectasis in the same lobe or segment (Fig. 8.9.1). The HRCT is the most sensitive imaging method to detect bronchiectasis (Fig. 8.9.2). For patients with diffusely distributed bronchiectasis, the minimum evaluation includes testing for CF [sweat chloride or deoxyribonucleic acid (DNA) testing] and immunodeficiency (including CBC with differential, immunoglobulin’s and IgG sub classes). Ciliary dyskinesia should be considered in patients with recurrent sinus and ear infections, and evaluated with a nasal mucosal biopsy. Potential mechanisms of aspiration

c r

• Congenital anatomic defects – Tracheobronchomalacia – Pulmonary sequestration – Cartilage deficiency (Williams-Campbell syndrome) – Tracheobronchomegaly (Mounier-Kuhn syndrome)

•



k f e iat i s

Table 8.9.1 Bronchiectasis in children - predisposing conditions

vip.persianss.ir

P

Diseases Of Respi at

revention

Appropriate management of aspiration [gastroesophageal reflux (GER), foreign body] aggressive management of lower respiratory infections to prevent postinfectious bron­ chiectasis and routine immunization against respiratory pathogens (measles, pertussis, H. influenzae, viral influenzae, pneumococcal) are helpful in preventing bronchiectasis.

rognosis

-

Overall, the prognosis for patients with bronchiectasis has improved considerably in the past few decades. Earlier recognition or prevention of predisposing conditions, more powerful and wide spectrum antibiotics, and improved surgical outcomes are likely reasons.

or S

Both anaerobic and aerobic organisms can cause lung ab­ scesses. Common anaerobic bacteria that can cause a pul­ monary abscess include Bacteroides species, Fusobacterium species, and Peptostreptococcus species. Abscesses can be caused by aerobic organisms such as Streptococcus species; Staphylococcus aureus, Escherichia coli, Klebsiella pneumoniae and Pseudomonas aeruginosa. Staphylococcus aureus is the organism most frequently involved. F

Clinical eatures Clinical manifestations of lung abscess are nonspecific and similar to those of pneumonia. They include fever, cough, dyspnea, chest pain, anorexia, hemoptysis and putrid breath. The course may be indolent. Physical examination typically reveals tachypnea, dyspnea, and retractions with accessory muscle use, decreased breath sounds and dullness to percussion in the affected area. Crackles may be heard on examination.

Diagnosis -

-

-

The diagnosis is suggested by a chest radiograph demonstrating a thick walled cavity with an air fluid level (Fig. 8.9.3) and confirmed by contrast enhanced computed tomography. Lung abscess is often accompanied by parapneumonic effusion. Lung abscess should be suspected when consolidation is unusually persistent, when pneumonia remains persistently round or mass like, and when the volume of the involved lobe is increased (as suggested by a bulging fissure). Interventional radiology may be helpful in obtaining a specimen from the abscess cavity for diagnostic studies. -

P

-

The initial therapy for patients with bronchiectasis is medical and aims at decreasing airway obstruction and controlling infection. Chest physiotherapy (postural drainage), anti­ biotics and bronchodilators are essential. Two to four weeks of parenteral antibiotics are often necessary to manage acute exacerbations adequately. Antibiotic choice is dictated by the identification and sensitivity of organisms found on sputum (induced or spontaneous), or BAL fluid cultures. Low dose long term macrolide therapy is found to be beneficial in idiopathic bronchiectasis. Nutritional support is important in children. They should be routinely advised on adequate calories and good protein diet. Any underlying disorder (immunodeficiency, aspiration) that may be contributing must be addressed. When localized bronchiectasis becomes more severe or resistant to medical management, segmental or lobar resection may be warranted.

E

-

M

anagement

tiology



 

should be assessed using video fluoroscopy, esophageal pH monitoring, and/or nuclear scintigraphy. For patients with focal bronchiectasis, imaging and/or bronchoscopy should be performed to assess for airway obstruction (e.g. airway foreign body or congenital pulmonary anomalies). Pulmonary function tests can be helpful to evaluate the severity of lung disease and should be performed in older children. Most patients with bronchiectasis have features of obstructive lung disease, indicated by low FEV1 and FEV1/ FVC ratio.

A lung abscess is an accumulation of inflammatory cells, accompanied by tissue destruction or necrosis that produces one or more cavities in the lung. A primary lung abscess occurs in a previously healthy patient with no underlying disorders. A secondary lung abscess occurs in a patient with underlying or predisposing condition. Aspiration is the most important predisposing factor for lung abscess, which may develop 1–2 weeks after the aspiration event; other predisposing factors include airway obstruction and congenitally abnormal lung.

y ystem

Figure 8.9.2 Bilateral bronchiectasis of lower lobes

r

A

Lung bscess

503

vip.persianss.ir

o

P

d

r

c

k f e iat i s

increased risk of complications that occurs in children with necrotizing pneumonia. Percutaneous drainage should be considered in children with lung abscess whose condition fails to improve or worsens after 72 hours of antibiotic therapy. At least 3 weeks of IV antibiotic therapy should be delivered before lobectomy is considered for treatment failure.

boo

Complications

T

ext

The most common complication of lung abscess is intracavitary hemorrhage. This can cause hemoptysis or spillage of the abscess contents with spread of infection to other areas of the lung. Other complications of lung abscess include empyema, bronchopleural fistula, septicemia and cerebral abscess.

M

P

 

Figure 8.9.3 Lung abscess right lower lobe with air fluid level

anagement

Overall, prognosis for children with primary pulmonary abscesses is excellent. Most children become asymptomatic within 7–10 days. Radiologic abnormalities usually resolve in 1–3 months.

Bibliography 1. Chang AB, Redding GJ. Bronchiectasis. Kendig’s Disorders of the Respiratory Tract in Children. Philadelphia, PA: Saunders. 2006.pp.463 77. 2. Fakhoury K, Kanu A. Clinical manifestations and evaluation of bronchiectasis in children. UpToDate; www.uptodate.com Apr 18, 2012. 3. Lakser O. Bronchiectasis, Lung abscess. Nelson Textbook of Pediatrics, 18th edition. 2008.pp.1800 3. 4. Redding GJ. Bronchiectasis in children. Pediatr Clin North Am. 2009;56(1):157 71. 5. Sethi GR, Batra V. Bronchiectasis: causes and management. Indian J Pediatr. 2000;67(2):133 9. 6. Vijayasekaran D, Shivbalan So. Bronchiectasis. Essentials of Pediatric Pulmonology, 3rd edition. 2008. pp.196 200.





-





-





-





-





-

-





-

Treatment of lung abscess requires a prolonged course of antibiotic therapy usually initiated parenterally. Antibiotic choice should be guided by gram stain and culture but initially should include aerobic and anaerobic coverage. Treatment regimens should include a penicillinase resistant agent active against S. aureus and anaerobic coverage, typically with clindamycin or ticarcillin/clavulanic acid. If Gram negative bacteria are suspected or isolated, an aminoglycoside should be added. The duration is determined by the clinical response, but is usually a total of 4 weeks or 2 weeks after the patient is afebrile and has clinical improvement. The average duration of fever is 4–8 days. Eighty to ninety percent of lung abscesses in children resolve with antibiotic therapy alone, provided that bronchial obstruction is removed. In cases that fail to resolve with antibiotics alone, needle aspiration or percutaneous catheter drainage may provide diagnostic information and therapeutic benefit without the

rognosis

504

vip.persianss.ir

Bronchial sthma A





Diseases Of Respi at

8.10

E E

The incidence of asthma has steadily increased in both developed and developing countries from 1970 to 2000. There is a marginal decrease in the incidence in Australia, Hong Kong, UK, Italy and México after 2000 most probably due to saturation of the genetically predisposed population. However, prevalence of persistent asthma needing constant medications is increasing. A hospital based study in Bengaluru showed that the prevalence of asthma steadily increased from 9% in 1979 to 29.5% in 1999. It decreased by 3% in 2004 and further by 1% in 2009. However, persistent asthma increased by 20–72% and persistent severe asthma by 4–11% from 1999 to 2009. The increased prevalence of asthma is noticed in the following situations: • Urban children • Rapid urbanization increasing prevalence in the semirural areas compared to rural • Children attending schools in heavy traffic areas especially from lower socioeconomic population • Children living in poorly ventilated homes and single room dwelling huts • Children living in houses with tobacco smoking persons • Children living in houses where cow dung cakes, agricultural waste and firewood are used as cooking fuel • During Diwali and similar festivals (by 100%) due to increase in SO2 • Children in the age group of less than 5 years (≈75%); above 5 years of age ≈25% • Male predominance—the male to female ratio is 2:1 owing to the relatively small airways with which they are born and inherited as an autosomal dominant trait • Positive family history of asthma either in parents, siblings or grandparents.

tiopathogenesis

The etiological factors can be classified as biologicals and irritants (Table 8.10.1).

A

or

r

Children develop sensitivity to indoor allergens as they grow older in the atmosphere they are reared. The predominant indoor allergen is the house dust mite. It takes 100 mites/g of dust to get sensitization and 500 dust mites/g of dust to produce wheezing. Fifty percent of perennial asthma is due to dust mites. The pollen and mould sensitivity is observed less frequently whereas cockroach sensitivity is fairly common.

S

Viral nfections I

pidemiologyv

erobiologicals

y ystem

Asthma is a non-communicable chronic lung disease, characterized by the following: • Airway inflammation • Airway obstruction mainly due to muscle spasm, associated with mucosal edema and stagnation of the mucus • Airway hyper-reactivity to aerobiologicals and irritants • Airway remodeling in uncontrolled asthma.

The precipitating factor for an asthma attack in 40% of the children is viral URTI. Most children develop rhinitis with or without fever followed by cough. In these children chest congestion persists over 10 days and often along with wheeze. The incidence varies from 29% to 54% in both atopic and non-atopic individuals. Respiratory syncytial virus and rhinoviruses are the predominant viruses triggering asthma. S

ntroduction

eason

Seasonal variation of asthma attacks is experienced by 35% of children. Of these the incidence during monsoon, followed by winter and less common in summer. Recently there is an increase of asthma attacks in summer from 3% to 20%. This is attributed to increase in automobile emission and the bright sun converting the oxides of nitrogen to O3, which aggravates the asthma sensitivity. F

I

H Paramesh

ood

The role of food allergy in asthma is difficult to prove in children. Parents do observe that the children do relatively well when a suspected food allergen is avoided. The most blamed offenders include grapes, banana, guavas, citrus fruits, ice creams, fried foods, and tomatoes with other items less common. Table 8.10.1 Etiological factors in asthma Biologicals

Irritants

Dust mites

Tobacco smoke

Cockroaches

Cooking fuel smoke

Pollens

Mosquito coil smoke

Fungi

Sprays

Pets - saliva, urine

Perfumes

Viral infections Food

505

vip.persianss.ir

P

Air pollution is both outdoor and indoor. The important outdoor pollutants are black smoke, respirable suspended particulates, SO2, NO, CO, O3 and lead produced by combustion of fossil fuels, factories, mines, paper pulp mills and automobile exhaust. Nitrogen oxide triggers asthma in 65% of patients and also sensitizes to dust mites allergy. Ozone triggers asthma in 43% of patients and SO2 sensitizes the asthmatics by tenfold.

• • • •

athophysiology

Asthma is a complex condition where interaction of genetics and environment occurs involving many inflammatory cells which release a wide range of variety of mediators. These mediators act on the cells of the airway leading to smooth muscle contraction, mucus hypersecretion, plasma leakage, edema, activation of cholinergic reflexes and activation of sensory nerves, which lead to amplification of the continuing inflammatory response. The chronic inflammation leads to structural changes, including sub-epithelial fibrosis and smooth muscle hypertrophy and hyperplasia. This late process is less easily reversed than the acute changes and might end up with airway remodeling. To mount an allergic response one has to be sensitized to an allergen. When an allergen enters the epithelial cell the dendritic cells below the epithelia cell act like custom officer, catch the allergen by their long arms and break the allergen into small pieces called epitomes. These are handed over to the T lymphocytes, the orchestrator of the immune system, which give the instruction to various cells for proper action. The B lymphocytes are instructed to handle pollens which produce interleukins, IL4 and IL13 to make immunoglobulin E (IgE) antibodies. The IgE arms the mast cell, the muscle man of immune system. The mast cell binds very large number of IgE molecules of various allergens. The activation of mast cell (mast cell degranulation) releases histamine. The continued exposure to allergen brings eosinophils (dreaded cell for parasites) into action. The eosinophilic granules release toxic chemicals such as eosinophil cation protein, eosinophil derived neurotoxin and major basic protein, which damage the epithelium and sensory nerves causing hyper-responsiveness to nonallergic stimuli. The mast cells are responsible for early phase reaction and eosinophils for late phase reaction in general.

linical eatures F

C

•

All that wheezes is not asthma but most asthmatics don’t wheeze. The common clinical features include the following: • Recurrent cough: The majority of children present with recurrent cough 90% of the time. The cough is more 506

at night, or early morning time; induced by physical or emotional stress (crying, laughing and shouting) Recurrent wheeze is a prominent feature of lower airway obstruction. Many people mistake rattling in the chest as wheeze. Wheezing is not felt on palpation of the chest Retractions are a common feature for airway obstruction, depending on the severity of asthma they may be present over subcostal, intercostal, or suprasternal area, with flaring of nostrils Post-tussive vomiting (vomiting after a bout of coughing) occurs in 5% of cases Abdominal pain rarely occurs due to over-working of expiratory abdominal muscles Chest pain is present rarely Other comorbid conditions like allergic rhinitis, sinusitis, serous otitis media, eczema and conjunctivitis may be present.

Diagnosis Diagnosis of asthma is mainly by history and physical examination. The diagnosis of asthma in infants and preschoolers is difficult due to poor cooperation in diagnostic procedures, hesitation by pediatricians, time consuming and practicality, and ethical issues. However, the need for diagnosis is essential for early treatment, to improve the quality of life, physical and psychological development, to avoid chronic pulmonary disease “airway remodeling” and to educate on the preventive measures, to cut the health care cost and to prognosticate.

reschoolers

The diagnosis of asthma is made based on national guidelines in an infant who has more than 3 episodes of wheeze in 1 year with family history of asthma, has atopic features, afebrile episodes and cough persisting more than 2 weeks with good response to bronchodilators. In some children a therapeutic trial of treatment with quick relievers and inhaled steroids for 8–12 weeks with good improvement and relapse after stopping treatment is diagnostic of asthma.

•

• •

chool-Going hildren C

A

P

ir ollution and sthma

•

P

P A

Pets are not the major factors for the asthma in India. Cats are more allergic than dogs. The saliva, urine and dander are the causes for allergy sensitization.

S

o

P

d

r

k f e iat ics boo

ext T

ets

Peak expiratory flow (PEF) measurement before and after salbutamol nebulization with improvement (12–15%) is highly suggestive of reversible airway obstruction in asthma. This simple and less expensive procedure can be used to monitor the therapeutic response. Early asthma attack can be recognized by measuring diurnal variation PEF. It measures the air coming out of larger and medium size airways Spirometry is the central tool for defining the obstructive airway disease. In asthma there is decreased FEV1, FEV1/ FVC ratio and FEF 25–75 Eosinophil count: The increased eosinophil count in the blood is suggestive of an allergic phenomenon

vip.persianss.ir

P

F

The drugs used in the management of asthma include quick relievers, preventers and long-term symptoms relievers as listed in Table 8.10.2. The choice of the drugs depends on the age and severity of the wheezing episodes (Table 8.10.3). It should be ideally follow carried out in a stepwise manner. Always use quick reliever as a rescue treatment in acute exacerbation of persistent asthmatics that are on preventers. Inhalation method is the best way to administer medication to avoid adverse reactions and for quick actions.

C

ducation

E

harmacotherapy

S

ollow-up

Regular follow-up is needed to assess the control of asthma by using the parameters as listed in Table 8.10.4. Always give hope, confidence and encourage sports activities.

The management of asthma includes: • Education • Environment control • Pharmacotherapy • Regular follow-up.

The pediatrician must spend time to clear the misconceptions about the disease, sexual bias, non-communicability of the disease, fear of inhalers, steroids, etc. In India, doctors should always include the grandparents in counseling. Individual counseling is preferred in adolescents. C

nvironment ontrol

E

r

•

or

•

ungus: Attend to damp walls, have good ventilation, and clean the shower curtains weekly ets: Keep them away from sleeping area, if possible outside the house Avoid strong odors, smoke, mosquito coil burning, and especially tobacco smoke Have indoor plants to absorb formaldehyde and volatile organic compounds from modern furniture, etc. and expose to sunlight once a week and remove the water from trays once a week. P

•

F

M

anagement

•

y ystem

Total IgE level: It is beneficial only to recognize the atopic background of asthma. One can predict the possibility of development of persistent asthma, prognosis, strongly advocate environment control and good response to steroids • Specific IgE level: It is needed for specific immunotherapy and before the use of anti-IgE antibody treatment • Chest X-ray is not needed to diagnose asthma. It is needed only when the diagnosis is not clear or any complications are suspected. Please note that in children under-5 years who are devoid of lung collaterals, one can see sub-segmental, segmental or lobar atelectasis quite often, which might be treated as recurrent pneumonia • Breath nitric oxide is used in some pulmonary centers for monitoring the eosinophilic airway inflammation. It is not used much in children for various reasons • Skin testing with allergens is the gold standard to identify the specific allergens and used before immunotherapy for aeroallergens • Bronchial challenge tests and other physiological tests do not have a major role in the diagnosis of asthma in children.

Diseases Of Respi at

•

C

It is the most important factor in the control of asthma. The aim should be to avoid allergens and irritants: • Dust mites: Avoid carpets, upholstered furniture, use plastic covers to pillows and mattresses; and expose to sunlight once a week; wash soft toys periodically; and wet mop the floorings • ockroach: Cover garbage and unused food containers

omorbidities

The most common comorbid conditions are: • Allergic rhinitis: Nearly three-fourths of asthmatic children develop allergic rhinitis which complicates and worsens asthma. Nasal breathing is protective to asthma. Always evaluate as a unified airway disease and successful treatment of allergic rhinitis can control asthma • Sinusitis: Nearly 9% of asthmatics have sinusitis and the incidence in uncontrolled asthma is nearly 35%. The sinusitis is a result of blockage of osteomeatuses of sinuses due to edematous nasal mucosa and compromise in the mucociliary function of sinus • Otitis media: Nearly 25% of children have serous otitis media due to blockage of eustachian tube from edema of nasal mucosa

Table 8.10.2 Classification of drugs used for the management of asthma











Used to relieve bronchospasm for longer hours. • Long acting beta-2-agonists – Salmeterol – Formoterol – Bambuterol • Always use with inhaled Steroids



Long-term symptoms relievers

Used for long-term to control the inflammation and to prevent further attacks. • Leukotriene receptor antagonists • Steroids – Oral – Inhaled (ICS) • Theophylline



Preventers

Used for acute attacks to relieve bronchospasm as and when needed. • Short acting beta-2- agonists – Salbutamol – Terbutaline • Adrenaline • Aminophylline

Quick relievers

507

vip.persianss.ir

 

Step 1 (Intermittent) • The SABA as needed • If needed more than 2 times/week add preventers

Step 2 (Persistent mild) • LTRA or • ICS low dose

Step 2 (Persistent mild) • Low dose ICS or • LTRA

Step 3 (Persistent moderate) • Low dose ICS + LTRA or • Double the dose of ICS

Step 3 (Persistent moderate) • Low dose ICS + LTRA or • Low dose ICS + LABA or • Low dose ICS + Theophylline sustained release or • Double the dose of ICS

Step 4 (Persistent severe) • Medium dose of ICS + LTRA or • High dose ICS Use oral steroids during acute severe exacerbation

Step 4 (Persistent severe) • Medium dose ICS +LABA or • Medium dose ICS + LTRA or • Medium dose ICS + Theophylline SR or • High dose ICS











More than 5 years

Step 1 (Intermittent) • The SABA as and when needed • If SABA is needed more than 2 times/week, add preventers 

Less than 5 years















o

P

d

r

k f e iat ics boo

ext T

Table 8.10.3 Selection of drugs

Step 5 • Add oral steroids • Anti-IgE antibody treatment • Immunotherapy Abbreviations: SABA: Short acting beta-2-agonists; LABA: Long acting beta-2-agonists; LTRA: Leukotriene receptor antagonist; ICS: Inhaled corticosteroids; SR: Sustained release Key: Low dose of ICS: 200 µg Medium dose of ICS: 400 µg High dose of ICS: 400 to 500 µg

 

Table 8.10.4 Level of control of asthma Parameters

Controlled

Partly controlled

Poorly controlled

Daytime symptoms

Less than 2 times/week

More than 2 times/week

Limitations of activities

None

Any

Nocturnal symptoms of disturbed sleep

None

Any

Three or more features of partly controlled asthma in any week

Need for relievers or rescue treatment

Less than 2 times/week

More than 2 times/week

Lung functions (PEF or FEV1)

Normal

Less than 80% of predicted or personal best

Exacerbation

None

One or more per year

508 •

hospitalization for intensive treatment. This occurs when the early signs of severity are missed or ignored by the patients, parents and physicians. A

S

A

anagement of cute evere sthma in the Hospitalized atient P

Gastroesophageal reflux disease: GERD is quite common in infants, but GERD as a cause for worsening of asthma is seen in few cases of refractory asthma. It should be suspected in children with prominent asthma symptoms while eating or sleeping or who prop up in beds themselves to relieve nocturnal symptoms. The gold standard for diagnosis is esophageal pH monitoring Acute severe asthma: Asthma attacks can be very severe in untreated or improperly treated cases needing

M

•

One in any week

To cover all aspects in acute severe attack of asthma and to prioritize the treatment, please remember six ‘M’s in aiming therapy (Table 8.10.5).

vip.persianss.ir

A

or

r

Recognition of unified airway disease Recognition of chronic inflammatory disease concept Appreciating the asthma phenotypes based on infla mmatory cells involved and mediators release by eosinophils, neutrophils, T-lymphocytes and vascular endothelial cells The importance of environment control The use of breathing exercise or yoga to avoid chest and spinal deformity which have increase morbidity and mortality Use of Anti-IgE antibody treatment in difficult case The use of immunotherapy preferably by sublingual route.

• •

• •

S

y ystem

The primary prevention is to prevent sensitization to allergens. The deprivation of protective germs in early life might drive early immune development with T-helper cells (TH2) stimulation and from allergic sensitization, persistent airway inflammation and remodeling. The farming environment has protective effect from exposures to lipopolysaccharide endotoxin from microorganism. Avoid unnecessary use of high spectrum antibiotics. Use of probiotics has doubtful effect in the prevention of development of asthma. The secondary prevention is to prevent occurrence of symptoms in sensitized child is to avoid triggering factors like environmental factors predominantly the tobacco smoke and indoor pollutants like dust mites, cockroach, fungi, pollens and other smokes. Prolonged breast feeding

• • •

­

revention

Recent dvances

P

P P

Long term follow-up of published studies are not available in India. Viral infection triggered asthmas tend to outgrow by 5 years of age when the immune system reaches adult levels. Some more outgrow by 8 years when the airway caliber reaches adult levels, more so in boys. During adolescent period majority tend to outgrow the attacks. However, those with atopic asthma (high IgE level) and children with low lung capacities and girls have the tendency for indefinite period.

up to 6 months, which is Indian tradition, can help to prevent infection triggered asthma. The tertiary prevention is to control the symptoms by environment control and long-term use of antiinflammatory drugs and quick relievers as rescue medicines in any exacerbations.

Diseases Of Respi at

rognosis

ractice Guidelines

Use quick reliever as and when necessary and use preventers in step 2–5 as described in Table 8.10.6.

 

Table 8.10.5 Six ‘M’s in the management of acute severe asthma in children Principle of management

Pathogenetic mechanism

Management

Metabolic correction

Hypoxemia, metabolic acidosis

Humidified oxygen by mask, nasal prongs

Muscle spasm to be relieved

Airway obstruction

Beta-2-agonists + ipratropium bromide in O2 driven nebulizers

Mucosal edema

Inflammation, airway obstruction

Systemic or oral prednisolone or methylprednisolone (IV) or inhaled fluticasone 1,500 µg

Mucus secretions

Excess mucus secretion and airway obstruction

Appropriate hydration, staccato type of coughing

Monitor for infection

Pneumonia, otitis media, sinusitis

Antibiotics

Mechanical ventilation

Respiratory acidosis, apnea ventilation failure

Ventilatory care as last resort

Table 8.10.6 Stepwise management of bronchial asthma Step 1

Step 2

Step 3

Step 4

Step 5

Asthma education - include grand parents Environment Control Beta-2-agonists SOS Controller options

Beta-2-agonists in rescue treatment SOS Select one

Select one

To step 3. add any one in the list

To step 4. add any one

ICS low dose or LTRA

ICS low dose + LABA or LTRA or Theophylline SR or ICS med dose

Medium or high dose ICS + LABA or LTRA or Theophylline SR

Oral steroid (short course) or Anti-IgE (Omalizumab) or Immunotherapy

509

Abbreviations: LABA: Long acting beta-2-agonists; LTRA: Leukotriene receptor antagonist; ICS: Inhaled corticosteroids; SR: Sustained release

vip.persianss.ir

• • •

K

•

Aim at good control of asthma with good education and bonding with patient, parents, and grandparents for better compliance Avoid prolonged use of systemic steroids or high dose of inhaled steroids Always use spacer preferably clear, valved one for better delivery. For metered dose inhaler (MDI) slow inhalation and for powder inhaler faster inhalation is needed Always assess the compliance and technique of inhalation during follow-up while infusing trust, hope and confidence Counsel the adolescent separately and stress on ill effect of TOBACCO smoke. Stress on good environment control and to have indoor plants which needs to be exposed to sunlight and to remove water from the tray once a week.

ey

M

•

ractical ips T

P

•

T

ext

boo

o

P

d

r

k f e iat ics

Depending on the response either step-up or stepdown the dose of preventers, some studies recommend starting with high dose and then reducing the dose earlier.

essages

• Please keep it in mind that while dealing with a biological system based on expert opinion there should be enough room for individualization • Always look for availability, accessibility and affordability in managing our children with asthma • Do realize that different forms of asthma respond differently to drugs

• Phenotype based treatments are likely to be more effective and safer than standard uniform treatment that we currently offer to all asthma phenotypes • Be optimistic; newer research needs a link between clinicians, researchers and pharmacological companies for better, costeffective individual treatment of patients

Bibliography 1. Bisgaard H, Bonnelykke K. Long-term studies of the natural history of asthma in childhood. J Allergy Clinical Immunol. 2010;126(2):187-97. 2. IAP and Allergy and applied immunology chapter – Allergic Rhinitis and comorbidities training module (ARCTM), 2011. 3. IAP and Respiratory chapter: asthma training module (ATM). 2011. 4. Pan SJ, Kuo CH, Lam KP, et al. Probiotics and allergy in children – an update review. Pediatr Allergy Immunol. 2010;21(4 Pt 2): e659-66. 5. Paramesh H. Epidemiology of asthma in India. Indian J Pediatr. 2002;69:309-12. 6. Paramesh H. Asthma and environment, Indian J Pediatr. 2006;73(Suppl):S51-5. 7. Paramesh H. Asthma in children: seasonal variations. International J Environ Health. 2008;2:410-6. 8. Paramesh H. Textbook of Practical Pediatric Pulmonology. New Delhi: Jaypee Brothers Medical Publishers (P) Ltd, 2009. 9. Petsky HL, Cates CJ, Lasserson TJ, et al. A systemic review and meta-analysis: tailoring asthma treatment on eosinophilic markers (exhaled nitric oxide or sputum eosinophils). Throax. 2012;67(3):199-208. 10. Zhang WX, Li CC. Airway remodeling: a potential therapeutic target in asthma. World J Pediatr. 2011;7(2):124-8.

510

vip.persianss.ir

Section

9

Diseases of Gastrointestinal System and Liver







































9.1 9.2 9.3 9.4 9.5 9.6 9.7 9.8 9.9 9.10 9.11 9.12 9.13 9.14 9.15 9.16 9.17 9.18 9.19 9.20











































Section Editor : VS Sankaranarayanan Acute Diarrhea: Ashok K Patwari Persistent and Chronic Diarrhea in Children: Gadadhar Sarangi Malabsorption Syndrome in Children: Ujjal Poddar Vomiting in Children and Gastroesophageal Reflux Disease: Shashidhararao Nagabhushana Parasitic Bowel Disease: BD Gupta Gastrointestinal Bleeding in Children: Neelam Mohan Constipation in Children: VR Ravikumar Hirschsprung Disease: Kanishka Das Abdominal Pain in Children: Srinivas Sankaranarayanan Hepatomegaly: A Practical Diagnostic Approach: Sheila Bhave Clinical Relevance of Liver Function Tests and Imaging Modalities in Hepatobiliary Disorders: Geetha M Viral Hepatitis: Malathi Sathiyasekaran Chronic Liver Disease and Cirrhosis of Liver in Children: VS Sankaranarayanan Neonatal Cholestasis Syndromes: A Riyaz Cholestatic Disorders in Older Children: B Bhaskar Raju Fulminant Hepatic Failure: Rajiv Chandra Mathur Ascites: Balvir S Tomar Common Metabolic Liver Disorders in Children: Ashish Bavdekar Acute Pancreatitis in Children: D Nirmala Chronic Pancreatitis in Children: Seema Alam

vip.persianss.ir





Acute Diarrhea Ashok K Patwari

Diarrhea is usually defined as passage of three or more loose or watery stools in a 24-hour period a loose stool being one that would take the shape of a container. However, for practical purposes, it is the recent change in consistency and character of stool and its water content rather than the number of stools that is important. Infants who are exclusively breastfed normally pass several soft or semi-liquid stools each day; for them, it is practical to define diarrhea as an increase in stool frequency or liquidity that is considered abnormal by mother.

Acute Watery Diarrhea It refers to diarrhea that begins acutely, lasts for less than 14 days, with passage of frequent loose or watery stools without visible blood. Vomiting may occur and fever may be present. Loss of large volume of water and electrolytes can result in dehydration and dyselectrolytemia.

Dysentery It is the term used for diarrhea with visible blood and mucus. It is often associated with fever and tenesmus. Common clinical features of dysentery include anorexia, rapid weight loss and complications like renal failure and encephalopathy.

Persistent Diarrhea It represents diarrhea, presumed to be caused by infectious agents that begins acutely, but is of usually long duration (more than 14 days). The episode may begin either as acute watery diarrhea (AWD) or as dysentery. Marked weight loss is common. Diarrheal stool volume may also be great, with a risk of dehydration. Persistent diarrhea should not 512 be confused with “chronic diarrhea” which is recurrent or

E

Acute diarrhea constitutes a leading cause of morbidity and mortality among children below 5 years of age in developing countries. On an average, 3.3 episodes of diarrhea are experienced per child per year, but in some areas the average exceeds 9 episodes per year. More than 2 million deaths are estimated to result each year as a consequence of diarrheal disease in under-fives. Eighty percent of these deaths occur in the first 2 years of life, main causes being dehydration, complications associated with dysentery, malnutrition and serious infection such as pneumonia. Most of the diarrheal episodes occur during the first 2 years of life (highest incidence 6–11 months), low socioeconomic status, in non-breastfed infants, and in association with measles, severe malnutrition and immunodeficiency. ­

Definition

pidemiology



Worldwide, particularly in developing countries, diarrheal diseases still constitute significant causes of mortality and morbidity. There is considerable reduction in the mortality in infants suffering from acute diarrhea which is attributed to the proven efficacy of treatment with reduced osmolar oral rehydration solutions (ORS), oral zinc and proper diet monitoring and continuation of breastfeeds in young infants, avoiding unnecessary medications and commercial milk-free foods. It is essential to understand various types of diarrhea based on duration and etiology as the approach and management differs.

long lasting diarrhea due to non-infectious causes such as sensitivity to gluten or inherited metabolic disorder.

E

Introduction

tiopathogenesis

In developing countries, the organisms most frequently associated with AWD include enterotoxigenic Escherichia coli (ETEC), enteropathogenic Escherichia coli (EPEC), Shigella dysenteriae and Campylobacter jejuni. Rotavirus is a common cause of severe diarrhea, vomiting and fever leading to rapid dehydration. Vibrio cholerae is an important organism in endemic areas and during epidemics. Nontyphoidal Salmonella is a common organism in areas where commercially processed foods are widely used and in hospital outbreaks. Most of these organisms produce watery diarrhea. The main cause of acute dysentery is S. dysenteriae, C. jejuni and infrequently enteroinvasive Escherichia coli (EIEC) or Salmonella. Epidemics of dysentery are usually caused by S. dysenteriae type 1. Entamoeba histolytica can cause dysentery in adults but is a less common cause in young children. Diarrhea may also be caused by a number of antibacterial agents like ampicillin, cotrimoxazole, chloramphenicol, amoxicillin, clindamycin, etc. Pseudomembranous colitis is the most severe form of antibiotic associated diarrhea.

Tex

tboo

k

of

Pe

diatrics

9.1

Clinical Features Most enteropathogens can cause diarrhea by more than one mechanism. Hence the clinical presentation depends upon the underlying pathophysiological changes taking place in the gastrointestinal tract. Three clinical types of diarrhea have been defined, each reflecting a different pathogenesis and requiring different approach to treatment.

vip.persianss.ir

is as s of



astroint



stinal st and

m

e L e

Management

iv

Injury to enterocytes may result in brush border damage and epithelial destruction leading to decreased mucosal disaccharidase activity. Clinical presentation is characterized by passage of large, frothy, explosive and acidic stools. High osmolar solutions given orally (e.g. carbonated soft drinks and ORS with high sugar content) can also result in osmotic diarrhea. Besides worsening in the hydration status of the child, there is a serious danger of developing hypernatremia.

Sy

Osmotic Diarrhea

e

Intestinal mucosal cells are actually invaded by the microorganisms which set up an inflammatory reaction clinically presenting with blood and mucus in the stools. This group is prone to develop other complications like intestinal perforation, toxic megacolon, rectal prolapse, convulsions, septicemia and hemolytic uremic syndrome (HUS).

G

Invasive Diarrhea (Dysentery)

e

It is characterized by AWD with profound losses of water and electrolytes due to sodium pump failure as a result of the action of identified toxins. This group is at risk for rapid development of dehydration and electrolyte imbalance. Common causes are ETEC and V. cholerae.

of dehydration. The first symptom of dehydration appears after fluid loss of 5% of body weight. When fluid loss reaches 10%, shock often sets in, and the cascade of events that follows can culminate in death unless there is immediate intervention to rehydrate. Routine stool examination is not recommended except in situations such as young infants with fever, suspected protozoan Giardia or Entamoeba histolytica as a cause, extra gut infections or persistent colitis or disaccharide intolerance and prolonged/persistent diarrhea with malnutrition. Stool culture is invariably non-contributory. Diarrhea when prolonged or recurrent, is a major cause of malnutrition in children, owing to use of bottle feeds, stoppage of breastfeeds and lack of energy dense feeds and hygiene like hand washing, low food intake during the illness (poor appetite, vomiting, oral thrush or stomatitis, diluting/withholding of food, etc.), reduced nutrient absorption in the intestines, and increased requirements as a result of infection. Repeated and prolonged episodes of diarrhea have even more deleterious effects and may eventually result in growth failure, intercurrent infections and problems associated with severe malnutrition and even death.

D e

Secretory Diarrhea



• • • •

General assessment of the child. Assessment of hydration status. A number of clinical signs and symptoms can help in detecting dehydration. However, a simple assessment chart can be referred for quick assessment of dehydration (Table 9.1.1) and administration of appropriate fluids for prevention and treatment of dehydration. Correction of electrolyte and acid-base imbalance. Proper feeding to provide normal nutritional require ments. Zinc supplementation. Treatment of associated problems like dysentery and persistent diarrhea. ­

Diagnosis of acute diarrhea is based on clinical history of passing frequent, loose or watery stools, with or without vomiting, fever, pain in abdomen or blood in the stools. Many children may have symptoms and signs of other associated illnesses like cough, skin rashes/measles or urinary symptoms. The clinical triad of rotaviral diarrhea is fever, vomiting and profuse watery stools with tendency for dehydration. Dehydration is the commonest and life-threatening consequence of diarrhea. Loss of water and electrolytes in the diarrheal stool results in depletion of the ECF volume, electrolyte imbalance and clinical manifestation

• •

r

Principles of Treatment

Diagnosis

 

Table 9.1.1 Assessment of hydration status in a patient with diarrhea Clinical signs Well, alert

Restless, irritable

Lethargic or unconscious

Normal

Sunken

Sunken

Thirst*

Drinks normally, not thirsty

Drinks eagerly, thirsty

Drinks poorly, not able to drink

Skin pinch

Goes back quickly

Goes back “slowly”

Goes back “very slowly”

Decide hydration status

The patient has “no signs of dehydration”

If the patient has two or more signs, there is “some dehydration”

If the patient has two or more signs, there is “severe dehydration”

Treatment plan

Plan A

Plan B

Plan C



General condition Eyes

* In a young infant less than 2 months of age, thirst is not assessed and decision regarding “some” or “severe dehydration” is made if “two” of the three signs are present.

513

vip.persianss.ir

Nutritional rehabilitation. Health education for prevention of diarrhea.

Table 9.1.3 Guidelines for replacement of fluid and electrolytes in children with “no dehydration” (Plan A)  

Oral Rehydration Therapy

Age

After each loose stool, offer*

Concept of oral rehydration therapy (ORT) has revolutionized the management of diarrhea with the discovery of coupled active transport of glucose and sodium in the small bowel, resulting in the passive absorption of water and other electrolytes even during copious diarrhea. Oral rehydration therapy includes: • ORS solution of recommended composition • Solution made from sugar and salt (if prepared correctly) • Food based solutions with appropriate concentration of salt, like lentil soup, rice, kanji, butter milk, etc. • Plain water given along with continued feeding.

< 6 months

Quarter glass or cup (50 mL)

7 months to 2 years

Quarter to half glass or cup (50–100 mL)

2–5 years

Half to one glass or cup (100–200 mL)

Older children

As much as the child can take

* Fluids which can be offered include ORS, lemon water, butter milk, rice kanji, lentil soup, light tea, etc.

Correction of Dehydration For correction of fluid and electrolyte deficit on account of dehydration, 50–100 m /kg body weight (75 m /kg) of ORS must be administered, over a period of 4 hours. If the child wants more, more ORS should be given. Breastfeeding should be continued. Older children should have free access to plain water. Acceptance of ORS, purge rate and vomiting should be closely monitored. l

•

Low Osmolarity Oral Rehydration Salts (ORS) Solution The standard WHO-ORS, used for over three decades, has saved millions of lives but did not decrease diarrheal duration or stool output. More effective low osmolarity ORS (Table 9.1.2) is now recommended as the universal solution for treatment and prevention of dehydration for all causes of diarrhea and at all ages.

Management of “No Dehydration”

•

The objective of treatment is prevention of dehydration and malnutrition (Plan A). The management can be successfully carried out at home, by the mother/caretaker who is advised to: • Give more fluids than normal (Table 9.1.3) • Continue feeding • Bring the child back after 2 days, or earlier if he has any of the danger signs (thirsty, irritable/restless, fever, high purge rate, repeated vomiting, and blood in the stool, eating or drinking poorly, and lethargic).

Management of “Some Dehydration” The objective of treatment is to treat dehydration and electrolyte imbalance, and to continue feeding. These children should be rehydrated with ORS under supervision in a health facility (Plan B).

 

Table 9.1.2 Low osmolarity ORS formulation recommended by WHO/UNICEF

514

Reassess After 4 Hours •

Prevention and Treatment of Dehydration

Reduced osmolarity ORS

g/L

Reduced osmolarity ORS

mmol/L

Sodium chloride

2.6

Sodium

75

Glucose, anhydrous

13.5

Chloride

65

Potassium chloride

1.5

Glucose, anhydrous

75

Potassium

20

Citrate

10

Total osmolarity

245

Trisodium citrate, dihydrate 2.9

• •

l

diatrics

Pe of

k tboo

Tex

• •

•

If still dehydrated, deficit therapy should be repeated (Plan B) and milk/food should also be offered. If rehydrated, it is treated as “no dehydration” with maintenance therapy with ORS as in plan A. If ORT is not successful, it is treated as “severe dehydration” with intravenous (IV) fluids as in plan C.

Management of “Severe Dehydration” The primary objective is to quickly rehydrate the child in a hospital with facilities for IV fluid therapy. Ringer’s lactate is the preferred solution for rehydration and is given as 100 mL/kg over 6 hours in infants less than 1 year and over 3 hours in older children (Table 9.1.4). If Ringer’s lactate is not available, other alternatives like normal saline may be used.

Rehydration of Severely Malnourished Children Rehydration of severely malnourished children deserves special attention owing to certain pathophysiological changes in water and electrolyte balance peculiar to protein energy malnutrition (PEM). Dehydration may be over or underestimated in the presence of marasmus or edema, respectively. These children are at risk to develop hypoglycemia and electrolyte imbalance. Rehydration with ORS solution should be preferred because IV fluids can easily cause overhydration and heart failure. Therefore, it is recommended that severely malnourished children are slowly rehydrated, carefully monitored and feeding is started early.

Feeding During Acute Diarrhea and Dysentery Nutritional management of acute diarrhea and dysentery takes optimal advantage of intestinal absorption capacity, which is affected to some extent during diarrhea, by

vip.persianss.ir

D e

 

is

Table 9.1.4 Deficit fluid therapy for “severe dehydration” (Plan C)

of

l



l

Assess for improvement every 1–2 hours: •  If not improving, give IV infusion more rapidly •  Encourage oral feeding by giving ORS 5 mL/kg/hour, along with IV fluids, as soon as the child is able to drink Reassess hydration status: •  After 6 hours (infants) and 3 hours (older children) assess hydration status and choose appropriate plan for hydration (Plan A, B or C)

e

Monitoring

as

Older child (> 1 year)

30 mL/kg body weight within first 1 hour, followed by 70 30 mL/kg body weight within half an hour, followed by 70 m /kg body weight over next 5 hours m /kg body weight over next 2½ hours

s

Infants (< 1 year) Volume of Ringer’s lactate



astroint



G stinal

e

 

Table 9.1.5 Feeding during diarrhea Stage of hydration During rehydration phase •  Breastfed infants •  Non-breastfed infants

Recommended schedule of feeding

st

   

 

m and

 

•  Breastfeed more frequently •  Offer undiluted milk as before •  Give easily digestible energy rich complementary foods in addition to breast/animal milk •  Give thick preparation of staple food with extra vegetable oil or animal fats, rich in potassium (legumes, banana), carotene (dark green leafy vegetables, red palm oil, carrot, pumpkin) •  Encourage to eat at least six times a day 

r

iv



L e

Zinc Supplementation for Treatment of Diarrhea Zinc deficiency is common in children from developing countries because of intake of predominant vegetarian diets and the high content of dietary phytates. Increased fecal losses during many episodes of diarrhea aggravate pre-existing zinc deficiency. WHO and Indian Academy of Pediatrics (IAP) recommends zinc supplementation as an adjunct to ORS in the treatment of diarrhea. The National IAP Task Force recommends that all children older than 6 months suffering from diarrhea should receive a uniform dose of 20 mg of elemental zinc as soon as diarrhea starts and continue for a total period of 14 days. Children aged 2–6 months should be advised 10 mg per day of elemental zinc for a total period of 14 days.

Use of Antimicrobial Agents Antibiotic therapy should be reserved only for cases of dysentery and suspected cholera (Table 9.1.6). Every case of diarrhea needs to be carefully evaluated for the

presence of blood in the stools which indicates dysentery and to identify cases of suspected cholera (high purge rate with severe dehydration in a child above 2 years in an area where cholera is known to be present). In view of widespread resistance to trimethoprim-sulfamethoxazole (TMP-SMX) and reported resistance to nalidixic acid, IAP Task Force recommends ciprofloxacin as first-line drug in areas where resistance rates to TMP-SMX exceeds 30%. No chemoprophylaxis is needed for contacts. Associated non-gastrointestinal infections like pneu monia, meningitis, urinary tract infection, etc. should also be carefully looked for and appropriately treated. In severe malnutrition, the usual signs of infection such as fever are often absent, yet multiple infections are common in these children. Therefore, it is assumed that all severely malnourished children may have an underlying infection which should be treated with broad-spectrum parenteral antibiotics. ­

feeding small, frequent, energy dense food taking into consideration the age, pre-illness feeding pattern and state of hydration of the child (Table 9.1.5). Feeding is continued in children with no dehydration, and resumed as early as possible in some dehydration.



•  For older children

e

After rehydration phase •  Breastfed infants •  Non-breastfed infants •  Infants 6–12 months

Sy

•  Breastfed infants – Should be preferable given only ORS till they are rehydrated – Animal milk/food should be offered, if rehydration takes longer than 4 hours •  Severely malnourished children •  Offer some food as soon as possible

Nutritional Rehabilitation Nutritional support to a child following an episode of acute or persistent diarrhea is of immense importance in view of the known adverse impact of diarrheal diseases on the nutrition of a young child. The need for proper feeding after an episode of diarrhea has even greater importance 515

vip.persianss.ir

diatrics

 

Table 9.1.6 Antimicrobials used to treat specific causes of diarrhea in children Drug of choice First line Doxycycline Second line Ciprofloxacin *

of

Pe

Cause Cholera

Tex

tboo

k

Dysentery

First line Ciprofloxacin ** Second line Pivmecillinam Ceftriaxone

Dose Single dose of 6 mg/kg PO Single dose 15 mg/kg 15 mg/kg two times a day orally x 3 days 20 mg/kg four times a day PO x 5 days 50–100 mg/kg once a day IM x 2–5 days

Amebic dysentery

Metronidazole

30 mg/kg/day in three divided doses PO x 5–10 days

Acute giardiasis

Metronidazole OR Tinidazole

15 mg/kg/day in three divided doses PO x 5 days 15 mg/kg/day in three divided doses PO x 5 days

* Can be used if there is resistance to doxycycline or no clinical response ** In view of widespread resistance to trimethoprim-sulfamethoxazole (TMP-SMX) and reported resistance to nalidixic acid, Indian Academy of Pediatrics Task Force recommends ciprofloxacin as first-line drug in areas where resistance rates to TMP-SMX exceeds 30%.

E

Complications lectrolyte Imbalance

With appropriate use of oral rehydration therapy, access to plain water and continued feeding, the risk of electrolyte disturbances is minimized. However, following electrolyte disturbances may be encountered in some cases.

Hypernatremia Some children with diarrhea, especially young infants, develop hypernatremic dehydration which usually follows use of hypertonic drinks (canned fruit juices, carbonated cold drink, and incorrectly prepared salt and sugar solutions, ORS with high glucose content). Children with hypernatremic dehydration (serum sodium > 150 mEq/L osmolality > 295 mOsm/kg) are extremely thirsty, out of proportion to their other signs of dehydration and sometimes have convulsions. These children can be successfully treated with low osmolarity ORS. However, if the child is unable to drink orally, Ringer’s lactate can be initially given to treat shock and later switch over to ORT with low osmolarity ORS.

Hyponatremia Patients who ingest only large amount of water or watery drinks that contain very little salt, may present with hyponatremia (serum sodium < 130 mEq/L, osmolality < 516 275 mOsm/kg), which may be clinically associated with

lethargy and seizures. ORS is safe and effective therapy for hyponatremia as well. For children who are unable to drink orally, IV infusion of Ringer’s lactate can effectively treat hyponatremia.

Hypokalemia Inadequate replacement of potassium losses during diarrhea can lead to potassium depletion and hypokalemia (serum potassium < 3 mEq/L), which may result in muscle weakness, paralytic ileus, renal impairment and cardiac arrhythmias. Severe potassium depletion particularly in malnourished children may lead to acute onset flaccid paralysis ranging from neck flop to quadriparesis and even respiratory paralysis. The potassium deficit can be corrected by using ORS solution for rehydration therapy and by feeding potassium rich foods (e.g. banana, fresh fruit juices) during and after diarrhea. Oral potassium supplementation (2 mEq/kg/day) is indicated in malnourished children. In transient flaccid paralysis due to hypokalemia, potassium can be administered parenterally by using 15% solution of potassium chloride (1 mL = 2 mEq of potassium), but not exceeding 40 mEq/L of IV fluids after ensuring adequate renal functions.

Hypoglycemia Continued feeding during an episode of diarrhea minimizes the risk of getting hypoglycemia. However some children, particularly those severely malnourished, are at a risk of getting hypoglycemia. Early feeding can prevent hypoglycemia in these cases. Sick young infants (less than 2 months) who are not able to breastfed or have low weight for age and a child with symptoms of hypoglycemia should be given 20–50 mL (10 mL/kg) expressed breast milk or locally appropriate animal milk (with added sugar). If neither of these is available, give 20–50 m (10 mL/kg) sugar water. l

particularly because the efforts made by the mother/ caretaker to feed during convalescence are more rewarding when these children tend to have better appetite. Therefore, one extra meal, at least for 2 weeks after an episode of acute diarrhea and for at least 1 month after persistent diarrhea, stressing the need for “catch up growth”, is likely to help in nutritional rehabilitation of these children.

vip.persianss.ir

is as

Diarrheal diseases can be prevented to a great extent by improving infant feeding practices and personal and domestic hygiene. Some of the interventions which are feasible and cost effective include: • Promotion of exclusive breastfeeding up to 6 months of age • Improved complementary feeding practices • Use of clean drinking water and sufficient water for personal hygiene • Hand washing • Use of sanitary toilets • Safe disposal of the stool of young children • Measles vaccination.

s of stinal

e

astroint

G

Rotavirus Vaccines

Sy

K

m and r

iv

L e

Severe dehydration and shock lead to decrease in renal blood flow resulting in prerenal type of acute renal failure. Immediate replacement of fluids is generally helpful to revive kidney functions unless renal failure is irreversible. In case fluid challenge, after rehydration fails to reverse the process, the child needs to be hospitalized and managed as per acute renal failure protocol.

st

Recent studies have demonstrated safety and efficacy of two new live, oral, attenuated rotavirus vaccines in middle and high income countries, thereby suggesting a combined preventive and treatment strategy (vaccine, low osmolarity ORS and zinc supplementation) to significantly reduce child mortality. However, the diversity of rotavirus strains and high prevalence of mixed infections are unique features of rotavirus epidemiology in India. Two new vaccines against Indian rotavirus strains are under review.

e

Acute Renal Failure

Prevention

e

During acute diarrhea, large amounts of bicarbonate may be lost in the stool. If the kidneys continue to function normally, most of the lost bicarbonate is replaced and a serious base deficit does not develop. Metabolic acidosis tends to correct spontaneously in most of the cases as the child is properly rehydrated. ORS solution contains adequate bicarbonate/citrate to counter acidosis in less severe cases. However, in severe dehydration, compromised renal function leads to rapid development of base deficit and metabolic acidosis. Hypovolemic shock occurs as a consequence of rapid loss of water and electrolytes in severe diarrhea. This results in excessive production of lactic acid, which may further contribute to metabolic acidosis. Rapid IV infusion of Ringer’s lactate, containing 28 mEq/L of lactate (metabolized to bicarbonate), is recommended in severe dehydration. However, in the presence of circulatory failure, bicarbonate precursors (e.g. citrate, lactate) may not be readily metabolized in the body. If the patient presents with severe metabolic acidosis, (pH < 7.20, serum HCO3− < 8 mEq/L) sodium bicarbonate in a bolus dose of 2–3 mEq/kg can be given to correct acidosis. Attention should be paid to serum potassium concentration as correction of acidosis in a patient with low potassium can lead to life-threatening severe hypokalemia.

D e

Metabolic Acidosis

ey Messages

• Diarrhea is a self-limiting disorder and does not need any antibiotics for treatment. • Oral rehydration with low osmolarity ORS, continued feeding and zinc supplementation is the key to treatment of diarrhea in children. • Intravenous fluids are indicated in a few cases who have severe dehydration or unable to drink orally. Ringer’s lactate is the most suitable solution for IV therapy. • A child with diarrhea should be thoroughly examined for associated illnesses. • Exclusive breastfeeding up to 6 months of age and hand washing significantly reduce incidence of diarrhea.

Hemolytic Uremic Syndrome Some children with invasive diarrhea due to S. dysenteriae or enteroinvasive E. coli may have HUS due to nephrotoxin liberated by these organisms. These children develop intravascular hemolysis with acute renal failure. This is a serious condition and needs to be managed in a hospital setting.



1. Consensus Statement of IAP National Task Force: Status Report on Management of Acute Diarrhea. Indian Pediatr. 2004;41:335-48. 2. Integrated Management of Neonatal and Childhood Illness. Physician Chart Booklet. Ministry of Health and Family Welfare, Govt. of India; 2009.



­

Acute diarrhea is a self-limiting disorder. Early administration of ORS or home available fluids prevents onset of dehy dration. Appropriate use of low osmolarity ORS in some dehydration and Ringer’s lactate in severe dehydration can prevent all diarrheal deaths.



Bibliography

Prognosis

517

vip.persianss.ir

C

D

C





Persistent and hronic iarrhea in hildren

efinition

E

The World Health Organization (WHO) has defined persistent diarrhea (PD) as a diarrheal illness with passage of three or more loose stools of presumed infectious etiology, starting acutely and lasting for more than 14 days. Intractable diarrhea of infancy often begins before the age of 3 months with more than three liquid stools lasting for more than 2 weeks under 1 year of age with either weight loss or no weight gain during this period.



Three clinical types of persistent diarrhea are recognized: 1. Mild form is characterized by several motions/day without significant weight loss and dehydration and can be managed successfully as outpatients with good follow-up. 2. Moderate form is characterized by several motions/ day with marginal weight loss, without dehydration and non-tolerance to milk and milk products and need frequent admissions due to acute exacerbations with complications, improper treatment and no follow-up. 3. Severe form of persistent diarrhea is often lifethreatening and is heralded by dehydration, weight loss and nontolerance to milk and cereals. Secondary infection often coexists with this category (Figs 9.2.1A and B) and these infants need to stay in the tertiary care hospitals for

Less than 10% of infants are likely to suffer from persistent diarrhea and the risk factors broadly include host factors, disease characteristics, contributed by caregiver or attending physician. The severity may vary from mild to severe type and whatever the type of presentation, the basic defect is small intestinal mucosal damage, infection including small intestinal bacterial overgrowth (SIBO) and malnutrition and growth failure with significant morbidity and mortality depending on the severity.

linical Presentation



D

Persistent iarrhea

C

Gadadhar Sarangi

D

t

t

Tex book of Pedia rics

9.2

tiology

Common causes of persistent diarrhea include persistent infection with one or more enteric pathogens, secondary malabsorption of carbohydrates and fat, intestinal parasitosis and infrequently dietary protein allergy/ intolerance.

a

Pathology

518

B Figures 9.2.1A and B Persistent diarrhea with marasmic kwashiorkor as a result of infection and malnutrition  

Prolonged cell-mediated immune (CMI) form of damage to the small intestinal mucosa is probably the final common pathway by which a variety of noxious influences like nutritional, infective and possibly allergic perpetuate the syndrome of persistent diarrhea in children in developing countries. There is impairment and considerable delay in the repair of damaged epithelium of the gut. Carbohydrate, fat and protein malabsorption ensues as a result of the damage to the upper small intestinal mucosal absorptive surface. The loss of brush border enzymes and direct absorption of macromolecular foreign proteins result in food intolerance and allergy (Cow’s milk or wheat protein allergy). Overgrowth of bacteria in small bowel and altered intestinal flora are also marked as a consequence. Infection is injury to the small intestinal absorptive mucosa and malabsorption of both macro and micronutrients are the untoward events in these unfortunate victims.

vip.persianss.ir

D

t

Criteria for changing over from Plan A to B to C Purge volume and rate more than 7 stools/day at the end of 7 days Tendency for dehydration No weight gain/weight loss despite oral intake of 100 cal/kg/day x 3 days

 

Table 9.2.2 Diets for persistent diarrhea Plan C (chicken based diet for persistent diarrhea)

Ingredient Amount (g) Puffed rice 12.5 Milk 40.0 Sugar 2.25 Oil 2.0 Water to make 100.0 The above will yield following: Energy density 96 Kcal/100 g Protein 10.0% Carbohydrate 55.87% Lactose 1.73% Fat 33.9% Amino acid score 1.0% Note: Puffed rice is ground and appropriate quantities are mixed with sugar and oil. Boiled water is then added to make a thick gruel. This feed has a shelf life of around 3 hours.

Ingredient Amount (g) Puffed rice 13.5 Egg 11.0 Sugar/Glucose 3.5 Oil 3.5 Water to make 100.0 The above will yield following: Energy density: 92.2 Kcal/100 g Protein: 9.5% Carbohydrate: 56.9% Fat: 33.29% Amino acid score: 1.0%

Ingredient













l





l





Chicken 100 g Glucose 20–40 g Coconut oil 40–50 g KCL (15%) 7.5 mL NaHCO3 (7.5%) 20–30 m Total 1000 m The above will yield energy 720 Kcal and protein 26 g









Note: •  It is prepared by grinding the precooked boneless chicken stuff in a mixer. Glucose, oil and some water are added to it and the feed is brought to a boil. Additional Note: water is added to make a final volume of 1 liter. Finally Egg white is added to the mixture of KCl and NaHCO3 are added to safeguard against weighed rice, sugar and oil. Boiled water spoilage it is stored in a refrigerator. is added to make a thick gruel weighing •  Glucose is initially added in 2% concentration and then 100 g. built up to 4% by increasing 1% every alternate day. To reduce osmolar load a mixture of glucose and sugar may be employed. •  Any vegetable oil may be employed in place of coconut oil.











Amount/L















Plan B (egg based milk free diet for persistent diarrhea)



Plan A (milk rice diet for persistent diarrhea)



A

ntimicrobials in Persistent iarrhea

t

5 days onwards (nutritional rehabilitation) Monitor weight Hypo-osmolar, hypoallergenic, home available caloridense non-offending (lactose free) feeds (plan B) in gradual increments depending upon tolerance. If it fails, diet of plan C (chicken/egg white, glucose and oil) along with PPN to be given. If no response, total parenteral nutrition (TPN) will be lifesaving.

t

Phase - III

S

Partial control of diarrhea, sustained maintenance of vital signs Electrolyte, metabolic and hemodynamic balance by partial parenteral nutrition (PPN), intravenous fluids, colloids, parenteral antimicrobials (1–4 days)

t

Resuscitation < 24 hours

Phase - II

The management of persistent diarrhea is given in Tables 9.2.1 and 9.2.2.

These are useful in the presence of gross blood in the stool or >10 pus cells/HPF (quinolones/oral third generation cephalosporins), systemic sepsis (parenteral ampicillin and aminoglycosides), severe malnutrition (ampicillin and aminoglycosides), very young infants (< 3 months of age) associated extra-gut infections (e.g. UTI, LRTI) and HIV.

D

 

Phase - I

L

anagement

Severe persistent diarrhea



M

The emergency risk factors arising out of dehydration, malnutrition and infection should be assessed. Stool examination for culture and reducing sugar with pH will help in management. The effect of previous treatment modalities and diet regimen should be evaluated. The attitude and cooperation of the parents remains the cornerstone in therapy.

Mild persistent diarrhea Try low milk formula feeds (rice, milk, sugar and oil—diet of plan A) Moderate persistent diarrhea Do not try milk. Permit cereal based feeds (Rice/wheat/bengal gram/ragi, sugar, oil—diet of plan B)



D

iagnosis

Table 9.2.1 Management guidelines for persistent diarrhea

iseases of Gas roin es inal ys em and iver

indefinite period till they recover, needing intestinal biopsy, total care including total/partial parenteral and enteral nutrition support with elemental diets. The mortality is still high in most of the centers in developing countries.

519

vip.persianss.ir

D

actobacilli and Saccharomyces Boulardii

These are only adjuncts with little benefit. N

D

I

ndications for Total Parenteral utrition in Persistent iarrhea

upplemental itamins and

M

Protracted diarrhea with recurrent dehydration Intolerance to plan C treatment Weight loss or no weight gain even after plan C treatment. V

inerals

alnourished nfants I

Magnesium sulfate IM and oral potassium in recommended doses should be given for at least 2 weeks.

Prevention Promotion of breastfeeding, active and prompt management of acute diarrhea, and appropriate dietetic management during diarrhea with judicious administration of drugs will prevent PD of infancy. Rotavirus and other enteric vaccines do help in reducing PD. Role of zinc and probiotics in prevention is controversial.

The common causes of chronic diarrhea are given in Table 9.2.3.

valuation

The evaluation should be done in a stepwise manner in order to avoid confusion in diagnosis (Table 9.2.4).

Table 9.2.3 Common causes of chronic diarrhea

F

Predisposing actors

auses

 

everely

M

S

­

About twice recommended daily allowance of supple mental multivitamins and minerals are to be given for at least 2–4 weeks (special attention to be given for vitamin A (200,000 units for children > 12 months or 1,00,000 IU for infants elemental zinc 10 mg/day from 2 months and 20 mg/day above 6 months of age for 14 days. Folic acid (1 mg/day), elemental copper (0.3 mg/kg/day) and vitamin D (200–400 U/day) are recommended.

C

• • •

responsible for digestion as well as secretion. The mechanisms of diarrhea with the involved intestinal sites are as follows: • Osmotic diarrhea in which the undigested nutrients get fragmented to short chain fatty acids and increase the intraluminal osmotic load in colon. It shows good response to fasting. • Secretory diarrhea is one in which due to noxious agents or exotoxins there is increase of intracellular adenosine monophosphate or guanosine monophosphate (GMP) which results in sodium and fluid secretion. • Mutation in apical membrane transport protein like chloride bicarbonate exchange transporter which results in chronic diarrhea from neonatal period with failure to thrive. • Reduction in anatomic surface area of the gut due to extensive resection in necrotizing enterocolitis, midgut volvulus or intestinal atresia results in loss of fluid, electrolyte and nutrients from the gut. • Alteration in intestinal motility as in malnutrition and diabetes mellitus causes secretory diarrhea. • Inflammatory processes like regional enteritis and ulcerative colitis involving a significant portion of the gut causes chronic diarrhea.

E

A L

For Giardia or Entamoeba histolytica trophozoites in the stool (metronidazole).

S

t

t

Tex book of Pedia rics

ntiprotozoal rugs

D

hronic iarrhea D

C

efinition

Chronic diarrhea is defined as diarrhea greater than 2 weeks duration, with an insidious onset and usually due to noninfectious cause. Almost all patients need a complete workup for underlying malabsorptive state.

520

Pathophysiology Chronic diarrhea results from breakdown of intraluminal factors responsible for digestion and mucosal factors

Infancy •  •  •  •  • 

Post-gastroenteritis malabsorption syndrome Protein energy malnutrition Cow’s milk/soy protein intolerance Secondary/primary disaccharidase deficiencies Cystic fibrosis

Childhood •  Excessive consumption of carbonated fluids (Chronic nonspecific diarrhea) •  Secondary disaccharide deficiency •  Intestinal parasites: Giardia, E. histolytica, Cryptosporidium. •  Post-gastroenteritis malabsorption syndrome •  Celiac disease •  Cystic fibrosis •  Intestinal infection: Enteropathogens, Mycobacterium tuberculosis •  Tropical sprue 

Prevention factors during the management of AWD such as bottle feeding and stopping breast milk, inadvertent and empiric use of banned drugs, malnutrition, extragut infections especially non-breastfed infant of less than 4 months of age, immune deficiency, infections like adenovirus, enteroaggregative E. coli (EAggEC), EPEC, Salmonella, Clostridium difficile, Candida, HIV, etc. small bowel overgrowth with significant mucosal atrophy should be focused.

Adolescence •  •  •  • 

Irritable bowel syndrome Inflammatory bowel disease: Crohn’s disease, ulcerative colitis Giardiasis Lactose intolerance

vip.persianss.ir

•

D t t S t





L















n U

ti e



Treatment depends upon the cause. • Restriction of carbonated drinks or excess fruit juice will reduce stool frequency in chronic nonspecific diarrhea. In diarrhea, due to secondary carbohydrate intolerance, reduction of lactose or sucrose in the diet will help. Lactase can be used to aid in digestion of lactose. If diarrhea persists, elimination of lactose/sucrose depending upon the situation is indicated. • If stool examination reveals more fat, malabsorption syndrome (MAS) remains a distinct possibility. Postgastroenteritis MAS needs predigested formula to which a great proportion responds favorably.

G R

V d

Treatment



Hormonal studies: vasoactive intestinal polypeptide 5-hydroxyindoleacetic acid, gastrin, secretin assays



Phase- IV



Endoscopic studies Small bowel biopsy Sigmoidoscopy or colonoscopy with biopsies Barium studies

1. Bhutta ZA, Ghishan F, Lindley K, et al. Persistent and chronic diarrhea and malabsorption: working group report of the second world congress of pediatric gastroenterology, hepatology, and nutrition. J Pediatr Gastroenterol Nutr. 2004;39 Suppl 2:S711-6. 2. Bhutta ZA, Hendricks KM. Nutritional management of persistent diarrhea in childhood: a perspective from the developing world. J Pediatr Gastroenterol Nutr. 1996;22:1737. 3. Cohen SA. Use of nitazoxanide as a new therapeutic option for persistent diarrhea: a pediatric perspective. Curr Med Res Opin. 2005;21(7):999-1004. 4. Donowitz M, Kokke FT, Saidi R. Evaluation of patients with chronic diarrhea. N Engl J Med. 1995;332:725. 5. Fayez K. Ghisshan diarrhea. In: Behrman RE, Kliegman RM, Jenson HB (Eds). Nelson’s Textbook of Pediatrics, 18th edition; 1621-6. 6. Grimwood K, Forbes DA. Acute and persistent diarrhea. Pediatr Clin North Am. 2009;56(6):1343-61. 7. Gupte S, Anderson RA. Persistent diarrhea and chronic diarrhea. In: Gupte S (Ed). The Short Text Book of Pediatrics, 10th edition; 2004. pp. 382-7. 8. IAP Guidelines for management of diarrhea in children; 1994. 9. IAP Specialty series on Pediatric Gastroenterology, Vol. 5 and 6; 2008. pp. 42-76. 10. Lima AA, Guerrant RL. Persistent diarrhea in children: epidemiology, risk factors, pathophysiology, nutritional impact, and management. Epidemiol Rev. 1992;14:222-42.

Phase- III

ibliography



Sweat chloride 72-hour stool collection for fat determination Stool electrolytes, osmolality Stool for phenolphthalein, magnesium sulfate, phosphate, breath H2 test



Phase- II



B

•



Clinical history including specific amounts of fluids ingested per day Physical examination including nutritional assessment Stool exam (pH, reducing substances, smear for white blood cell count, fat, ova, and parasites) Stool culture Stool for Clostridium difficile toxin Blood studies (complete blood cell count, erythrocyte sedimentation rate, electrolytes, blood urea nitrogen, and creatinine)

t

 

Phase- I

Infants presenting with secretory diarrhea in the first month of life need nutritional support as the likely cause is congenital defect in transport proteins. In instances, where chronic diarrhea is a manifestation of a disease, the etiology should be established and specific treatment instituted. Nitazoxanide therapy can be instituted where Giardia lamblia or Cryptosporidium parvum are suspected or found.

iseases of Gas roin es inal ys em and iver

•

Table 9.2.4 Evaluation of patients with chronic diarrhea

521

vip.persianss.ir

C

S





Malabsorption yndrome in hildren

Malabsorption

ntroduction

Malabsorption syndrome (MAS) is defined as the failure of absorption of one or more nutrients. Over the years, the spectrum of MAS in India has changed. Initial studies of 1960s and 1970s were mainly on young (< 5 years) malnourished children and the main focus was on stool examination to isolate infections and infestations. Almost half of those cases were due to gastrointestinal infestations like Giardia. Nevertheless, with improvement of nutritional status, personal hygiene and frequent use of antimicrobials, the prevalence of gastrointestinal infestations (especially Giardia) as a primary cause of MAS has decreased tremendously. Due to increasing awareness, easy availability of diagnostic tests including serology and endoscopic biopsy, the focus has now been shifted from infestation to celiac disease. As a result of which, recent studies have shown that celiac deisease is the commonest cause of MAS in the West as well as in India (especially in North India) both in children as well as in adults.



Maldigestion

n U

The luminal phase of digestion requires pancreatic enzymes, bile salts and the absence of either of them give rises to failure of digestion of carbohydrate (lack of pancreatic amylase), fat (pancreatic lipase and bile salts) and proteins (pancreatic trypsin and chymotrypsin). Similarly, small intestinal bacterial overgrowth (SIBO) due to surgical causes (stricture, fistulae, blind loop, diverticula, etc.) or due to reduced motility of gut (pseudo-obstruction) can cause maldigestion by breaking down bile salts (deconjugation of conjugated bile salts) in the proximal small intestine by bacteria and thereby rendering them (conjugated bile salts) unavailable for mixed micelle formation for fat digestion. Even carbohydrate and protein malabsorption can happen in SIBO, primarily due to bacterial degradation of carbohydrate and mucosal injury by bacterial overgrowth, leading to brush-border enzyme deficiency. 522 Other prominent feature of SIBO is vitamin B12 deficiency, as bacteria utilize vitamin B12.





Malabsorption syndrome is grossly divided into two categories: 1. Impaired breakdown of nutrients (maldigestion). 2. Defective mucosal uptake and transport of adequately digested nutrients (true malabsorption). The latter one may be specific malabsorption of a particular nutrient or a group of nutrients or generalized malabsorption or pan-malabsorption (malabsorption of broad range of nutrients).

G R

V d

ti e

Pathogenesis

Diseases that causes small intestinal mucosal damage like celiac disease, tropical sprue, food protein allergy [like cow’s milk protein allergy (CMPA)], etc. give rise to panmalabsorption of carbohydrate, proteins and fat due to deficiency of brush-border enzymes and lack of transport system located on the apical surface of enterocytes. The important brush-border enzymes for carbohydrate digestion are lactase (lactose malabsorption), maltase (maltose: up to nine residue oligosaccharides) and sucrase (sucrose malabsorption). For proteins, these are oligopeptidases (cleave amino acids from 3–8 residue peptides), dipeptidases and tripeptidases. In malabsorption, the unabsorbed carbohydrate (oligosaccharides, disaccharides like lactose, sucrose) and proteins (oligopeptides, dipeptides and tripeptides) reache colon, where colonic bacteria ferment carbohydrate and produce short chain fatty acids (propionic acid, acetoacetic acid and butyric acid), and gas (hydrogen, methane and carbon dioxide). Short chain fatty acids (2–4 carbon length) in colon produce osmotic effect and draw water into the lumen and the net result is diarrhea and bloating. Bacterial degradation of proteins (especially sulfur containing amino acids) produces odor in flatus (hydrogen sulfide, mercaptan, etc.). Similarly, unabsorbed fat and bile salts produce cathartic effect in colon and cause diarrhea. Congenital absence of selective brush-border enzymes like congenital alactasia and sucrase-isomaltase deficiency gives rise to selective malabsorption of lactose and sucrose, respectively. Post-mucosal phase of digestion is basically transport of fat from mucosa through lymphatics. Lymphatic blockage (congenital: primary intestinal lymphangiectasia or acquired due to lymphoma, tuberculosis, retroperitoneal fibrosis or surgery) gives rise to dilated lacteals in the intestinal mucosa which rupture due to overdistension leading to fat malabsorption (selective malabsorption).

I

Ujjal Poddar

E

t

t

Tex book of Pedia rics

9.3

tiology

The etiology of malabsorption depends on the age and geographic location of the patient. In the West, celiac disease, cystic fibrosis, immune deficiency and Crohn’s disease are the main causes of MAS in children. In India, etiology is different in North from South and also between younger (less than 2 years) and older children. In North India, celiac disease is the commonest cause but in South India, celiac disease is a rare entity. We do not have much information in the literature about the etiology of MAS from south India. In a recent report from Delhi, it has been shown that the majority of children (74%) with MAS were due to celiac disease and gastrointestinal infestations with

vip.persianss.ir

t t t S t m L

I

V d

nvestigations

Approach to MAS can be divided into three stages: 1. Firstly, clinical suspicion of MAS on the basis of history and physical examination 2. Secondly, confirmation of its presence by laboratory tests 3. Lastly, demonstrating its cause by structural tests like endoscopy, mucosal biopsy, imaging, etc.

Yachha et al. (1993) PGI, Chandigarh

Poddar et al. (2010) SGPGI, Lucknow

Behera et al. (2008) AIIMS, New Delhi

N = 62 (< 2 years) %

N = 75 (> 2 years) %

N = 135 (< 5 years) %

N = 50 (up to 12 years) %

73

0

—

—

13

0

29.6

—

Celiac disease

5

43

55.5

74

Parasites

3

15

4 (Giardia)

26 (Giardia, cryptosporidia, isospora)

IBD

—

—

6

—

2

—

Tuberculosis

0

9

0.7

—

Miscellaneous*

6

9

1.5

—

Unknown

0

24

0

—





ti e

n U

D

D

C

F

C

C

G R

and iver

The commonest presentation of MAS in children is chronic diarrhea. However, almost one-fifth of them present without diarrhea with nutritional deficiency signs and symptoms like short stature, anemia, rickets and even constipation. The diarrhea of MAS is usually of small bowel type, i.e. large volume stools with features of carbohydrate (explosive diarrhea, abdominal distension and flatulence), proteins (offensive stools, edema) and fat (steatorrhea) malabsorption. Presence of chronic malnutrition with features of water-soluble vitamins deficiencies (anemia, glossitis, angular stomatitis, etc.) substantiates the clinical suspicion of MAS. In severe cases, features of fat-soluble vitamins deficiency may present. In selective malabsorption, like in lymphangiectasia, the predominant manifestations are of fat malabsorption and loss of plasma proteins and lymphocytes due to rupture of intestinal lymphatic channels (diarrhea with disproportionate edema, lymphopenia, hypokalemia and hypocalcemia).

iseases of Gas roin es inal ys e

linical eatures

F

linical eatures of eliac isease In the West, the age of onset of the disease is 6–12 months and the age of diagnosis is around 18 months. The latent period between introduction of gluten and the onset of symptoms is variable (months to years). In India, it has been reported that the age of onset of symptoms is 2.4–3 years and the age of diagnosis is 6.3–8.3 years. This delay (3–6 years) in diagnosis is mainly due to lack of awareness among parents and pediatricians and the delay in the onset of symptoms may be due to prolonged breastfeeding and delayed weaning. Almost 80–90% cases of celiac disease in India present with typical features of chronic diarrhea (small bowel type with features of malabsorption), with failure to thrive and anemia (Table 9.3.2). However, a proportion of celiac disease cases present with nutritional deficiency features without diarrhea (atypical celiac disease) and attend various specialties (Table 9.3.3). In the West, almost half of the cases of celiac disease do not present with diarrhea (Table 9.3.2). With the increasing awareness and easy availability of diagnostic tests especially celiac serology, the ratio of typical to atypical cases of celiac disease is fast changing. In a recent study from Lucknow, we have shown that 44% of all celiac disease cases are atypical.

pathogenic parasites (like Giardia) are often associated with some underlying causes of MAS like celiac disease. However, a proportion of children (10%) with MAS were due to giardiasis and the other common infestation was Cryptosporidium (14%) which was seen in malnourished young children of less than 2 years of age (71%). Cow’s milk protein allergy is an important cause in less than 3 years of age but tuberculosis is a rare cause of MAS even in a developing country like India. The recent data suggests that inflammatory bowel disease (IBD) and AIDS are becoming common in India too. The etiology of MAS in India is given in Table 9.3.1.

 

Table 9.3.1 Etiology of malabsorption syndrome in children in India Etiology

Protracted diarrhea CMPA

AIDS

Abbreviations: CMPA, Cow’s milk protein allergy; IBD, Inflammatory bowel disease; AIDS, Acquired immune deficiency syndrome. *Miscellaneous: Short bowel syndrome 2, acrodermatitis enteropathica 2, cystic fibrosis 2, tropical sprue 2, trichobezoar 1, nodular lymphoid hyperplasia 1, isolated lactase deficiency 1.

523

vip.persianss.ir

Tex book of Pedia rics

Presentation

George et al. (n= 185)

Mohindra et al. (n = 42)

Poddar et al. (n=300)

Mean age at diagnosis (years)

3.1 ± 3

8.3 (3–14)

6.7 ± 3

Diarrhea

63%

88%

84%

Abdominal distension

55%

—

48%

Growth failure

50%

90%

91%

Undernutrition

60%

90%

92%

Short stature

5.4%

100%

80%

Anemia

5%

90%

84%

Short stature

Endocrinology

Refractory anemia

Hematology

Constipation with abdominal distension

Pediatric surgery

Rickets with fracture and deformity

Orthopedic

Neuropathy, ataxia

Neurology

Infertility, impotence, amenorrhea

Gynecology

V d

S

linical uspicion of Malabsorption yndrome

ti e

S

C

Clinical features of MAS have already been elucidated.

onfirmation of Malabsorption yndrome

Simple laboratory tests help in finding out the presence or absence of malabsorption. The presence and type of anemia is assessed by complete hemogram. Besides anemia some specific features of MAS like lymphocytopenia (in lymphangiectasia), thrombocytosis (in celiac disease), and acanthocytes in peripheral blood film (in abetalipoproteinemia) can be picked up in hemogram. Stool pH (< 5.5) and presence of reducing substances confirm carbohydrate malabsorption. Similarly fat malabsorption is diagnosed by fecal fat estimation (fat globules or fatty acid crystals on microscopy and quantitative fecal fat estimation). Though it is not easily available, fecal alpha-1-antitrypsin estimation is the test for intestinal protein loss. Other tests which are commonly used in MAS are D-xylose excretion test, lactose tolerance and lactose hydrogen breathe test, Schilling test (for vitamin B12 malabsorption).

S

emonstrating the ause of Malabsorption yndrome C

D

­

n U

The third stage of approach to MAS is to find out the cause. Structural tests like endoscopy, small intestinal biopsy, barium meal follow through CT scan, etc. play an important role in finding out the cause of MAS. In addition to structural tests, some other specific tests, like celiac serology, sweat chloride test and mutation analysis help in finding a specific cause. Upper gastrointestinal (UGI) endoscopy and duodenal biopsy are the mainstay of all 524 investigations for MAS. Mere presence of villous atrophy does not give a diagnosis of MAS. Mucosal biopsy is

G R

iagnosis of eliac isease D

Primary specialty

diagnostic (means always positive) in abetalipoproteinemia (fat globule within enterocytes), Whipple’s disease (finding a specific acid fast organism), and agammaglobulinemia (absence of plasma cells in the lamina propria). There are conditions where biopsy changes are diagnostic but patchy (diagnostic if found) like lymphangiectasia (dilated lacteals in lamina propria), giardiasis, strongyloidosis, lymphoma, eosinophilic gastroenteritis, Crohn’s disease, etc. However, in the majority of cases of MAS, the mucosal biopsies are abnormal (show villous atrophy) but not diagnostic of a particular condition like celiac disease, tropical sprue, cow’s milk protein allergy, severe protein energy malnutrition (PEM), prolonged iron and folate deficiencies, etc. Approach to MAS is given in Flow chart 9.3.1. In a suspected case of CMPA, rectal biopsy plays a pivotal role (eosinophilic proctitis: > 6 eosinophils per high power field). In this era of sophisticated investigations, a simple stool examination (consecutive three days) cannot be ignored especially in young malnourished children to document parasitic infestations. C

Presentation

S

C

 

Table 9.3.3 Atypical presentations of malabsorption syndrome

D

t

t

 

Table 9.3.2 Clinical presentation of celiac disease: East versus West

Celiac disease is diagnosed by modified European Society of Pediatric Gastroenterology and Nutrition (ESPGAN) criteria. According to this criteria, small intestinal biopsy should be suggestive of celiac disease; means there should be villous atrophy and the patient should show unequivocal clinical response to gluten-free diet (GFD) in weeks. However, there are many conditions other than celiac disease, which can give rise to villous atrophy, especially in India. Hence, if these criteria are applied in our population then celiac disease will be overdiagnosed. To overcome this problem, additional criteria, over and above ESPGAN criteria are needed. The best option to confirm the diagnosis is gluten challenge but it is cumbersome and requires repeated endoscopic biopsies, and needs parents and child’s co-operation. On the other hand, celiac serology is simple, effective, and if positive at the time of diagnosis and becomes negative on followup on “GFD”, it confirms the diagnosis. The best antibody test is anti-endomysial antibody (EMA) with a sensitivity and specificity of 97%, but it is a technically demanding test (done by indirect immunofluorescent technique). On the other hand anti-tissue transglutaminase (tTG) is almost as good as EMA and it is done by enzyme linked

vip.persianss.ir

 

D

Flow chart 9.3.1 Approach to malabsorption syndrome

t

t

t

iseases of Gas roin es inal ys e

Management



Besides managing the underlying cause of MAS, these patients need supplements with iron, folic acid, multivitamins and calcium. Severely malnourished children should be supplemented with extra calorie and proteins. Caution should be maintained to not to be too aggressive in restarting feeding in a severely malnourished MAS patient as they are at risk of developing “refeeding or nutritional recovery syndrome”. All such patients should be supplemented with thiamine, calcium, phosphate, magnesium, potassium and feeding should be started with half of the daily requirements and to be increased gradually over a week. Parasitic infestations are quite common in MAS patients but many of them are associated with another cause for MAS. Hence, just treatment of isolated parasites (like metronidazole for Giardia, nitazoxanide

S t m L



K

n U

Small intestinal biopsy should show villous atrophy. Celiac serology (EMA or tTG) should be positive. There should be unequivocal clinical response to GFDs in weeks.

for Cryptosporidium) may not cure the condition. All such children should be followed up and if their symptoms persist despite anti-parasitic treatment they should be investigated for underlying diseases like celiac disease or CMPA. In CMPA, besides stopping cow’s milk the attention should be paid to milk products like biscuits, dairy products, butter, ghee, etc. as even a minute quantity of milk proteins can cause persistence or flare of the disease. All such children should be supplemented with calcium as milk is the richest source of calcium. Fortunately CMPA is a transient condition and the majority of children (> 90%) grow out of this condition by 3 years of age. On the contrary, celiac disease is a lifelong condition and the patient (and parents) should be given a clear understanding that the gluten cannot be introduced at any stage.

• • •

V d

ti e

Proposed Criteria to Diagnose Celiac Disease in India

and iver

immunosorbent assay (ELISA) technique. The sensitivity and specificity of tTG is around 95%. In a prospective study in 180 children with celiac disease, we have shown that the tTG has got 95% concordance with EMA and its sensitivity and specificity were 94% and 97%, respectively. Hence, in Indian setting tTG is the best antibody to diagnose celiac disease.

G R

ey Messages

• The etiology of MAS depends on age and geographic location. • Protracted diarrhea due to CMPA and parasitic infestations are common causes in first 2 years of life. • Celiac disease is the commonest cause of MAS in more than 2 years of age in North India. • Consecutive 3 days stool examinations needs to be done in all cases before embarking on sophisticated investigations. • Endoscopy and mucosal biopsy play important role in the diagnosis.

525

vip.persianss.ir





















V d

ti e

n U

G R









B

1. Behera B, Mirdha BR, Makharia GK, et al. Parasites in patients with malabsorption syndrome: a clinical study in children and adults. Dig Dis Sci. 2008;53:672-9. 2. George EK, Mearin ML, Franken HCM, et al. Twenty years of childhood celiac disease in the Netherlands: a rapidly increasing incidence? Gut. 1997;40:61-6. 3. Mohindra S, Yachha SK, Srivastava A, et al. Celiac disease in Indian children: assessment of clinical, nutritional and pathologic characteristics. J Health Popul Nutr. 2001; 19:204-8.



t t

ibliography

4. Poddar U, Thapa BR, Nain CK, et al. Celiac disease in India: are they true cases of celiac disease? J Pediatr Gastroenterol Nutr. 2002;35:508-12. 5. Poddar U, Thapa BR, Nain CK, et al. Is tissue transglutaminase autoantibody is the best for diagnosing celiac disease in children of developing countries? J Clin Gastroenterol. 2008;42:147-51. 6. Poddar U, Thapa BR, Singh K. Clinical features of celiac disease in Indian children: are they different from the West? J Pediatr Gastroenterol Nutr. 2006;43:313-7. 7. Poddar U, Yachha SK, Krishanani N, et al. Cow’s milk protein allergy (CMPA): an entity for recognition in developing countries. J Gastroenterol Hepatol. 2010;25:178-82. 8. Rastogi A, Malhotra V, Uppal B, et al. Aetiology of chronic diarrhea in tropical children. Tropical Gastroenterol. 1999;20:45-9. 9. Sharma A, Poddar U, Yachha SK. Time to recognize atypical celiac disease in India. Indian J Gastroenterol. 2007;26:269-73. 10. Walker-Smith JA, Guandalini S, Schmitz J, et al. Revised criteria for diagnosis of celiac disease. Arch Dis Child. 1990;65:909-11. 11. Yachha SK, Misra S, Malik AK, et al. Spectrum of malabsorption syndrome in north Indian children. Indian J Gastroenterol. 1993;12:120-5. 12. Yadav P, Das P, Mirdha BR, et al. Current spectrum of malabsorption syndrome in adults in India. Indian J Gastroenterol. 2011;30(1):22-8.

Tex book of Pedia rics

• Non-diarrheal manifestations of celiac disease are not so uncommon in India and a high index of suspicion is required to diagnose atypical celiac disease. • Parasitic infestations are common in MAS and many a times they are not the primary cause of malabsorption. • Adequate supplements with minerals and vitamins, and gradual introduction of feed is the key to prevent nutritional recovery syndrome in severely malnourished MAS patients.

526

vip.persianss.ir

V

R



Diseases of Gastrointestinal ystem an

omiting in Children and Gastroesophageal eflux Disease

9.4

I

Shashidhararao Nagabhushana Flow chart 9.4.1 Mechanism of vomiting in children  

ntroduction

Vomiting is a common symptom in pediatric practice associated with not only gastrointestinal but also other system involvement like CNS, renal, psychological, etc. Red flags like persistent/severe vomiting, copious bilious vomiting with colic/visible peristalsis, presence of other signs of raised intracranial pressure and failure to thrive warrant specific evaluation for their cause.

n U

Pathophysiology and tiology E

ti e

pproach to

M

A

The causative mechanisms can be depicted as shown in Flow chart 9.4.1. The common causes of vomiting in children and at different age groups are provided in Tables 9.4.1 and 9.4.2, respectively.

anagement

•

• • •

• • •

An accurate history and thorough physical examination helps in proper diagnosis. The child’s age and the clinical profile dictate the most probable etiology (Table 9.4.2).

•

d

Enquire about the duration, frequency, presence of blood or bile in the vomitus. Ask about associated abdominal pain, recent changes in feeding pattern, changes in urine color, drug consumption and presence of associated fever or altered sensorium. Look out for symptoms and signs attributable to the respiratory, gastrointestinal, urinary and central nervous system in that order. Assess the child for signs of dehydration. In neonates and infants with acute vomiting, the possibility of serious infections like sepsis, meningitis or urinary tract infection needs to be considered and ruled out, i.e. the cause of vomiting may be outside the gastrointestinal tract. In a neonate, vomiting may be the first sign of intestinal obstruction. Vomiting due to benign non-organic causes does not lead to significant dehydration or weight loss. If a behavioral element is present, non-organic causes can be considered. Remember that parental perception of how sick their child is in between episodes of vomiting helps us

L



V d •

iver

Vomiting is forceful expulsion of the stomach contents. It occurs in three phases, i.e. (a) nausea, (b) retching and (c) emesis. In very young children and in those with raised intracranial pressure, vomiting is induced without nausea. Regurgitation (posseting) is effortless expulsion of gastric contents through the mouth. It is common in neonates and infants, is often a developmental process and does not need therapy; symptoms resolve with age and the child thrives well. In contrast, gastroesophageal reflux disease (GERD) is abnormal as in addition to vomiting it is usually associated with complications like aspiration, pneumonia and esophagitis and the child may not thrive well. It therefore needs early recognition and prompt therapy.

S

G R

Definition

 

Table 9.4.1 Etiology of vomiting Common

Less common

Rare

Gastroenteritis/gastritis Motion sickness Acute hepatitis

Surgical abdomen Gastroesophageal reflux Pyelonephritis Cholecystitis Raised intracranial pressure Tumor, intracranial infection Pseudo-tumor Vestibular dysfunction

Inborn errors of metabolism Migraine variant Cyclic vomiting syndrome Chronic renal failure Endocrine causes Diabetic ketoacidosis Addison’s disease Psychogenic

527

vip.persianss.ir

extbook of Pe iatri s

 

c

Table 9.4.2 Common causes of vomiting by age of presentation Newborn

Infant and child

Infections

Sepsis Meningitis

Gastroenteritis Meningitis Respiratory tract infections

Anatomic

Atresia and webs Duplications Malrotation/volvulus

Pyloric stenosis Intussusception

Gastrointestinal

Overfeeding/posseting Gastroesophageal reflux Gastritis-swallowed meconium

Gastroesophageal reflux Gastritis Hepatitis Appendicitis

Renal

Urinary tract infection

Urinary tract infection

Neurologic

Birth trauma

Subdural hematoma Increased intracranial tension Migraine Uremia

Endocrine

Congenital adrenal hyperplasia Diabetes mellitus Acute intermittent porphyria

Others

Cyclical vomiting Toxin ingestion

V d •

I



to determine the seriousness of the illness and the measures to be adopted. The clinical features that indicate common organic causes of vomiting are given in Table 9.4.3.

nvestigations

The history, age of the child and the clinical features on presentation most often guide the appropriate investigations to be done. These include: • Urine for evidence of infection (pus cells, granular casts, bacteria, Gram stain, culture and sensitivity), proteinuria and abnormal metabolites. • Blood for evidence of systemic infection [leukocytosis, toxic granules, band forms, C-reactive protein (CRP), appropriate cultures, etc.]. • Liver function tests. • Renal function tests, electrolyte studies and metabolic screening tests (e.g. lactate, organic acids, ammonia, etc.). • Stool for blood, pus cells, evidence of parasitic infestations. 528

•

anagement

The steps of management include recognition and treatment of the primary causes of vomiting in addition to symptomatic therapy and correction of dehydration (Flow chart 9.4.2). Symptomatic treatment includes stomach wash in neonates and infants, withholding oral fluids for a few hours and gradually restarting in sips. If a child is persistently vomiting, dehydrated or has electrolyte imbalances, IV fluids are indicated. The preferred fluid is Normal saline. Vomiting due to simple gastroenteritis is relieved by a single dose of antiemetic. It is always prudent to remember that organic causes of vomiting do not satisfactorily respond despite adequate doses of antiemetics. In clinical practice, hasty use of an antiemetic without definite diagnosis of the cause has to be avoided. Antiemetics like Metaclopromide or Domperidone hasten stomach emptying and are useful if used judiciously. Ondansetron, a serotonin antagonist, is effective in the treatment of chemotherapy-induced as well as in managing refractory causes of vomiting.

Cyclic omiting yndrome S

n U

ti e

•

Radiological studies: Plain and contrast X-rays of abdomen, ultrasound or endoscopy. Lumbar puncture and CSF analysis in children with clinically suspected intracranial infection. CT or MRI scan of skull and/or sinuses or abdomen as and when indicated.

V

Persistent forceful vomiting Abdominal distension Palpable mass/abdomen or visible peristalsis Failure to gain weight/loss of weight Altered sensorium/failure to accept/demand feeds Bulging fontanels/persistent headache Sudden onset in a well-child/vomiting in an ill child with fever Persistent irritability in an infant with vomiting Persistent copious bilious vomiting

M

 

Table 9.4.3 Features indicating organic causes •  •  •  •  •  •  •  •  • 

G R

Metabolic



T

d

Etiology

Typically cyclic vomiting syndrome (CVS) is seen in schoolgoing children (4–18 years of age) presenting with severe,

vip.persianss.ir

 

Diseases of Gastrointestinal ystem an

Flow chart 9.4.2 Management of vomiting in children

S d L

V

ecurrent omiting

Common causes of recurrent vomiting in children include gastroesophageal reflux (GER) or GERD and functional vomiting due to CVS. Other causes include following chemotherapy, intracranial space occupying lesions (SOL), anatomic obstruction, metabolic causes, psychogenic and organic causes especially of Pancreatic and Renal etiology. In adolescence GERD, functional causes especially migraine, CVS, intracranial SOL and psychogenic vomiting are the leading causes. Management depends upon the cause. It is important first to assess the hydration status and attend to life

Table 9.4.4 Differential diagnosis of cyclic vomiting and chronic vomiting Cyclic vomiting syndrome Chronic vomiting High intensity Low intensity Low frequency High frequency Very high need for IVF and rehydration Not often required Family h/o migraine +ve (5 fold) Low Migraine symptoms +ve with triggers Nil Evaluation of lesions outside gut Gut - peptic esophagitis  

It is mainly clinical with high degree of suspicion especially when there are no positive findings in physical examination and by exclusion. CVS needs to be differentiated from chronic vomiting (Table 9.4.4), gastroparesis and intestinal pseudo-obstruction.

iver

Diagnosis

n U

R

V d

ti e

periodic, discrete, stereotypical, short term, early morning or nocturnal, self-limiting episodes with abdominal pain with high intense retch and apprehension. In 70–80%, a uniform symptom-free interval of 4 weeks in children and 3 months in adults is noted. Associated headache, sometimes with family history of migraine gives a clue for diagnosis. Alarming features are absent except dehydration needing IV fluids.

G R

Management Cyclic vomiting syndrome was previously thought to be a migraine variant (because of positive family history and response to anti-migraine treatment), but the current view is that both are separate clinical entities. Treatment of CVS consists of rest, IV rehydration with dextrose saline administration and Ondansetron and preventers such as Amitriptyline and Cyproheptadine.

529

vip.persianss.ir

•

Gastroesophageal eflux

•

•

R

• •



•



B























1. Anderson CM, Burke V, Gracey M. Pediatric Gastroenterology, 2nd edition. Oxford: Blackwell Scientific Publication; 1987. p. 86. 2. Flake ZA, Scalley RD, Bailey AG. Practical selection of antiemetics. Am Family Physician. 2004;69:1169-74, 1176. 3. Graef JW. Manual of Pediatric Therapeutics, 4th edition. Boston: Little Brown Company; 1988. pp. 33-34. 4. Illingworth RS (Ed). Common Symptoms of Disease in Children, 9th edition. Oxford: Blackwell Scientific Publication; 1998. pp. 69-82. 5. Indian Academy of Pediatrics. Vomiting: In: Rapid Approach to common symptoms- Standardization of pediatric office practice; 2005. pp. 51-53. 6. Kliegman RM, Behrman RE, Jenson HB, Stanton BF (Eds). Nelson’s Textbook of Pediatrics, 19th edition. Philadelphia: Saunders Elsevier; 2004. pp. 1199-200. 7. McIntosh N, Helms P, Symth R, Logan S (Eds.). Forfar and Arneil’s Textbook of Pediatrics, 7th edition. Edinburgh: Churchill Livingstone; 2008. pp. 658-63. 8. Rudolph AM, Hoffmann JIE, Rudolph CD (Eds). Approach to a child with acute, chronic or cyclic vomiting. In: Rudolph”s Pediatrics, 20th edition. USA: Prentice Hall International Inc, 1991;(2):1523-24.

Physiological GER in infants, being a benign condition, needs often lifestyle modifications (LSM) like positioning (head up prone position at 45° angle), small, frequent, thick feeds; and in severe cases nasogastric drip feeds, addition of rice cereal and prokinetics (e.g. domperidone). Acid inhibitors like Ranitidine 3–5 mg/kg/dose twice or thrice daily along with LSM for non-erosive reflux.



ER

ER

O

D





•

n U and G

G R

ibliography

V d

ti e

M

•

anagement utlines of G

Proton pump inhibitors (PPI) 1 mg/kg/day 1 hour before breakfast along with LSM for relief of symptoms of erosive esophagitis and healing are recommended. Barrier agents like Sucralfate and Alginates may give symptomatic relief. Antacids are outdated in view of better and effective drugs being available. Prokinetic drugs like levosulpiride, cinitapride are useful in adolescents and adults only. Extraesophageal manifestations of GERD need specific tests to confirm the etiology and more aggressive treatment with twice daily long-term PPI (for at least 3 months) and follow-up. Indications for surgery include refractoriness to adequate medical treatment, life-threatening complications like aspiration pneumonia, failure to thrive, dysphagia, odynophagia, hematemesis, anemia, and rarely Barrett’s ulcer especially in adolescents. In impaired neurodevelopmental children, Nissen fundoplication laparoscopic or endoscopic techniques are popular modalities of treatment.



Gastroesophageal reflux is one of the common causes of chronic abdominal pain in children with increasing incidence (1–8%). Children often suffer from involuntary passage of gastric contents into esophagus. Pathological GER is GERD characterized by symptoms like irritability, abdominal colic, epigastric or retrosternal burning pain, failure to thrive, GI bleed, dysphagia or odynophagia, belching, apnea, satiation, extraesophageal manifestations such as globus, throat clearing, recurrent aspirations, nocturnal cough or wheeze, dental erosions and caries of teeth. Regurgitation with abnormal posturing in neonates and very young infants is termed Sandifer’s syndrome. The high-risk group includes children with neurological abnormalities, obesity, transient LES relaxations (TLESR), asthma and those with nocturnal refluxing. Clinically regurgitation of milk lasts up to 3–4 months of life and many get over it by 12–24 months. Chronic untreated esophagitis is rare and can result in strictures, Barret’s esophagus and adenocarcinoma in adults. Diagnostic modalities include barium swallow which has low sensitivity and specificity, 24-hour ambulatory esophageal pH monitoring (which however cannot detect non-acid reflux) and nuclear scintigraphy (99mTc milk scan). These are especially useful in children with GERD-associated asthma or pneumonia. Upper GI endoscopy grading and biopsy are useful for Barret’s esophagus. Other special tests such as esophageal manometry and impedance for bolus nonacid reflux are done depending on specific situations.

T

d

c

extbook of Pe iatri s

threatening complications before going in for specific investigations for etiology. Always ascertain whether the vomitus is bilious, especially copious as in GI obstruction, bloody or contaminated with blood.

530

vip.persianss.ir





Diseases of Gastrointestinal ystem an

9.5

Parasitic Bowel Disease BD Gupta

Definition



pidemiology

n U

Parasitic bowel diseases are group of infectious diseases due to protozoa and helminths, and are a major cause of morbidity in infants and children in many parts of the world. Parasitic bowel diseases may be classified according to their etiological agents as under: • Protozoan diseases: Due to Entamoeba histolytica, G. lamblia, Balantidium coli, Cryptosporidium parvum, Blastocystis, Isospora belli, Cyclospora cayetanensis and Microsporidia. • Helminthic diseases: Due to – Nematodes: Ascaris lumbricoides, Enterobius vermicu­ laris, hookworms, i.e. Ankylostoma duodenale and Necator americanus, Trichinella spiralis, and Trichuris trichiura. – Trematodes: Fasciolopsis buski, Nanophyetus salmincola, and Heterophyes heterophyes. – Cestodes: Taenia solium, Taenia saginata, Diphyllo­ bothrium latum, Hymenolepis nana, and Echinococcus granulosus. E

tiopathogenesis

Parasitic bowel diseases are endemic in areas of world with poor levels of sanitation and low socioeconomic standards.

S d L



­



Clinical eatures F

–

–

–



V d

ti e

Classification

G R

iver

Intestinal parasitic infestation is more common in developing countries, though worldwide the vulnerable groups are children of both urban poor and rural population. Parasitic infestation is seen in both asymptomatic and symptomatic in a hospital-based study. A study on rural and urban school children showed an infection parasitic infestation rate of 91% and 33%, respectively. Most often the larva or ova of intestinal parasites are passed intermittently in stools and hence repeated fresh stool examination of stools are recommended for better yield rate especially in immune deficient children when we screen them for parasites causing diarrhea such as Giardia lamblia, Cryptosporidium, and S. stercoralis and so on.



E

A parasite is an organism that grows, feeds and is sheltered by or within a different organism while contributing nothing to survival of its host. Unlike protozoa, most helminths do not multiply within the human body, except Strongyloides stercoralis.

Two distinct modes of transmission are known, namely feco-oral route and cutaneous route. 1. Protozoa like E. histolytica, G. lamblia and B. coli infect humans by ingestion of cysts while nematodes like A. lumbricoides, T. trichiura, E. vermicularis and cestodes like T. saginata, T. solium and D. latum spread by ingestion of contaminated food and water with eggs of respective parasites. 2. Hookworms like A. duodenale and N. americanus enter the human body through skin penetration by their larvae. These larvae undergo extraintestinal migration through the venous circulation and lungs before they are swallowed to reach intestines. Various parasites localize themselves at various sites in small as well as large intestine as per their suitable environment, e.g. E. histolytica dwell in colon, G. lamblia colonize in the lumen of duodenum and proximal jejunum, B. coli infests the large intestine, A. lumbricoides in small intestine, T. trichiura in cecum and ascending colon, E. vermicularis typically inhabits cecum, appendix and adjacent areas of ileum and ascending colon. Various parasites cause symptoms due to invasion (E. histolytica), obstruction (A. lumbricoides), reduced absor ptive surface (G. lamblia) and blood sucking (Hookworms). It has been observed that parasitic bowel disease have a role in prevention of IBDs like Crohn’s disease, which are most prevalent in highly industrialized countries with temperate climate and occur rarely in tropical third world countries with poor environmental sanitation. Many helminths live within and migrate through human gut and interact with the mucosal immune system. The host mounts a mucosal response including Th2 cytokine production limiting helminthic colonization, as helminthes and their eggs are probably most potent stimulus of mucosal Th2 response. This may modulate immune reaction to parasitic bacterial and viral infections. Perhaps, failure to acquire these parasites and experience mucosal Th2 conditioning predisposes to Crohn’s disease, which is an overtly active Th1 inflammation.

Parasitic bowel disease is associated with wide variety of clinical manifestations ranging from asymptomatic carrier stage to various intestinal and extraintestinal manifestations. The clinical features depend largely on the parasite, site of involvement, mechanical factors and interference with host’s nutrition.

531

vip.persianss.ir

S



Most of parasites present with pain abdomen and diarrhea which can be acute, chronic or recurrent; bloody or nonbloody; associated or not associated with tenesmus, abdominal cramps, bloating, flatulence, etc. Other symptoms include nausea, vomiting, anorexia, weight loss, fever, abdominal distension, malaise, myalgia, headache, etc. Various other clinical features caused by different parasites are summarized in Table 9.5.1. L

aboratory Diagnosis

Parasitic bowel diseases can be diagnosed by examination of stool samples under direct microscopy. Repeated

fresh samples may be required to reach diagnosis of clinically suspected organisms. The stool examination may be supported by blood examination for evidence of eosinophilia and various serological tests specifically designed for the organism under consideration. Stool examination along with endoscopically obtained smears and tissue biopsy helps a lot in diagnosis of specific parasitic infestation. Ideally fresh stools should be examined within 30 minutes of passage for evidence of trophozoites/cysts in case of E. histolytica or G. lamblia. Stool samples preserved in polyvinyl alcohol helps in diagnosis of these organisms. Serological tests like indirect hemagglutination are available for E. histolytica. Enterotest on duodenal fluid for giardiasis is another alternative. Stool examination and demonstration of oocysts helps in diagnosis of spore forming intestinal protozoa like Cryptosporidium, isospora, cyclospora, etc. Other tests for these parasites are enzyme immunoassay, indirect immunofluorescence and polymerase chain reaction (PCR). Demonstration of eggs of helminthes in stool is the mainstay of diagnosis of most of parasites. For ascariasis, Kato’s thick smear examination of stool is easy and sensitive method. Fertilized eggs signify infection with both male and female worms while unfertilized eggs show infection with female worm only. Enterobiasis can be diagnosed by examining cellophane tape imprint from perianal area. For trichinosis serologic tests like Bentonite flocculation test, muscle biopsy, levels of muscle enzymes like creatine kinase and lactic dehydrogenase (LDH) help in diagnosis.

M

 

Table 9.5.1 Clinical manifestations Clinical manifestation Parasitic bowel disease(s) Ascariasis, giardiasis, and infection by Nutritional deficiency intestinal flukes (Vitamin A deficiency) Anemia Hookworm disease Iron deficiency Diphyllobothrium latum B12 or folic acid deficiency (Diphyllobothriasis), trichuriasis Giardiasis, ascariasis, hookworm disease Malabsorption syndrome and infestation by intestinal flukes Giardiasis, hookworm disease, Weight loss diphyllobothriasis and spore forming protozoa like cryptosporidiasis, isosporiasis and cyclosporiasis Ascariasis, taeniasis Intestinal obstruction Giardiasis, trichuriasis Rectal prolapse Extraintestinal involvement Amebiasis, intestinal flukes Liver Trichinosis, flukes, spore forming protozoa, Muscles e.g. microsporidia Cutaneous larva migrans—hookworm, Skin Strongyloides stercoralis Amebiasis, trichinosis, microsporidiasis, Brain taeniasis—neurocysticercosis Amebiasis, ascariasis, hookworm disease Lungs Strongyloides stercoralis Associated parasites: Cryptosporidium Immunodeficiency states parvum, Isospora belli, Cyclospora cayetanensis, microsporidium (AIDS), giardiasis, amebiasis





T

b

d

c

ext ook of Pe iatri s

General ymptoms

anagement

Treatment of various parasitic infestations is summarized in Table 9.5.2.

Prevention and Control Parasitic bowel diseases essentially are much more prevalent in areas of poor sanitation and environmental conditions. Hence, these diseases can be prevented by following measures, viz. safe disposal of human excreta, safe water supply, proper food hygiene, personal and community hygiene, health education, and early diagnosis and treatment of symptomatic and asymptomatic cases.

532

vip.persianss.ir

 

Diseases of Gastrointestinal ystem an

Table 9.5.2 Common parasitic infections and their treatment Etiological agent Major clinical features Treatment Entamoeba histolytica

Diarrhea Dysentery Liver abscess

Giardia lamblia

Diarrhea Malabsorption

Isospora belli

Cyclospora Microsporidium

Strongyloides stercoralis

Enterobius vermicularis

Trichuris trichiura

Hookworm infection (Ankylostoma duodenale, Necator americanus) Hymenolepis nana

Trichinella spiralis Diphyllobothrium latum

S

iver

Ascaris lumbricoides

Quinacrine 2 mg/kg tid orally for 5 days Albendazole 400 mg OD for 5 days Tinidazole 50 mg/kg once Diarrhea Tetracycline 40 mg/kg q 6 hours for 10 days (>8 Dysentery years) Painful abdomen Iodoquinol 40 mg/kg/day q 8 hours PO (10 days) Severe diarrhea with Nitazoxanide 100 mg bid orally for 3 days Paromomycin 1 g bid orally + malabsorption in AIDS Azithromycin 600 mg/day orally for 4 weeks patients followed by Paromomycin 1 g bid orally for 8 weeks As above Trimethoprim 5 mg/kg/dose + Sulfamethoxazole Ciprofloxacin or pyrimethamine +/− folinic acid 25 mg/kg/dose 8 hourly for 10 days; then bid for in sulpha-intolerant patients 3 weeks As above Trimethoprim 5 mg/kg/dose + Sulfamethoxazole Ciprofloxacin 25 mg/kg/dose bid orally for 7 days As above Albendazole 400 mg bid for 3 weeks Nitazoxanide 7.5 mg/kg bid for 3 days Atovaquone Piperazine citrate 15 initially followed by 65 mg/ Abdominal pain Albendazole 400 mg orally once Cough Mebendazole 100 mg bid orally for 3 days or 500 kg/dose 12 hourly for six doses Ivermectin 200 mcg/kg/day od orally for 1–2 Nausea mg once days Pyrantel pamoate 11 mg/kg once Loeffler like syndrome Ivermectin 200 mcg/kg/day once orally for 1–2 Thiabendazole 50 mg/kg bid orally for 2 days days Albendazole 400 mg once for 2 days Abdominal pain Diarrhea Malabsorption Ivermectin 200 mcg/kg/day orally for 1–2 days Pruritus ani Pyrantel pamoate 11 mg/kg once Sleeplessness Mebendazole 100 mg bid PO for 3 days Albendazole 400 mg once (Therapy to be repeated after 2 weeks Chronic dysentery Mebendazole 100 mg bid orally for 3 days or 500 Albendazole 400 mg once Rectal prolapse mg once Anemia Chronic dysentery Albendazole 400 mg/day orally once Pyrantel pamoate 11 mg/kg/day once for 3 days Rectal prolapse Mebendazole 100 mg bid for 3 days Anemia Praziquantel 25 mg/kg orally once (if available) Albendazole 400 mg/day for 3 days Abdominal pain Nitazoxanide 7.5 mg/kg bid orally for 3 days Loss of appetite Diarrhea Anemia Hypoalbuminemia Abdominal pain Mebendazole 200–400 mg tid orally for 3 days Albendazole 400 mg bid orally for 8–14 days Discomfort then 400–500 mg tid for 10 days Praziquantel 5–10 mg/kg orally once Niclosamide 50 mg/kg once Diarrhea Fever Periorbital edema Myalgia Megaloblastic anemia Leukopenia Thrombocytopenia

d

Cryptosporidium

Ornidazole 30–50 mg/kg/day orally in two doses Nitazoxanide 7.5 mg/kg twice daily for 3 days

L

Balantidium coli

Metronidazole 30–50 mg/kg/day orally in 3 doses for 10 days Diloxanide furoate 20 mg/kg/day orally in 3 doses for 10 days Dehydroemetine 1 mg/kg/day SC or IM daily for 7–10 days Metronidazole 5–10 mg/kg tid orally for 5 days Furazolidone 6 mg/kg/day q 6 hours for 10 days Nitazoxanide 7.5 mg/kg twice daily for 3 days Metronidazole 45 mg/kg/day q 8 hours orally for 5 days

Alternative Drug(s)

533

vip.persianss.ir





















1. Chen X-M, Keithly JS, Paya CV, et al. Cryptosporidiasis. N Engl J Med. 2002;346:1723-31. 2. Conteas CN, Berlin OG, Ash LR, et al. Therapy for human gastrointestinal microsporidiasis. Am J Trop Med Hyg. 2000;63:121-27. 3. Elliott DE, Urban JF, Arco CK, et al. Does the failure to acquire helminthic parasites predispose to Crohn’s disease. The FASEB Journal. 2000;14:1848-55. 4. Kliegman RM, Behrman RE, Jenson HB, Stanton BF (Eds). Infectious diseases. Nelson’s Textbook of Pediatrics, 18th

edition. New Delhi: Saunders Elsevier Indian Edition; 2008. pp. 1448-519. 5. Pankaj V. Intestinal parasites in children. IAP Specialty Series on Pediatric Gastroenterology, Vol. 9. New Delhi: Jaypee Brothers Medical Publishers (P) Ltd; 2008. pp. 107-24. 6. Park K. Intestinal infections. In: Park K (Ed). Park’s Textbook of Preventive and Social Medicine, 21st edition. Jabalpur: Banarsidas Bhanot; 2011. pp. 167-205. 7. Tripathi KD. Antiamoebic and other antiprotozoal drugs and anthelmintic drugs. In: Tripathi KD (Ed). Essentials of Medical Pharmacology, 6th edition. New Delhi: Jaypee Brothers Medical Publishers; 2010. pp. 797-816.

T

b

d

c

ext ook of Pe iatri s

Bibliography

534

vip.persianss.ir

Gastrointestinal Bleeding in Children





Diseases of Gastrointestinal ystem an

9.6

E



•

• • •

S

L

L

d

­

Tables 9.6.1 to 9.6.3 list the common causes of gastro intestinal bleeding in children.

allory-Weiss ears

It occurs at the gastroesophageal (GE) junction primary on the gastric side, resulting, retching or coughing is classically reported prior to hematemesis. It is more common in adults and is less frequently seen with children.

Hemorrhagic and rosive Gastropathy It refers to subepithelial hemorrhage and erosions. These are restricted to the mucosa, where no large blood vessels are present and therefore do not cause major bleeding. Erosions are reported in around 2–10% of patients with upper gastrointestinal bleeding (UGIB). The most common causes are nonsteroidal anti-inflammatory drug (NSAID) use and stress. Prevalence in neonatal intensive care unit (NICU) is around 44% but out of these only 5% are clinically significant.

Table 9.6.1 Causes of upper gastrointestinal bleed  

•

tiology

iver

•

Upper GI bleeding is defined as overt or occult bleeding from a source in the esophagus, stomach or duodenum proximal to the duodenojejunal junction (ligament of Treitz) and lower GI bleeding occurs distal to the duodenojejunal junction. Obscure GI bleeding is overt GI bleeding from remote sites where both upper GI endoscopy and ileocolonoscopy do not reveal the cause for GI bleeding. Spurious hematemesis can present as swallowed blood in a neonate or the source can be from oral cavity or as part of malingering/attention seeking ill health behavior. Major GI bleeding is alarming with overt clots of blood, tarry stools (melena) with over 100 ml of bleeding, postural hypotension/shock needing at least two units of blood transfusion. The site of GI bleeding is categorized as: Upper GI bleeding: Bleeding above the ampulla of Vater. Mid GI bleeding: GI bleeding occurs between ampulla of Vater and terminal ileum ower GI bleeding refers to colonic GI bleeds. L

•

E

Definition

Upper GI bleeding is an indication for UGI endoscopy in 5% of children and 5% of total GI bleeding may present as obscure GI bleeding. ower GI bleeding needing hospitalization, is only one-third of upper GI bleeding patients. Etiological spectrum varies in neonates, infants and older children (Table 9.6.1). A meticulous history, physical examination and step by step evaluation is necessary in a tertiary care center preferably by trained pediatric gastroenterologist in association with team of imaging, nuclear medicine and intervention specialists and sometimes surgeons.

E

Gastrointestinal (GI) bleeding—upper or lower—refers to the pathology either proximal or distal to the ligament of Treitz and if obscure, massive and recurrent, it will be frightening and poses a challenge to both pediatrician and gastroenterologist. A systematic diagnostic and definite therapeutic approach is essential not to delay diagnosis, to do relevant and specific investigations for appropriate treatment in tertiary medical care center.

pidemiology

T

ntroduction

M

I

Neelam Mohan

Newborn

Infant

Children

•  •  •  •  •  •  • 

•  •  •  •  •  •  •  • 

•  •  •  •  •  •  •  •  •  •  •  • 

Swallowed maternal blood Hemorrhagic disease of newborn Stress gastritis Cow’s milk protein allergy Acid peptic disease Vascular anomaly Coagulopathy

Stress ulcer/gastritis Acid-peptic disease Mallory-Weiss tear Vascular anomaly Duplication cyst Varices Webs Intestinal obstruction

Abbreviation: GERD, Gastroesophageal reflux disease

Mallory-Weiss tear GERD/Acid-peptic disease Varices Stress ulcer/erosive gastritis Caustic injury Foreign body Vasculitis Crohn’s disease Intestinal obstruction Dieulafoy lesion Hemobilia Pancreatic pseudoaneurysm

535

vip.persianss.ir

Older child •  Inflammatory bowel disease •  Ischemic/collagenous colitis •  Vascular malformations •  Intestinal duplication •  Bleeding diathesis •  Henoch-Schonlein purpura •  Hemolytic uremic syndrome •  Hemorrhoids, rectal varices •  Perianal cellulitis •  Rectal prolapse •  Solitary rectal ulcer •  Hereditary polyposis syndrome •  Sexual abuse and anorectal trauma

Peptic Ulcer

V

–

–

–

–

–

–

S

Incidence of bleeding due to NSAID induced erosive gastritis is common but bleeding ulcers appears to be rare compared to adults. Approximately one-third of patients found to have an ulcer with active bleeding or a nonbleeding visible vessel will have further bleeding requiring surgery if treated expectantly. These patients should receive endoscopic therapy and IV infusion of PPI. In less than 10 years of age, around 77% of the peptic ulcer disease (PUD) is in duodenum. Hematemesis and perforation are more in secondary PUD. Factors associated with PUD include: • Primary: Related to Helicobacter pylori, bile reflux • econdary: Related to – NSAID intake – Stress: Shock, ischemia – Drugs – Corrosives – Ménétrier’s disease – Zollinger-Ellison syndrome.

ariceal Bleeding

In country like India, portal hypertension is caused by extrahepatic portal vein obstruction [(EHPVO (68–76%)], cirrhosis (24–28%); and infrequently due to congenital hepatic fibrosis (3%), non-cirrhotic portal fibrosis and Budd Chiari syndrome. Extrahepatic portal vein obstruction is also the most common cause of GI bleeding in children (70%). Predictors of variceal bleed include large varices and presence of red color signs (red wale marking, diffuse 536 redness, and hematocystic malformation).



O

V

E

L

T

Infant •  Vascular lesions •  Bleeding diathesis •  Hirschsprung enterocolitis •  Meckel diverticulum •  Malrotation with volvulus •  Intestinal duplication •  Intussusception

The mean age of presentation is 5–6 years. In India, 83% of patients with EHPVO present with UGIB before the age of 20 years. Hematemesis with or without melena is the commonest presentation; only 8–10% patients may not bleed. UGI bleeding is massive and recurrent but risk of rebleeding after major episode is less than cirrhosis but is fairly uniform and occurs once in every 2 years. The average number of bleed is 2.5–5 episodes per patient. Firm splenomegaly is almost universal in patient with EHPVO. It can be present as early as 1 month of age and is usually seen before 3 years of age. Splenomegaly is mild (< 6 cm) in 42%, moderate (6–10 cm) in 40% and massive (> 10 cm) in 18%. Children with EHPVO do not grow as do their healthy siblings. Variceal bleeding in EHPVO is well tolerated with reference to the liver function, viz. following the GI variceal bleeding, they usually do not develop encephalopathy even with massive GI bleed and they have normal liver functions. They may develop transient ascites following major bleeding episode. Persistent or massive ascites in children with EHPVO should doubt about the diagnosis or suggest the possibility of presence of coexistent cirrhosis. Recurrent GI bleeding in EHPVO decreases with age. Abdominal ultrasonography and Doppler study are diagnostic. Children with EHPVO when compared to chronic liver disease (C D) have more chances of UGI bleed (61.6% versus 14.7%), more previous bleeding episodes (2.7 versus 1.2), long duration of symptoms (26 versus 12 months), and absence of jaundice, preservation of liver function and less Hb value.

b

 

Table 9.6.3 Less common causes of rectal bleeding in children

xtrahepatic Portal ein bstruction and Portal Hypertension

E

Older child •  Anal fissure •  Intussusception •  Infectious enterocolitis, Amebiasis •  Meckel diverticulum •  Juvenile polyp

valuation of a Gi Bleeder

Vital signs include the pulse rate and the blood pressure in lying, sitting and if possible, in standing position for postural hypotension. It is essential to monitor urine output and fluid intake and respiratory rate throughout fluid resuscitation of patients in shock and altered sensorium/ coma. Hyperventilation is an early sign of a developing acidosis. Physical examination to evaluate capillary perfusion, skin color for the presence of cyanosis, pulse, blood pressure, respiratory pattern, and level of consciousness should continue on an ongoing basis during active bleeding. Nasogastric aspirations that are grossly bleeding confirm upper GI sources but a negative aspiration does not rule out. Some clinical clues to etiology in a GI bleed patient are given in Table 9.6.4. Presence of melena followed by any bright colored blood in stool indicates rebleed and these patients of GI bleeding with melena should be monitored and managed in the pediatric intensive care unit.

 

c d

Infant •  Anal fissure •  Milk protein intolerance •  Necrotizing enterocolitis •  Swallowed maternal blood •  Vitamin K deficiency

Gastric varices are found most commonly with splenic vein thrombosis or after endoscopic sclerotherapy (EST) of esophageal varices.



ext ook of Pe iatri s

Table 9.6.2 Common causes of rectal bleeding in children

vip.persianss.ir

 

Clinical clues

Mallory-Weiss tear

Emesis before hematemesis, pain +

Esophageal ulcer

Odynophagia, GERD, H/O pill ingestion

Stress gastritis/PUD

Sick patient in ICU, respiratory failure/NSAID ingestion/pain

Angiodysplasia

Renal failure, hereditary hemorrhagic telangiectasia

Aortoenteric fistula

H/O aortic aneurysm or surgery

Variceal bleed

Significant, painless, splenomegaly, jaundice, stigmata of CLD, ascites

erlipressin: This is a synthetic analog of vasopressin and acts by immediate vasoconstriction. Its use in children requires further evaluation though found to be more effective in controlling bleeding (up to 79%) than vasopressin without any adverse side effects. It can be given as IV injections (2 mg) every 4 hours till bleeding free interval of 24–48 hours is achieved. • omatostatin: It acts by inhibiting release of several vasodilatory hormones such as glucagon. It induces selective splanchnic vasoconstriction. The recommended dosage is one to three bolus injections (250 µg/bolus) during first hour of therapy followed by infusion of 250 µg/hour of continuous infusion for 2–5 days. There is lower failure rate and complications in comparison to vasopressin but the disadvantage is its short half-life. • ctreotide: It is a synthetic analog of somatostatin with half-life of 90 minutes. In children the dose is 1–2 µg/kg over 2–5 min, then 1–2 µg/kg per hour for 5 days. Sideeffects are uncommon.

anagement esuscitation

T

S

Balloon Tamponade

V

A

Control of cute ariceal Bleeding It can be by the following modalities:

Pharmacotherapy

V

The most widely used agents to stop variceal bleeds are: • asopressin: It is a potent non-selective vasoconstrictor. It lowers the portal pressure by causing splanchnic arterial vasoconstriction and reducing the splanchnic blood flow to the varices. It is given in a bolus of 1 unit per 3 kg of body weight diluted with 2 ml/kg of 5% dextrose given over a period of 15–20 minutes. Nitroglycerine, a vasodilator, may reduce the cardiac side effects of vasopressin by increasing local concentration of NO and reducing cardiac output.

L

L

d

Sengstaken-Blackmore tube (three lumen and two balloons for esophageal and gastric variceal bleed), inton-Nachlas tube (three lumen and single balloon more effective for gastric variceal than esophageal bleed) and the Minnesota tube (four lumen and two balloons) are handled by only by experienced specialists as a lifesaver in active variceal bleeding if emergency sclerotherapy or banding is unavailable or not technically possible because visibility is obscured. In patients with active bleeding, an endotracheal tube should be inserted to protect the airway before attempting to place the esophageal balloon tube. Continued bleeding during balloon tamponade indicates an incorrectly positioned tube and bleeding from another source. After resuscitation, and within 12 hours, the tube is removed and endoscopic treatment repeated.

iver

This includes achieving hemodynamic stability and treatment of hypovolemic shock. Adequate IV access should be established and carefully monitor pulse, blood pressure and central venous pressure. Oxygen is given to counter hypoxia due to acute blood loss. Nasogastric aspiration is done to know the magnitude of bleeding, to clear the stomach for endoscopy and to prevent hepatic encephalopathy.

Endotherapy (Endoscopic Variceal Ligation or Sclerotherapy) •

ndoscopic variceal ligation: Using multiband ligator banding has less complications and is more effective than sclerotherapy. However it is not possible in small children between 2 and 3 years of age (Table 9.6.5). E

R

M

Abrreviations: GERD, Gastroesophageal reflux disease; PUD, Peptic ulcer disease; NSAID, Nonsteroidal anti-inflammatory drug; CLD, Chronic liver disease

O

S

Bleeding etiology

•

Diseases of Gastrointestinal ystem an

Table 9.6.4 Clinical clues to etiology in a gastrointestinal bleed patient

 

Table 9.6.5 Comparison of endoscopic variceal ligation (EVL) and endoscopic sclerotherapy (EST) EVL

EST

Indications

Large varices, difficult in small children

Any size

Complications

Less (4%)

More (25%)

Rebleeding rate

Low (4%)

More (25%)

Sessions needed

More (17%)

Less (10%)

Recurrence of varices

Increase

IGV and GOV

Gastric varices

Increase

Increase

537

vip.persianss.ir

I

A

pecific anagement of Common Conditions Presenting as G Bleeding •

I

nal fissure: Treatment of constipation, laxatives, Sitz bath, topical 0.2% glyceryl nitrate twice daily • olitary rectal ulcer syndrome: Sucralfate enema, twice daily, for 3–6 weeks with training on “not to strain during defecation” and yttrium-aluminum garnet (YAG) laser treatment for severe bleeds. • Hemorrhagic infective colitis: Antimicrobials. • Polyps: Colonoscopic snare polypectomy. • nflammatory bowel disease and ulcerative colitis: 5 amino-salicylic acid, steroids, cyclosporine, total colectomy. • Crohn’s disease: Steroids, azathioprine, infliximab, metronidazole and ciprofloxacin. • ascular lesions: Therapeutic endoscopy. • Portal colopathy and variceal bleed: Octreotide, transjugular intrahepatic portosystemic stent shunt and shunt surgery. bscure G Bleed It may occur as overt or occult bleed often in small bowel such as Crohn’s, anastomotic, cytomegalovirus (CMV)/viral, or other infections like vascular (angiodysplasia, Dieulafoy’s lesion, varices, or lymphangioma) or tumors (polyps, carcinoid, or lymphoma) and others like Meckel’s diverticulum, diverticulosis, etc. Various diagnostic modalities include small bowel follow through, enteroclysis, push enteroscopy and capsule endoscopy. Rarely, surgery with intraoperative endoscopy may be required (Flow chart 9.6.1).

Indications for Surgery Failure of medical treatment, hypersplenism, isolated splenic vein thrombosis, patients from remote and underprivileged places and of rare blood group. Emergency devascularization and esophageal transection (modified Tanner’s) or elective Warren distal splenorenal shunt or rarely Rex shunt (superior mesenteric vein to left portal vein branch with venous graft) or liver transplantation are useful.

Identifying the Other Sources of GI Hemorrhage The other sources can be identified by esophago-gastroduodenoscopy, colonoscopy, Meckel’s scans (99mTcpertechatate scan), bleeding isotope scan, angiography, capsule endoscopy, double balloon enteroscopy, imaging, upper GI radiography and barium enema and preoperative endoscopy. ­

ndoscopy: Esophago-gastro-duodenoscopy or colono scopy is very useful to detect esophagitis, Mallory-Weiss E

•

I



Treatment depends on the cause of the bleeding. Various treatment modalities are H2 receptor antagonists/PPIs, vasoactive agents, endotherapy including electrothermal agents such as endoclips and argon plasma coagulation.

O

V

Control of Non-Variceal Bleeding

I

S

Transjugular intrahepatic portosystemic shunt (TIPS) is ideal for patients whose bleeding is not controlled by endoscopy. It is effective only in portal hypertension of hepatic origin and contraindicated in portal vein thrombosis, biliary block, septicemia and severe hepatic encephalopathy. Complications include thrombosis of stent and encephalopathy.

L

Transjugular Intrahepatic Portosystemic Shunt

M

Beta-blockers (propranolol) in a dose of 1–2 mg/kg lowers venous flow and the portal pressure by decreasing the heart rate by 25% and the blood pressure by 15 mm Hg and causing splanchnic vasoconstriction and decreasing portal venous flow.

A

For Secondary Prophylaxis

tears, varices, gastritis, ulcer, vascular malformations, etc. Bleeding from an ulcer is controlled using injection with adrenaline and recently hemoclips are also available for clipping at the site of vessel bleed at the base of the ulcer. Similarly, colonoscopy helps diagnosis and therapy of common causes like polyps and the less common vascular malformations and diagnosis of colitis, IBD, solitary rectal ulcer, vascular malformations, etc. • ngiography: It is indicated in active GI bleeding and the rate of bleeding must be at least 0.5 ml/minute. The diagnostic yield of emergency arteriography is low. • UG radiology: Radiographic studies are particularly useful in the diagnosis of esophageal strictures, malrotation of the bowel, or deep ulcerations. • Barium enema: Barium enema is useful in neonates and infants presenting with malrotation with secondary intestinal volvulus, intussusception which can be diagnosed and in many cases treated by barium enema. The barium enema is also effective in identifying the presence of polyps.

E

ndoscopic sclerotherapy: Injection into (intravariceal) or around (perivariceal) of sclerosant. Various sclerosants used in esophageal varices are polydocanol, sodium tetradecylsulfate, absolute alcohol, sodium morrhuate and in bleeding gastric varices, injection of glue (N-butyl2-cyanoacrylate) is done. • Complications of endotherapy: Fever, chest pain, dysphagia, superficial mucosal ulcerations (6–70%), esophageal perforation, pulmonary complications and esophageal stricture.

S

b

d

c

ext ook of Pe iatri s T

•

538

vip.persianss.ir

I

L

S

I

 

ndications for urgery in ower G Bleeding

Diseases of Gastrointestinal ystem an

L

Intraoperative enteroscopy using pediatric colonoscpe or enteroscope is very specific for small bowel imaging. • Surgery of Meckel’s diverticulum, duplication of small bowel, Hirschsprung disease (HD). • Portosystemic shunt, esophageal transection, TIPS. • iver transplantation may be necessary in the upper GI hemorrhage refractory to medical, endoscopic and radiologic interventions. • In lower GI hemorrhage, hemicolectomy or subtotal colectomy is occasionally required. • Complications due to endotherapy such as perforation.



L



iver



L

L

d

L





L

S

L

1. D’Amico G, Pagliaro , Bosch J, et al. Pharmacological treatment of portal hypertension. An evidence based approach. Semin iver Dis. 1999;19:475. 2. Garcia-Tsao G, Sanyal AJ, Grace ND, et al. and the Practice Guidelines Committee of the American Association for the Study of iver Diseases, the Practice Parameters Committee of the American College of Gastroenterology. Prevention and management of gastroesophageal varices and variceal hemorrhage in cirrhosis. AAS D Practice guidelines. Hepatol. 2007;646(3):923-38. 3. Gottumukkala SR, auren G, Das A, et al. American Gastroenterological Association (AGA) Institute Medical Position Statement on Obscure Gastrointestinal Bleeding. Gastroenterol. 2007;133(5):1694-6. 4. Poddar U, Borkar V. Management of extrahepatic portal venous obstruction (EHPO): Current strategies. Trop Gastroenterol. 2011;32(2):94-102.



Bibliography



Flow chart 9.6.1 Management of obscure bleeding

539

vip.persianss.ir

C

C



onstipation in hildren

O

C

efinitions onstipation

E

Constipation is the passage of firm or hard pellet like stools at infrequent (< 3 stools/week) and long intervals with difficulty to expel. Normally the stool frequency reduces progressively in childhood from an average of 4/day in the first month to 1.7/day at 2 years and 1.2/day at 4 years of age.

ncopresis

Encopresis is involuntary passage of stools soiling the underwear in the presence of functional or habitual constipation and the term fecal incontinence replaces the term encopresis.

C

Obstipation refers to the absence of passage of both feces and flatus and denote often an underlying organic obstruction or pseudo-obstruction.

auses



The common causes of constipation are listed in Table 9.7.1. The list is not exhaustive and a condition may manifest in both the age groups. Other rare causes of constipation are neuronal intestinal dysplasia, Chagas disease, small left colon syndrome, neurofibromatosis, and intrinsic hollow visceral myopathy leading on to pseudo-obstruction. Red flags on examination to be noted are failure to thrive, abdominal distension, pilonidal sinus, midline pigmentary abnormalities, patulous/anteriorly placed anus, empty rectum and gush of air and wind from rectum on withdrawal of finger on per rectal examination, absent anal wink or cremastric reflex and lower limb weakness. F

Constipation is a symptom of an underlying disorder commonly presenting with chronic abdominal pain. Approximately 10–25% of all patients attending the pediatric gastroenterology clinics present with various defecation disorders and 1.3–5% of them have encopresis. Almost all of them (Table 9.7.1) pose a real challenge to understand and to treat effectively. Nearly 90% of them are functional and often does not need extensive tests. A good history, physical examination including per rectal and checking for red flags is needed to differentiate functional from an organic cause (5%). Significant number of children has associated urinary bladder voiding dysfunction. Most protocols of treatment are based on experience and recommend prolonged therapy and follow-up over months and sometimes years.

bstipation

unctional onstipation (Habitual onstipation) C

ntroduction

C

I

VR Ravikumar

D

t

t

Tex book of Pedia rics

9.7

It is the most common cause of constipation in children between 4 years and 18 years due to improper toilet training or fissure in anus; mostly voluntary retention of stools avoiding normal calls of nature. However, it can manifest earlier during weaning, during toilet training or at the time of joining a school. The causes include a combination of poor diet including formula weaning, improper bowel training/habituation to move bowels and impact of varying other stressful childhood, e.g. joining the school.

 

Table 9.7.1 Common causes of constipation

540

Newborn

Infants and children

Prematurity Underfeeding, formula feeds Hypothyroidism Hirschsprung disease Low anorectal anomalies, e.g. anterior perineal/ectopic anus, anal stenosis Spinal abnormalities Meconium plug syndrome Pre-sacral/pelvic masses

Poor dietary habits, lack of fiber Weaning formulas Hypothyroidism Hirschsprung disease Functional or psychogenic Irritable Bowel syndrome—constipating type Hypoxic ischemic encephalopathy Cerebral palsy Mental retardation Drugs: Antispasmodics, antimotility drugs, phenothiazines, codeine, containing cough mixtures, vincristine and vinblastine

vip.persianss.ir

Treatment

isimpaction

Oral

Diet Diet in constipation includes fiber containing items like sprouted whole grains, pulses, beans, sorbitol rich fruits (apple with peel, guava, pomegranate, pear, and prune juice), green leafy vegetables and water. Fiber intake is recommended over 2 years of age and the dose is calculated as age in years plus 5 g/day. Involvement of dietician is beneficial.

D t S

t

t

Maintenance therapy is aimed to avoid reimpaction and to ensure regular passage of stools by diet, laxatives and behavioral therapy. We train the caregivers a simplified use of stool diary and recording of daily stool and defecation details.

t

aintenance

Behavioral Therapy Behavioral therapy includes proper toilet training, after feeds, three times daily for 5–10 minutes. One word, one person, one year, one stool/day, one sitting posture policy is ideal. School teachers are informed about the child’s problem.

Laxatives axatives such as lubricants, osmotic and stimulants for maintenance are required for a longer period even over years to regulate the bowel habits and hence the need for explaining to this parents at the onset. Guidelines for maintenance dose of commonly used drugs are given in Table 9.7.3.

Hirschsprung disease

More common

Less common

Meconium history—normal

Delayed passage

Onset beyond infancy

From birth

Fecal soiling

Spurious diarrhea

Abdominal distension—rare

Common

Loaded rectum

Empty rectum Grips finger Gush of air and fluid on release

FTT/enterocolitis—not common

FTT and enterocolitis—common

•

ollow-up chedule S

Functional constipation

onthly follow up till regular bowel movement is achieved: Check diary, physical and rectal examination. axative dose is to be adjusted. • ollow-up of 3 months for next 2 years: Continue same dose of laxative for at least 3 months (distended bowel to regain its function) and then slow tapering (early withdrawal of laxative is the most common cause 541 of recurrence). M

 

Table 9.7.2 Differences between Hirschsprung disease and functional constipation

F

L

Polyethylene glycol (PEG) orally or through Ryle’s tube in the hospital in a dose of 25 ml/kg/hour till the fluid per rectum is watery with no fecal matter or 1 to 1.5 g/kg/day

Twice daily anal applications of petroleum jelly or zinc oxide cream or Diltiazem or nitroglycerine or with 2% xylocaine jelly using ear bud are effective in anal cracks.

L

D

The main steps in the management of constipation are: • Disimpaction • Maintenance therapy with diet, laxative and behavioral training • Regular follow-up • Evaluation of refractory constipation.

Topical

L





In addition to constipation, many present with chronic recurrent abdominal pain and occasionally poor feeding. Enuresis and other voiding disturbances, some culminating in urinary infections, may be the presenting feature. Unlike HD, the abdomen is only mildly distended with hard palpable fecalomas in the left lower quadrant of the abdomen. Rectal examination reveals a dilated rectum filled with hard fecal masses. Children adopt peculiar postures during defecation with many crossing their legs (Vincent’s curtsy) or attempting to defecate in the standing position. With a vicious cycle leading to fecal retention and impaction, there is soiling of the undergarments (Table 9.7.2). Presence of a firm fecal mass in the lower abdomen, full of hard and dry stools on per rectal (PR) examination and fecal soiling suggest fecal impaction. Diagnosis is invariably clinical.

This route by digital is quick, invasive and but may increase the fear of defecation though effective. Rectal suppositories are useful in infants and include glycerine suppository 1 g for pediatric size and 2–3 g for adolescents. Rectal enema includes glycerine, sodium biphosphate, saline or dioctyl sodium sulphosuccinate enema.

M

linical Presentation

Rectal

F

C

The vicious cycle of events in the genesis of chronic functional constipation is as follows: fecal retention → rectal distension → decreased sensory perception → hard stool → fissure in ano → pain/bleeding during defecation, partial evacuation → impaction → fecaloma formation.

over 4 hours at home for 3 days or magracol can be used. Mineral oil 15–30 ml/year of age up to 240 ml OD or BD doses for 2–3 days may be tried in older children but not recommended in infants.

iseases of Gas roin es inal ys em and iver

Pathophysiology

vip.persianss.ir

Tex book of Pedia rics

Dose

Side effects

Lubricant: Mineral oil

1–3 mL/kg/day once daily or in divided doses for a short period only

Do not give to infants. Anal leakage, lipoid pneumonia

Osmotic: Lactulose Or Lactitol Or Sorbitol

1–3 mL/kg/day in two doses

Bloating, cramps and diarrhea

Polyethylene glycol

5–10 mL/kg/day

Nausea, vomiting, cramps, diarrhea

Magnesium hydroxide

1–3 mL/kg/day in two doses

Hypermagnesemia, hypophosphatemia, hypocalcemia

Stimulant: Bisacodyl

>2 years: 5–10 mg daily oral 5 mg per rectally

Cramps, diarrhea, anal irritation

Sodium pecosulphate

5–10 mg daily

Cramps, diarrhea

Senna

>6 years 5–15 ml/day (8.8 mg/5 ml) 2–6 years 2.5–7.5 mL/day

Melanosis coli, hepatitis

K

Y

early follow-up: Points to be remembered while treating infants with constipation are to exclude organic causes such as HD, cystic fibrosis, cretinism, etc. to avoid mineral oil, stimulant laxatives and glycerine enemas for fecal impaction. Stool softeners like sorbitol containing juices, lactulose or lactitol and polyethylene glycol (PEG) and magracol are recommended in infants.

essages



1. Clinical Practice Guidelines: Evaluation and Treatment of Constipation in Children. Summary of updated recommendations of North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. J Pediatr Gastroenterol Nutr. 2006;43:405-7. 2. Srivatsava A. Management of functional constipation. Indian J Pediatr. 2008;75Supp: 519-25. 3. Thapa BR. Constipation in children. IAP Specialty series on Pediatric Gastroenterology. 2008;10:125-44.

B





ibliography



efractory onstipation

Children with refractory constipation with recurrent impaction, not responding to routine use of laxatives, diet and behavioral therapy needs evaluation of organic diseases and timely referral for specific investigations such as rectal biopsy or to rule out anatomic defects by referral to pediatric surgeons or pediatric gastroenterologists for anorectal manometry, metabolic screen (hypothyroidism, cystic fibrosis, hypercalcemia, celiac disease, lead poisoning, mental retardation, etc.), colonic transit study, colonic manometry or ileo-colonoscopy, planning work for spinal dysraphism by MRI of lumbosacral spine and brain. These patients, especially school-going children, may need to be diagnosed whether they are functional or organic, or irritable bowel syndrome (IBS)-constipating type or if functional whether they are of slow transit or normal transit type or pelvic floor dysfunction (rectoanal dyskinesia) which needs biofeedback training.

ey

• Constipation is a common problem in children and often presents as chronic periumbilical pain with difficulty and delay in passing dry stools. • Nearly 95% is of functional and often does not need any investigation; diagnosis is invariably clinical including per rectal examination and long-term follow up is essential. • Management includes drugs, diet modification, toilet training and regular follow up and behavioral therapy. • Refractory cases need referral to the pediatric gastro­ enterologist to further workup to know the cause and for management guidance.

C

R

•

M

Laxative

t

t

Table 9.7.3 Laxatives: dose and side effects

542

vip.persianss.ir

Hirschsprung Disease





Diseases of Gastrointestinal ystem an

9.8

Clinical eatures F

ntroduction

S d L

iver

The ganglion cells originate in the neural crest and migrate aborally along the bowel till the proximal anal canal; further they migrate centripetally into the myenteric plexus and then the submucous plexus. These parasympathetic ganglion cells of the intrinsic enteric nervous system employ nitric oxide as the neurotransmitter and modulate smooth muscle excitatory/inhibitory interactions in the bowel to effect a relaxation during rest and a coordinated antegrade peristalsis with a luminal bolus. HD results from a failure of such migration or destruction of the ganglion cells in a hostile local environment. Additionally there is a deficiency of pacemaker cells of intestine—interstitial cells of Cajal (ICC). The resultant loss of receptive relaxation of the distal aganglionic bowel in response to the antegrade propulsive peristalsis of the proximal ganglionic bowel leads to a functional bowel obstruction. The extrinsic nerves supplying the bowel respond by proliferation and elaboration of both cholinergic (excitatory) and adrenergic (inhibitory, excitatory) neurotransmitters; the excitatory effects of both predominate to render the aganglionic colon and internal anal sphincter spastic.



E

E

mbryology, tiopathogenesis and Pathophysiology

A clinical presentation of large bowel obstruction presenting at any time from the postnatal period to adulthood but dating back to infancy/early childhood is characteristic. Currently, nearly 90% is diagnosed in the neonatal period or early infancy. Failure to pass meconium within 48 hours of birth is a cardinal clinical feature seen in 80–90% infants with HD but also with 30–40 % children with non-HD and 30–35% healthy preemies. Symptoms of abdominal distension, poor feeding, non-bilious vomiting and progressive constipation