The Hospital for Sick Children Handbook of Pediatrics [12 ed.] 9780323713405

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12.1 EDITION

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The Hospital for

DIGITAL VERSION Included

Sick Children HandbookofPediatrics

Stajf Editors

Dr. Deborah Schonfeld Dr. Shawna Silver Fellow Editors

ELSEVIER

Dr. Catherine Diskin Dr. Siobh å n Neville

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12 th EDITION

The Hospital for

Sick Children Handbook of Pediatrics

12 th EDITION

The Hospital for

Sick Children Handbook of Pediatrics EDITORS Deborah Schonfeld, MDCM, FRCPC Sta Physician, Division of Emerency Medicine Department of Pediatrics, e Hospital for Sick Children ssistant Professor, Faclty of Medicine, niversity of oronto

Shawna Silver, MD, FRCPC, PEng Sta Physician, Division of Pediatric Medicine Department of Pediatrics, e Hospital for Sick Children ectrer, Faclty of Medicine, niversity of oronto

Catherine Diskin, MB BCh BAO, MSc, MRCPI Pediatric Medicine Fellow Department of Pediatrics, e Hospital for Sick Children

Siobhán Neville, MB BCh BAO, MSc, MRCPI, MRCPCH Pediatric Medicine Fellow Department of Pediatrics, e Hospital for Sick Children

Elsevier 1600 John F. Kennedy Blvd. Ste 1800 Philadelphia, PA 19103899 E SPA F SK E ABK F PEAS, EF E Copyright © 2022 by Elsevier, Inc. All rights reserved.

SB 98033130

o part o this pliation ay e reproded or transitted in any or or y any eans, eletroni or ehanial, inldin photoopyin, reordin, or any inor ation storae and retrieval syste, ithot perission in ritin ro the p lisher. etails on ho to see perission, rther inoration aot the Plisher’s perissions poliies and or arraneents ith oraniations sh as the opy riht learane enter and the opyriht iensin Aeny, an e ond at or esite .elsevier.operissions. his oo and the individal ontritions ontained in it are proteted nder opyriht y the Plisher other than as ay e noted herein.

Notice Pratitioners and researhers st alays rely on their on eperiene and nolede in evalatin and sin any inoration, ethods, oponds or eperients desried herein. Bease o rapid advanes in the edial si enes, in partilar, independent veriation o dianoses and dr dosaes shold e ade. o the llest etent o the la, no responsiility is assed y Elsevier, athors, editors or ontritors or any inry andor daae to persons or property as a atter o prodts liaility, neliene or otherise, or ro any se or operation o any ethods, prodts, instrtions, or ideas ontained in the aterial herein.

Previos edition plished nder title he S andoo o Pediatris Library of Congress Control Nuber

ontent Strateist aryeth hiel ontent evelopent Speialist eredith adeira Plishin Servies anaer eepthi nni Proet anaer adan orde Selvanadin esin iretion Patri . Ferson Printed in the nited States o Aeria ast diit is the print ner

9 8

 6



 3

 1

NOTICE

is book is a general guide only and should never be a substitute for the skill, knowledge, and experience of a qualied medical professional dealing with the facts, circumstances, and symptoms of a particular case. Neither the ublisher nor the uthors assume any responsibility for any loss or inury andor damage to persons or property arising out of or related to any use of the material contained in this book. t is the responsibility of the treating practitioner, relying on independent expertise and knowledge of the patient, to determine the best treatment and method of application for the patient. – e ospital for ick hildren

v

DEDICATION

To… All the students, residents and fellows whom I have taught and have taught me, and to mom and dad—the most inspiring pediatricians I know. DS To… All the children, families and colleagues from whom I have learned so much, and my family—you are truly gems. SS To… ose that support me, family and friends.

CD

To… My closest champions and condantes, whose patience for me talking about this proect knew no bounds. SN

vi

CONTENTS

Foreword Preface Acknowledgments Chapter Authors Common Abbreviations

ix xi xii xiii xviii

Section I: Acute Care Pediatrics

1

Chapter 1: Resuscitation

2

Chapter 2: Emergency Medicine

30

Chapter 3: Poisonings and Toicoogy

1

Chapter : Pain and Sedation

3

Chapter : Procedures

Section II: Suspeciaty Pediatrics

10

13

Chapter : Adoescent Medicine 10 Chapter : Aergy

13

Chapter : Cardioogy

1

Chapter : Chid Matreatment

21

Chapter 10: entistry

22

Chapter 11: ermatoogy

23

Chapter 12: eeopment

2

Chapter 13: iagnostic Imaging

22

Chapter 1: Endocrinoogy

2

Chapter 1: uids Eectroytes and Acid–ase

32

Chapter 1: astroenteroogy and epatoogy

31

Chapter 1: enera Surgery

0

Chapter 1: enetics and Teratoogy

2

Chapter 1: roth and utrition



Chapter 20: ynecoogy



Chapter 21: ematoogy

1

Chapter 22: Immunoogy

0

Chapter 23: Immunoprophyais  Chapter 2: Inectious iseases

00

Chapter 2: Menta eath

2

Chapter 2: Metaoic isease



Chapter 2: eonatoogy

1

Chapter 2: ephroogy and roogy

0

Chapter 2: euroogy and eurosurgery

02

Chapter 30: ncoogy

30

Chapter 31: phthamoogy



Chapter 32: rthopedics



Chapter 33: toaryngoogy

02

Chapter 3: Pastic Surgery

2

Chapter 3: Respiroogy

0

Chapter 3: Rheumatoogy

2

vii

Chapter 3: Technoogy and Medica Compeity

1

Chapter 3: Transpantation

1001

Section III: aoratory Reerence aues and Transusion Medicine 1013

Contents viii

Chapter 3: aoratory Reerence aues and Transusion Medicine

101

Section I: rug osing uideines

1111

Chapter 0: rug osing uideines

1112

Inde

12

FOREWORD TO THE 12TH EDITION

It is a privilege and a pleasure to write the foreword to the 12th edition of e Hospital for Sick Children Handbook of Pediatrics. Wh do I regard this as a privilege and a pleasure It is a privilege to be associated with an iconic pediatric “bible” that has provided such value to so an generations of child health students and clinicians. e consistent ecellence in presenting coprehensive state of the art evidencebased practical guid ance has ensured that this handbook has not onl endured into its 12th edition over ore than  ears since the 1st edition but iproved and reinvented itself with ever new version. e Hospital for Sick Children or Sickids as it is aectionatel known has been a world leader in the provision of clinical care and research in child health for over 1 ears. enerations of learners have travelled fro across the world to train here and Sickids is faous for its leadership in teaching. ere are currentl 2 inpatient beds  visits to our ergenc epartent and 2 visits to our abulator clinics annuall. cross the hospital we have close to 1 clinical and research trainees. Sickids is the ost researchintensive hospital in Canada and our esearch Institute is a world leader in discover and cuttingedge science. f note is that this handbook has essentiall been written b our residents and fellows who are oen the unsung heroes of the teaching hospital long hours hard work and together with the nursing and support sta provide the energ that keeps the hospital ticking and the patients cared for 2. anks also to the facult chapter authors and espe ciall to the ditors rs. Shawna Silver and ebbie Schonfeld who have done an out standing ob supervising the trainees and diligentl driving this process fro concept to nish with great deterination talent and skill. Soe of the brand new features include helpful practical chapters on ental Health as well as echnolog and edical Copleit. It is a pleasure to write this foreword because it is so uch ore fun than  relation ship with the previous three editions. When I was a oung innocent rst ear resident in 1 I carried the th edition of this handbook around in the pocket of  neatl starched white coat. It was thick heav and had a serious red cover. I think I kept it under  pillow when I rarel had the opportunit to reach  oncall roo for a brief nap. oward the end of  residenc it was replaced b the 1th edition which was thinner had a ore soothing blue cover and was still seldo ore than an ar’s length awa fro e as I navigated the transition to  new role as unior facult. e 11th edition which was published in 2 was coedited b self and r. nne ipchand. It has a ellow cover with a bright picture of our hospital atriu and was the rst ix

edition that had an electronic version which was ust becoing popular at the tie. I reeber ver clearl ust how uch tie and eort it took over the course of two ears to coordinate and edit the ecellent contributions of the one hundred trainee and facult chapter authors. So honestl being invited to write this one page foreword and being able to pass the baton to rs. Silver and Schonfeld and their tea is trul a pleasure

Foreword to e 12th Edition x

I invite ou to eno and benet fro the cobined eperience and wisdo that coes fro 1 ears in the child health business. I believe that regardless of our level of epertise and contet ou will nd advice and practical approaches that will be infor ative and useful. I salute not onl all those who have contributed to this ecellent 12th edition but also all of ou readers who have dedicated our service to iproving the health of children all over the world. Jeremy Friedman, MBChB, FRCP, FAAP Interi PediatricianinChief Hospital for Sick Children Professor and Interi Chair epartent of Pediatrics niversit of oronto

PREFACE

ank you for choosing this 12th edition of e Hospital for Sick Children Handbook of Pediatrics. Much like the pediatric healthcare practiced at e Hospital for Sick Children, this book is up-to-date, evidence-based, interdisciplinary and patientfocused in its approach. ur intention as e undertook the unieldy task of updating this handbook as to create a practical and applied resource for bedside teaching, study and practice. e hope this tet ill act as a valuable reference for edical students, residents, pediatricians, faily doctors, eergency physicians, nurses and other practitioners ho provide care to children—at e Hospital for Sick Children, throughout Canada and orldide. Content has been etensively revised throughout the handbook, reecting current best practice. is edition has been reforatted, aking it easier to read and navigate. here possible, e have provided algorithic approaches to clinical probles, ensuring the content is accessible and easy-to-use. e have added a ne feature of “earls” and “itfalls” to each chapter, to highlight iportant clinical points and coon istakes or isconceptions. e chapters cover Mental Health, and echnology and Medical Copleity, reecting eerging areas of iportance in clinical practice. e are etreely grateful to the chapter authors ho have shared their epertise and isdo. e hope that the 12th edition of e Hospital for Sick Children Handbook of Pediatrics ill prove itself a orthy guide and copanion to your study or practice of the care of infants, children and adolescents. Deborah Schonfeld Shawna Silver Catherine Diskin Siobhán Neville

xi

ACKNOWLEDGMENTS

So many people have helped make the 12th edition of e Hospital for Sick Children Handbook of Pediatrics a reality. In particular, we would like to acknowledge Dr. Adelle Atkinson, the Director of Postgraduate edical ducation at e ospital for Sick hildren and Dr. eremy riedman and Dr. Anne Dipchand, editors of the 11th edition of e Hospital for Sick Children Handbook of Pediatrics for their guid ance, wise insight, and support. e are grateful to all of the authors and editors of the previous editions of e Hospital for Sick Children Handbook of Pediatrics for the opportunity to uild on all of their outstanding work. ank you to rika Schippel, e ospital for Sick hildren Pulishing oordinator, for her ongoing help with the contracts and ensuring smooth communication with the pulishers. ank you to Dr. rie ama for her help in the early stages of content organiation and development. any thanks go to aryeth iel, lsevier, for facilitating the process of a new edi tion. Special thanks go to our content development specialists and proect managers at dierent stages, eredith adeira and adan Selvanadin, for eing a tremendous source of advice and support throughout the production process. inally, we would like to thank all of the chapter authors—oth learners and sta—for their dedication and perseverance culminating in the 12th edition of e Hospital for Sick Children Handbook of Pediatrics. Deborah Schonfeld Shawna Silver Catherine Diskin Siobhán Neville

xii

CHAPTER AUTHORS Section I: ACUTE CARE PEDIATRICS

: Procedures achar Pancer, BPH, MBBS Fellow, Emergency Medicine

1: Resuscitation Melanie Bechard, BSc, MD

Mehan ille, MD, FRCPC

Fellow, Emergency Medicine (CHEO)

Jnahan Pirie, MD, Md, FRCPC

Andrew Helmers, MDCM, MHSc(c), MSc, FRCPC

Staff Physician, Emergency Medicine

Fellow, Emergency Medicine

Fellow, Critical Care Medicine

Lianne J. McLean, MB, BCh, BAO, MHI, FRCPC

Section II: SUBSPECIALTY PEDIATRICS

Staff Physician, Emergency Medicine

2: Emergency Medicine MariePier Liree, MBChB

: Adoescent Medicine alene Sinh, MD Resident, Pediatrics

Resident, Pediatrics

Samanha Marin, MD, FRCPC

Maa HarelSerlin, MD

Fellow, dolescent Medicine

Fellow, Emergency Medicine

Alene lan, MD, MSc, FRCPC

Iwna Baran, MD, FRCPC

Staff Physician, dolescent Medicine

Staff Physician, Emergency Medicine

Sanne Ben, MD, FRCPC, D(ABP) Staff Physician, Emergency Medicine

: Aergy Sehanie rdle, MD Fellow, mmnology and llergy (C Children’s

3: Poisonings and Toxicoogy amin Ladha, MD, MSc

Melanie Cnwa, MD, FRCPC

Resident, Pediatrics

Fellow, mmnology and llergy

lana a, MD

Adelle R. Ainsn, MD, FRCPC

Fellow, Emergency Medicine

Staff Physician, mmnology and llergy

Saihiri Ranaalan, MBBS, PhD, FRCP(), FRCP(C), FAAP Staff Physician, Emergency Medicine and Clinical Pharmacology & oicology

: Pain and Sedation ahira Daa, MD Fellow, Emergency Medicine (niersity of lerta)

Lisa Isaac, MD, FRCPC

Hosital)

: Cardioogy Michael D. Fridman, MD, FRCPC Fellow, Cardiology

Jnahan n, BMBS, FRCPC, FAAP Fellow, Cardiology

elle Panea, MD, FRCPC, FAAP Fellow, Cardiology

Jennier L. Rssell, MD, FRCPC Staff Physician, Cardiology

Staff nesthesiologist, nesthesia and Pain Medicine

xiii

: Cid Matreatment ani Aarwal, MD

1: Endocrinoogy J Swemim, BSc, MD

Resident, Pediatrics

Resident, Pediatrics

Reecca an, MD

Jlia Srara, MD, FRCPC

Resident, Pediatrics

Fellow, Endocrinology

ldie Aril, MD

Jnahan D. asserman, MD, PhD

Fellow, Pediatric Medicine

Staff Physician, Endocrinology

Rm Ch, MD, FRCPC Jennier Smih, BMBS, MSc, FRCPC

1: uids Eectroytes and Acid–ase Lara Becherman, MD

Staff Physician, Pediatric Medicine

Resident, Pediatrics

Staff Physician, Pediatric Medicine

Chapter Authors

1: entistry Rdd Mran, BDSc, DCD, FRACDS

mma lrich, MD Fellow, ehrology

aie Sllian, MBChB

Fellow, entistry

Fellow, ehrology

Jane H, DCD

Damien ne, MB BCh BAO, MSc

Fellow, entistry

Staff Physician, ehrology

Shnna Masse, HBSc, DDS, FRCDC Staff entist, entistry

11: ermatoogy Lara Mrrisse, MD Resident, Pediatrics

imerl anc, MD Fellow, ermatology

Reecca Le, MD, FRCPC Staff Physician, ermatology

12: eeoment Adre illran, MD Resident, Pediatrics

Claire . en, BSc, MSc, LLB, MPH, MD Fellow, eelomental Pediatrics

Jenna Di, MD, FRCPC

1: astroenteroogy and Heatoogy Ameilia ellar, MD, MSc Resident, Pediatrics

ileen Crwle, MB BCh BAO, MRCPI, MSc Fellow, astroenterology, Heatology and trition

mas alers, MBBS, MSc, FRACP Staff Physician, astroenterology, Heatology and trition

1: enera Surgery Jnahn Hael, MD Resident, Pediatrics

Jsna M. linsa, MD, FRCSC, MPH(c) Staff Srgeon, eneral and horacic Srgery

eres Aie, MD, FRCSC Staff Srgeon, eneral and horacic Srgery

Staff Physician, eelomental Pediatrics

Amer Main, MD, BHSc, FRCPC Staff Physician, eelomental Pediatrics

13: iagnostic maging Alisha Jamal, MD, MSc, FRCPC Fellow, Emergency Medicine

Jere raici, MD xiv

Staff Radiologist, iagnostic maging

1: enetics and Teratoogy Aree Mah, MD Resident, erology

rer Csain, MD, PhD Resident, Medical enetics and enomics

Rer MendaLndn, MD, MS, FRCPC, FCCM iision Head, Clinical and Metaolic enetics

1: rot and utrition Jsin Lam, MD

Heaher Millar, MIPH, MD, FRCSC

Resident, Pediatrics

Anali Aarwal, MD, MHSc, FRCSC

Lara inlin, MD, MPH, FRCPC

Staff Physician, ynecology

Staff Physician, ynecology

Fellow, Pediatric Medicine Staff Physician, Pediatric Medicine

21: Hematoogy Adam an, MD

Mara AleanianFarr, MSc, RD

Resident, Pediatrics

Clinical ietician, nfant and oddler rowth

ana Caric, MD

and Feeding

Resident, Pediatrics

Alisa BarDaan, RD

Smira le, MD, MSc, FRCPC

Clinical ietitian, Endocrinology

Fellow, HematologyOncology

Jrdan Bealie, RD

Michaela Cada, MD, FRCPC, FAAP, MPH

Clinical ietitian, Pediatric Medicine and

Staff Physician, HematologyOncology

Comle Care

else allaher, RD Clinical ietitian, Endocrinology and Rhematology

22: mmunoogy Ori Sc, MD, FRCPC Fellow, mmnology and llergy

Daina alnins, MSc, RD

Amiirah Anarain, MSc, MD, FRCPC

irector, Clinical ietetics

Fellow, mmnology and llergy

Alissa Seiner, RD

 im, MD, MScCH, FRCPC

Clinical ietitian, Endocrinology

Staff Physician, mmnology and llergy

Lri ira, RD Lara res, MSc, RD

23: mmunoroyaxis Ana C. Blanchard, MDCM, MSc, FRCPC

Clinical ietitian, Pediatric Medicine and

Fellow, nfectios iseases

Clinical ietician, Critical Care

Comle Care

Shama Sd, MD

ellie elch, BASc, RD

Fellow, mmnology and llergy

Clinical ietitian, Pediatric Medicine and

Shan . Mrris, MD, MPH, FRCPC, FAAP, DMH

Resiratory Medicine

Carline Crrie, R, IBCLC

Chapter Authors

Mea an den Heel, MD, PhD

Staff Physician, nfectios iseases

actation Secialist, reastfeeding Program Registered rse and actation Consltant

2: nectious iseases Ran ir, MD

Samanha Sllian, R, IBCLC

Resident, Pediatrics

Registered rse and actation Consltant

Jennier am, MD, MHP, FRCPC

Ashle raham, MScO, MHM

Fellow, nfectios iseases

Occational heraist, Rehailitation

Ari Binn, MD, MSc, FRCPC

Lara Mclean, BSc, IBCLC

Serices

Staff Physician, nfectios iseases

2: ynecoogy Laren Friedman, BHSc, MD

2: Menta Heat arielle Salmers, MBBS

Resident, Pediatrics

Fellow, Commnity Pediatrics

xv

Dahne J. rca, MD, MSc, FRCPC (eds), FRCPC (sch)

Ahaa larni, MD, PhD, FRCSC

Staff Physician, Child and dolescent Psychiatry

Lia Plcine, MD, MSc, FRCPC

Staff Srgeon, erosrgery Staff Physician, erology

2: Metaoic isease Carsen reer, MD

Chapter Authors

Resident, Pediatrics

3: Oncoogy Am L, BHSc, MD

Resham a, MD

Resident, Pediatrics

Resident, Clinical and Metaolic enetics

eal Sndheimer, MD, PhD

Mhammed Al aimi, BSc, MD, FRCPC

Staff Physician, Clinical and Metaolic enetics

Fellow, HematologyOncology

Reena Paari, MSc, MD, FRCPC 2: eonatoogy Am irs, MD

Fellow, HematologyOncology

Resident, Pediatrics

Staff Physician, HematologyOncology

Smi a, MD, PhD, FRCPC

Jlia DiLai, MD, MSc aas larni

31: Otamoogy Shel msn, BSc, MD

M Ch O, FRCPC

Resident, Pediatrics

Fellow, eonatalPerinatal Medicine

Asim Ali, MD, FRCSC

Aideen Mre, MD, MHSc, FRCPC

OhthalmologistinChief, Ohthalmology and

Fellow, eonatalPerinatal Medicine

Staff Physician, eonatology

ision Sciences

2: eroogy and Uroogy Madalena Riedl, MD, PhD

32: Ortoedics Allsn Shre, MD

Resident, Pediatrics

Resident, Pediatrics

Mallr L. Dwnie, MD, FRCPC Fellow, ehrology

nni araanan, MBBS, MSc, FRCSC

Anne Shie Blais, MD, FRCSC

Staff Srgeon, Orthoedic Srgery

Fellow, rology

Jana Ds Sans, MD, MHSc, FRCPC Staff Medical rologist, rology

Seeha Radharishnan, MDCM, FRCPC, MScCH

Resident, Pediatrics

Staff Physician, ehrology

Staff Srgeon, Otolaryngology

2: euroogy and eurosurgery Drda Drdeic, MD

3: Pastic Surgery Lara aman, MD

Resident, erology

Resident, Pediatrics

rin Char, MD Resident, erology

risen M. Daide, MD, MSc, FRCSC

Crisina . , MD

Staff Srgeon, Plastic Srgery

Staff Physician, erology

xvi

33: Otoaryngoogy alia reensn, MD, HBA Sharn L. Cshin, MD, MSc, FRCSC

3: Resiroogy ler res, MBBS Resident, Pediatrics

allace B. ee, MD, BASc, MHSc, FRCPC

Section III: LABORATORY REFERENCE VALUES AND TRANSFUSION MEDICINE

Fellow, Resiratory Medicine Staff Physician, Resiratory Medicine

3: Reumatoogy Desmnd She, MD Resident, Pediatrics

3: aoratory Reerence aues and Transusion Medicine hsrw Adeli, PhD, FCACB, DABCC, FAACC Head, Clinical iochemistry, Pediatric aoratory Medicine

Dilan Dissanaae, MD, PhD, FRCPC

Ssan Richardsn, MDCM, FRCPC

Fellow, Rhematology

Chief, Pediatric aoratory Medicine

Shirle M.L. se, MD, FRCPC

n Li, BSc

Staff Physician, Rhematology

Resorce echnologist, Hematology, Pediatric aoratory Medicine

3: Tecnoogy and Medica Comexity Sarna Sharma, MD

Chapter Authors

e J. Mraes, MD, PhD, FRCPC

end La, MBBS, FRCPC irector, ransfsion Medicine, Pediatric aoratory Medicine

Resident, Pediatrics

Benamin Jn, PhD, FCACB

Maria Maran, MD, FRCPC, MSc

Clinical iochemist, Pediatric aoratory Medicine

Fellow, Comle Care Medicine

icria Hiins, BSc

Reshma Amin, MD, FRCPC, MSc

Ph Candidate, Pediatric aoratory Medicine

Staff Physician, Resiratory Medicine

Mar ahrn Bhn, BSc

Sana Mahan, MD, FRCPC, MSc

Ph Candidate, Pediatric aoratory Medicine

Staff Physician, Pediatric Medicine

3: Transantation Lc Dan, MD

Section IV: DRUG DOSING GUIDELINES

Fellow, mmnology and llergy

Jessica P. lsn, MD, FRCPC Fellow, astroenterology, Heatology and trition

: rug osing uideines laine La, BScPhm, PharmD, MSc, RPh rg nformation Coordinator, Pharmacy

risa an Resel, M, PPediarics rse Practitioner, ier and owel ranslant

e All chaers were wrien  residens, ellws and sa  e Hsial r Sic

ic , MD, FRCPC

Children. Chaer ahrs are aliaed wih

Staff Physician, astroenterology,

e Hsial r Sic Children nless

Heatology and trition

herwise ned.

Medical irector, ier ranslant Program

xvii

COMMON ABBREVIATIONS h g .  , # AAP ABC ABG AD ALP AL AA AP A A A bCG BD B BP BA B C Ca CBC C Cl cm C CP Cr CP C C CL C C DBP DDx B CG LA   fu

xviii

increased decreased greater than greater than or equal to less than less than or equal to American Academy of Pediatrics airway, breathing, circulation arterial blood gas autosomal dominant alaline hoshatase alanine aminotransferase antinuclear antibody anteroosterior autosomal recessive asartate aminotransferase abdominal xray beta human chorionic gonadotroin twice daily body mass index blood ressure body surface area blood urea nitrogen culture and sensitivity calcium comlete blood count creatine inase chloride centimeter central nervous system Canadian Paediatric ociety creatinine Creactive rotein cerebrosinal uid comuted tomograhy central venous line cardiovascular system chest xray diastolic blood ressure differential diagnosis steinBarr virus electrocardiogram eutectic mixture of local anesthetic erythrocyte sedimentation rate endotracheal tube followu

grou A Streptococcus gammaglutamyltransferase gastrointestinal genitourinary hemoglobin bicarbonate human immunodeciency virus heart rate history incision and drainage inammatory bowel disease intracranial ressure intensive care unit intramuscular international normalied ratio intelligence quotient intravenous ilogram otassium lactate dehydrogenase liver function tests lumbar uncture meter micro male to female ratio maximum microgram milligram magnesium minimum millilitre months magnetic resonance imaging musculoseletal sodium sodium chloride nonaccidental inury neonatal intensive care unit nil er os normal saline nonsteroidal antiinammatory drug oxygen oerating room Pediatric Advanced Life uort osteroanterior eriherally inserted central catheter ediatric intensive care unit eriheral intravenous catheter er os by mouth hoshate aced red blood cells ro re nata as needed

Common Abbreviations

GA GG G G b C   x D BD CP C     g  LD Ls LP m µ  max mcg mg g min mL mos   a aCl A C P  AD   PAL PA PCC PC P P P BCs P rn

xix

Common Abbreviations xx

P P q  c BC  BP C L  tC  D C P     BG BC  yrs

arathyroid hormone artial thrombolastin time every eg, qh—every  hours every night corrected  interval red blood cell resiratory rate systolic blood ressure subcutaneous sublingual sexually transmitted infection total carbon dioxide total uid intae three times daily congenital infections including toxolasmosis, other agents, rubella, cytomegalovirus, heres total arenteral nutrition thyroid stimulating hormone uer resiratory tract infection ultrasound urinary tract infection venous blood gas white blood cell xray years

Section I Acute Care Pediatrics CHAPTER 1 CHAPTER 2 CHAPTER 3 CHAPTER 4 CHAPTER 5

Resuscitation Emergency Medicine Poisonings and Toxicology Pain and Sedation Procedures

1

CHAPTER

1

Resuscitation Melanie Bechard • Andrew Helmers • Lianne Mclean

Common abbreviations Useful calculations Team dynamics Clinical assessment of the ill child Respiratory support during resuscitation Circulatory support during resuscitation Advanced airway management PALS algorithms Post-resuscitation care

2

COMMON ABBREVIATIONS ABC ABG APLS B CPAP CP  i C LA PALS PA PC SC S S

airway, breathing, circulation arterial blood gas advanced pediatric life support bagvalve ask continuous positive airway pressure cardiopulonary resuscitation endotracheal tube fraction of inspired oxygen functional residual capacity laryngeal ask airway Pediatric Advanced Life Support pulseless electrical activity pediatric intensive care unit return of spontaneous circulation rapid seuence intubation systeic vascular resistance

Resuscitation

Also see page xviii for a list of other abbreviations used throughout this book

USEFUL CALCULATIONS  Hypotension (systolic blood pressure) a , onth , g b – onths os , g c – years yrs , 1 3age g d . yrs , g  Estimated weight in kg APLS th ed a – os 3age in os 1  b – yrs 3age in yrs 1  c – yrs 3age in yrs 1   Endotracheal tube (ETT) size a ncued  age in yrs 1  b Cued  age in yrs 1   ETT depth a , yr i ropharyngeal intubation c at lip age in yrs 1  ii asopharyngeal intubation c at nare age in yrs 1  b . yr i ropharyngeal intubation c at lip age in yrs 1   3 diaeter ii asopharyngeal intubation c at lip age in yrs 1 

1

3

TEAM DYNAMICS esuscitation teamwork, communication, and leadership all have a signicant ipact on patient outcoes

Resuscitation 1

 eore a resuscitation  nticipate needs a Contact necessary personnel eg, respiratory therapy if available, consider anesthesiology and consider available euipent b Seek help when needed eg, contact anesthesiology or otolaryngology if a dicult airway is anticipated  ssign roles clearly dene roles and responsibilities of each tea eber  now your resources a ailiarie yourself with the resuscitation euipent of your hospital ward and eergency departent b now your provincial or state resources for coordinating transfer to tertiary care centers, if applicable  uring a resuscitation  A leader should a er constructie eedback “Please perfor copressions faster—we’re aiing for  to  per inute” b ink out loud share thoughts and suggestions to create a shared ental odel “ suspect this child has septic shock” Avoid preature xation on a diagnosis and be ready to shi§ perspective c Perfor periodic reiews suarie and reevaluate the case ¨is helps to ensure adherence to algoriths and oers opportunities for new ideas to reduce bias d Encourage inormation sharing and bidirectional transission of ideas “Are there other thoughts about which edications should be used before intubation” e eain “hands o ” if possible leaders who focus on coordinating the tea rather than participating in the resuscitative eorts perfor better §en this necessitates delegating obs that can detract fro focused leadership ie, assigning a specic person to calculate resuscitation edication doses PEARL

4

Closed-loop communication is important in a resuscitation; e.g., wen ordering epineprine during a cardiac arrest Proider 1 “Te patient’s weigt is 2 g please gie .2 mg o 11, epineprine intraenously, wic is 2 m” Proider 2 “.2 mg o 11, epineprine, wic is 2 m, now ready to gie ” Proider 1 “Tan you, please administer tat dose” Proider 2 “ose administered”

 er a resuscitation  very eort should be ade to conduct a debrie ebrieng involves guided re¯ection of edical issues, eotional ipact on care providers, and provides perforance feedback to the tea  ¨ere are any debrieng forats eg, standardied fors, “hot” debrief within inutes to hours of the resuscitation, “cold” debrief days to weeks a§er resuscitation  nsure your hospital has a culture of safe and nonudgental debriefs, which follow a consistent structure to ensure that provider distress is addressed and that constructive feedback is incorporated into future events f it is clear that tea ebers have experienced distress eg, a resuscitation that ends with patient deise, it is iportant to identify this in the debrief and ensure channels exist to explore and support this further

Resuscitation

 All tea ebers should a se closedloop communication iproves the safety and eciency of care provided by reoving abiguity fro instructions and allowing for correctionsclarifying uestions if needed b Be respectul vidence suggests rudeness negatively ipacts resuscitation teas’ perforance c Support amily presence during resuscitation there is a strong parental preference to be present

1

CLINICAL ASSESSMENT OF THE ILL CHILD  Ealuation o Es See able 



Assessment of ABCEs

Tale 1.1

Resuscitation

Appearance

Examination

Monitor

irway

     

Patient position Respiratory eort ccessory muscle use est expansion rooling olor

    

Stridor oice uality oug uality Retractions Traceal position

 Respiratory rate  xygen saturation Sa2

reating

 Respiratory rate, pattern, and eort  ccessory muscle use  est expansion  olor

    

reat sounds runting, nasal aring Retractions repitus Traceal position

 Respiratory rate  xygen saturation Sa2

irculation

 eel o consciousness  olor pallor, cyanosis  Mottling

   

Pulses central, periperal Sin temperature apillary rell time perusion eart sounds

 eart rate  lood pressure  Rytm E monitor

isaility

eel o consciousness

 lasgow oma Score Tale 1.2  P lert, eral, Painul stimuli, nresponsie  Pupil symmetry responsieness  lood glucose cec

Exposure

Signs o inury or leeding

Temperature

1

eel o consciousness

ECG, Electrocardiogram. © The Hospital for Sick Children, 2019. Adapted from The Hospital for Sick Children ide to aediatric edical Emergencies.

6

Eye opening

eral response

Motor response

Pediatric aso Coma cae

CidAdoescentAdut

Ae ,2 rs

core

Spontaneous

Spontaneous



To oice

To oice

3

To pain

To pain

2

one

one

1

riented

ppropriate words, smiles, interacts



onused

ries, irritale



nappropriate

ries to pain

3

ncompreensile

Moans to pain

2

one

one

1

eys

Spontaneousoeys



ocalies to pain

ocalies to pain



itdraws to pain

itdraws to pain



normal exion decorticate

normal exion

3

normal extension decererate

normal extension

2

one

one

1

Resuscitation

Tale 1.2

odied from Teasdale , ennett . Assessment of coma and impaired consciosness. A practical scale. Lancet. 1921 and Holmes , alchak , acarlane T, ppermann . erformance of the pediatric lasgo coma scale in children ith lnt head trama. Acad Emerg Med. 200121.

1

 ediatric danced ie Support (S) systematic approach algorithm igure  outlines the approach to caring for a critically ill or inured child ote that in the eent o an arrest, the basic approach now recoended by the Aerican eart Association is irculation–irway–reathing () iure 11 Pediatric Adanced Life upport stematic Approac Aoritm

Work of breathing

Appearance

Circulation odied fro Aerican eart Association Pediatric Advanced Life Support Provider Manual allas,  Aerican eart Association copyright 



RESPIRATORY SUPPORT DURING RESUSCITATION ost cardiopulonary arrests in children are caused by respiratory causes ¨is section covers a nuber of considerations in providing respiratory support to the acutely ill child  oals o respiratory support  entilation reoval of C   xygenation adeuate oxygenation of the blood Resuscitation 1

 agalemask () entilation B ventilation is an iportant skill to aster because it provides oxygen and also ventilation support to a patient who is aking inadeuate respira tory eorts  Euipment ¯owin¯ating bag or selfin¯ating bag with oxygen reservoir connected to an oxygen source, and appropriately sied ask  osition a “Sning position” igure  slight neck ¯exion and occipital extension, ie, lower cervical vertebral ¯exion and atlantooccipital extension b Consider a shoulder roll in infants or a roll under the occiput in older children see igure  c Additional repositioning techniues “head tiltchin li§,” “aw thrust” igure   Seal use C placeent of hands to create seal, with the  position on the andible not the neck so§ tissues igure 

iure 12 Aira Positionin

O

A

T P

P

T

O

B

Preferred airway positioning for A infants using shoulder rolls, and B children using occipital rolls ro rden L, Stacy , Lough  Critical Care Nursing: Diagnosis and Management th ed lsevier 

8

A

B

A ead tilt chin li§ aneuver B aw thrust aneuver A, ro Lewis S, eitkeper , irksen S Medical-Surgical Nursing: Assessment and Management of Clinical Problems th ed St Louis osby  B, ro Shiabukuro , Liu L Cardiopulonary resuscitation

Resuscitation

iure 13 Aira Repositionin

n Stoelting , iller , eds Basics of Anesthesia th ed lsevier 

iure 14 EC Camp Tecniue 1

ro ucanto , atioc A oninvasive anageent of the airway n agberg CA, Artie CA, Ai , eds agberg and Benumof ’s Aira Management th ed lsevier 



 Techniues or improing  entilation SPA able  Tale 1.3

Resuscitation 1

Tecniue for mproin PositiePressure entiation  Mas

Correctie teps

Actions

M

Mas readustment

e sure tere is a good seal o te mas on te ace; consider twoand tecniue

R

Reposition airway

Ensure te ead is in “sning” position

S

Suction mout and nose

Suction secretions i present

O

pen mout

entilate wit te patient’s mout sligtly open and lit te aw orward “aw trust”

P

Pressure increase

radually increase te pressure eery ew reats until tere are ilateral reat sounds and isile cest moement wit eac reat

A

irway alternatie

onsider endotraceal intuation or laryngeal mas airway

Adapted from American Academ of ediatrics. Textbook of Neonatal Resuscitation. th ed. 201.

 irway aduncts Aduncts are useful for aintaining a patent airway in cases of obstruction igure   ropharyngeal airway a Sits fro outh to above the vallecula to prevent tongue fro touching posterior pharynx b Caution risk of so§ palate inury on insertion, gag re¯ex liits tolerance in awake patients  asopharyngeal airway a Sits fro nare to hypopharynx b Caution patients with bleeding tendencies, basilar skull fracture, craniofacial trauaanoalies iure 15 roparnea A and asoparnea B Airas

ro ¨opson A, Salonia  Airway anageent n uhran BP, ieran , eds

10

Pediatric Critical Care th ed lsevier 

 ygen deliery systems see Table 1.4

xen eier stems

Mode of 2 eier

o Rate Lmin

i2 eiered

lowy oxygen

ariale

ariale

sed or well patients tat reuire small amounts o supplemental oxygen

asal cannula

,

23– 21 1 ow in min33

i2 depends on sie o cild, respiratory rate, tidal olume. it small cildren, ow is a greater portion o tidal olume, so more i2 is receied.

Comments

Simple mas

–1

3–

pen oles allow ariale amounts o room air

enturi mas

–1

3–

ontrols i2 y allowing precise amounts o oxygen and room air

Partial rereater mas

1–1

p to 

Two open exalation ports and aleless oxygen reseroir ag. ttaced reseroir ag proides iger i2. eeds 1–1 min to preent ag collapse.

onrereater mas

1–1

p to 

Two oneway ale ports 1 etween oxygen reseroir ag and mas, ten 1 etween mas and exalation port. May use wit oxygen lender to adust i2 etween  and .

Resuscitation

Tale 1.

1

FiO2, raction of inspired ogen.

E Heated humidity high ow therapy eated huidity high ¯ow therapy can be delivered through face ask or nasal cannula Gas is heated and huidied to optiie secretion clearance and tolerance  Heated high ow ace mask provides oxygenation without the addi tion of positive pressure ventilation Adust i  as needed  Heated high ow nasal cannula a low  to  Lin axiu depends on achine and ode typically started at  Lkgin, increase to eect as per anufacturer guidelines 11

b Provides potential, variable aounts of positive endexpiratory pres sure higher PP achieved with saller children, reduces breathing eort assists peak inspiratory ¯ow and optiies intended oxygen delivery liits entrainent of roo air PTALL  selinlating ag witout a positiepressure respiratory support ale only deliers oxygen wen te ag is sueeed to proide a reat; i.e., it cannot e used to proide continuous positie airway pressure and may not delier oxygen wen te ag is not eing depressed.

Resuscitation 1

 ositiepressure respiratory support Positive pressure helps with both ventilation and oxygenation aerates alveoli, prevents alveolar collapse, increases functional residual capacity B with a ¯owin¯ating bag is an iportant way to achieve this in eergency situa tions Consider insertion of a nasogastricorogastric tube to iniie gastric distension which can provoke eesis or reduce C caused by intraab doinal pressure  ontinuous ositie irway ressure () a elivered via snug nasal ask or face ask b seful for low C eg, atelectasis, pneuonia and extrathoracic obstruction eg, obstructive sleep apnea, postextubation edea, laryngotracheitis  ileel ositie irway ressure (i) a Provides constant positive pressure during expiration and additional positive pressure support during inspiration b ay adust inspiratory and expiratory pressures separately to change tidal volue c seful for neurouscular weakness, spinal inury or ventilation failure

CIRCULATORY SUPPORT DURING RESUSCITATION PEARL Cardiopumonar Resuscitation Ratios PAL 22 Single rescuer 3 compressions 2 reats Multiple rescuers 1 compressions 2 reats danced airway ontinuous compressions 1 reat eery 2–3 seconds 2–3min

12

 ascular access See Chapter  Procedures for all procedurerelated details  eripheral intraenous access see igure  a General guide  gauge for infants,  gauge for children, gauge for adolescents and adults b rauaunstable patients insert the largest gauge possible  Intraosseus access see igure  a Allows for rapid establishent of access if peripheral intravenous access unsuccessful b Coon sites proxial tibia, distal feur, edial alleolus, proxial huerus, sternu see Chapter  Procedures  entral enous access see igures  and , able   mbilical catheters ubilical vein access usually viable for the first  days of life see igures – and newborn resuscitation in Chapter  eonatology for further details  rterial access see igure  a Provide continuous and accurate blood pressure onitoring b elpful if freuent arterial gas easureents are needed

Resuscitation

 Highuality R  ush hard  of anteroposterior diaeter of chest and ast –in  Allow coplete chest recoil ¨is is necessary for blood to ll the heart  iniie interruptions in copressions Cardiac output increases progressively with consecutive copressions  Avoid excessive ventilation  otate copressor every  inutes or sooner if fatigued  ndtidal C onitoring ay be considered to evaluate the uality of chest copressions PALS  suggests targeting copressions to an C value of at least  g, and ideally  g or greater, ay be useful as a arker of CP uality

1

 Inotropic and asopressor therapy  See able   Inotropes increase cardiac contractility, whereas asopressors increase systemic ascular resistance S

13

asoactie Aents Effects on Mocardium b1, b2 agonist alpaadrenergic eects at iger doses

h contractility h R

outamine

b1 agonist

Epineprine

orepineprine

opamine

Effects on ascuature

Commentsndications

ntermediate dose –1 mcg gmin b1, b2 agonist

h contractility, eart

ig dose .1 mcggmin alpaadrenergic eects predominate

h SR

mportant or distriutie soc

h contractility h R

b1 agonist

imited eect

mportant in eart ailure and cardiogenic soc

b1, b2 agonist

h contractility h R

a1, a2 agonist t iger doses te alpaadrenergic receptor eect predominates

h SR iger dose

mportant in anaylaxis and cold soc starting dose typically . mcggmin

b1 agonist

imited eect

a1, a2 agonist

h SR

mportant in warm septic soc wit asodilation starting dose typically . mcggmin

asopressin receptor 1 1R

h SR

May e added to norepineprine to raise mean arterial pressure or decrease norepineprine dose

PE  niitor

g SR

Potential role in some congenital eart diseases or oter conditions in wic cardiac aterload sould e reduced; use guided y consultation wit critical care expertise

asopressin Milrinone

Resuscitation

Medication

1

14

Tale 1.

PE  iniitor

h contractility

HR, Heart rate PDE, phosphodiesterase SVR, sstemic asclar resistance.

mportant or cardiogenic soc

rate, cardiac output

ADVANCED AIRWAY MANAGEMENT  ediatric airway anatomy and respiratory physiology  natomic eatures o the pediatric airway see Box  Anatomic Features of the Pediatric Airway

Anatomic eature • ccipital prominence nec exed in supine position • arrow nasal passages • arge tongue • arge tonsils and adenoids • Epiglottis large and accid wit less tensile yoepiglottic ligament • arrow cricoid • lottis ig and superior • Sort tracea

 ediatric respiratory physiology a inute oxygen consuption is higher in children copared with adults  Lkgin vs  Lkgin b Children have liited ability to adust tidal volue alveolar ventilation is priarily controlled by respiratory rate c Children desaturate uickly if apneic because of their higher oxygen consuption and the strong eect of respiratory rate on alveolar ventilation

Resuscitation

Box 11

1

 aryngeal mask airway ()  A supraglottic airway device which is not a secure airway ie, potential for aspiration of gastric contents but can be life saving when ask ventilation or intubation is dicult PEARL Cuffed ETT iin for 1–1 rs ge in years 1 3. PS 22 guidelines now recommend cued ETTs or all patients reuiring intuation ETT ept .1 r engt cm at lip 5 ge2 112 R 3 3 diameter o ETT engt cm at nose 5 ge2 11

 Endotracheal intubation  Indications a nadeuate oxygenation or ventilation despite optiiing noninva sive support b nability to aintain andor protect the airway eg, pooling secre tions, central nervous syste depression with loss of airway re¯exes

1

c Potential for loss of airway eg, epiglottitisbacterial tracheitis, severe anaphylaxis, severe burns d nstable patient reuiring transportprolonged diagnostic studies PEARL

Resuscitation

ntuation Euipment Cecist AP ME uction xygen Airway Euipment oralnasal airways, agalemas, endotraceal tues, stylets, laryngoscope andles and lades, tape, laryngeal mas airway, ideo laryngoscope, etc. Parmacology Tale 1. Monitoring Euipment Electrocardiogram monitor, pulse oximeter, lood pressure P monitor, 2 detectorendtidal 2

1

Tale 1.

ntuation Euipment iin  Ae and eit

Ae

A t 

ETTa mm

Bade

LMA

uction 

irt

3.

2.–3.

 straigt

1–1.

–

 mo

.

3.

1 straigt

1.–2



1 yr

1

.

1 straigt

1.–2



2 yr

12

.

2 straigt

2

–1

3 yr

1

.–

2 straigt or cured

2

1

 yr

1

.

2 straigt or cured

2

1

 yr

2

.

2 straigt or cured

2–2.

1

 yr

2

. 

2 or 3 cured

2.

1

2–

.–. 

3 cured

3–

1–12

–12 yr a

Stract 0. for cffed ETT sie. AS 2020 gidelines no recommend cffed ETTs for all patients reiring intation. C, Cffed endotracheal te ETT, endotracheal te LMA, larngeal mask aira mo, month , ear.

16

 Rapid Seuence Intubation (RSI) S refers to a seuential process to facilitate safe eergency tracheal intuba tion ¨e steps involved include preparation preoygenation premedi cation (optional) sedation and paralysis positioning tube placement and conrmation postintubation management  reparation a ocused history and physical to look or highrisk conditions i nown dicult airway ii Presentipending upper airway obstruction iii Anatoical challenges eg, liited cspine obility, acroglossia, icrognathia Also see igure  for allapati classication iv Physiologic risk factors eg, heodynaic instability, severe astha

b ontingency plan for failed intubation backup approach and additional airway specialists c Euipment check “S E” Also see able  for intubation euipent siing by age and weight iure 1 Maampati Cassication Hard palate Soft palate Resuscitation

Uvula Pillars

Class 1

Class 2

Class 3

Class 4

Classes  and  associated with relatively easy intubation, classes  and  associated with increased diculty ro ohnson BL Conscious sedation n e niversit of Cincinnati esidents e Mont eid Surgical andboo: Mobile Medicine Series th ed lsevier 

 reoygenation to axiie tolerable apnea tie a Apply   as soon as decision to intubate is ade b onrebreather ask for spontaneously breathing patients B ventilation for inadeuately breathing patients c Apneic oxygenation during intubation process B should be avoided a§er induction  remedication these edications are optional for selected patients and not routinel given a tropine no longer routinely recoended for pretreatent before endotracheal intubation PALS  but ay be useful in infants , yr of age predilection for vagalinduced bradycardia or other patients at risk of progressive unstable bradycardia b europrotective agents entanyl or lidocaine ay attenuate any ad ditional increase in intracranial pressure CP associated with laryngos copy and intubation in high risk patients ust be given , inutes before intubation Clear evidence for eectiveness lacking in children

1

PEARL Tere are many strategies aailale or rapid seuence intuation RS medications. Te ideal sedaties and paralytics or RS will dier depending a ariety o clinical actors emodynamic instaility, status astmaticus, status epilepticus, increased intracranial pressure, etc..

1

 Sedation and paralysis see able  for S edications a Sedation iportant to ensure anesia, analgesia blunt sypathetic response to laryngoscopy and to optiie intubating conditions b Sedation should always precede paralysis c Paralytic edications ay not be needed if a patient is copletely unresponsive Tale 1.

Medications for Rapid euence ntuation

Premedication

Resuscitation

tropine

.2 mggdose  max . mg single dose, 1 mg cumulatie dose

ot gien routinely; consider wen tere is iger ris or radycardia

idocaine 2

1–2 mggdose 

ot gien routinely; consider in cases o increased P eidence inconsistent

.3 mggdose  oer 3– s

oid in septic soc adrenocortical suppression

edaties Etomidate

Maintains emodynamic staility ecreases P appropriate or ead trauma

1

entanyl

2– mcggdose  oer 3– s

 patient is in soc sould start at lower dose and titrate to eect

etamine

1–2 mggdose 

Preerred agent in context o septic soc, and astma. ppropriate coice or ypotensie patients

Midaolam

.1–.2 mggdose 

oid use in patients wit emodynamic compromise myocardial depressant ppropriate coice or status epilepticus

Propool

1–2 mggdose ; dose an cause asodilationypotension; aoid use in depends on emodynamic situations o emodynamic compromise state o patient. Start low and titrate to eect

Paratics Rocuronium

1–1.2 mggdose  or RS

ewer side eects tan succinylcoline ypertension or transient ypotension possile Proides longer paralysis

Succinylcoline

1–2 mggdose 

Sortacting

ie single dose and aoid repeated dosing

Side eects yperalemia, ypertension, ypoten sion, arrytmia, increased intraocular pressure, increased intragastric pressure  T SE wit renal insuciency, yperale mia, urns, crus inuriespolytrauma, extensie deneration o seletal muscle or upper motor neuron inury, positie personal or amily istory o malignant ypertermia T wit unidentied, undiagnosed, or unex plained neurodeelopmental delay or muscular illness

18

CP, ntracranial pressre O, intraosseos V, intraenos. Adapted from The Hospital for Sick Children eormlar, 2020.

Resuscitation

 ositioning for direct laryngoscopy a Laryngoscope should be held in operators le§ hand and inserted into right side of patient’s outh, sweeping the tongue to the le§ b ¨e laryngoscope blade should be advanced into the vallecula acintosh blade, curved before applying an upwardforward force along the long axis of the laryngoscope, at about  degrees, to li§ the epiglottis and expose the glottic opening n infants and younger children, a straight blade iller is used this is designed for posi tioning below the epiglottis, to directly li§ that structure o the glottic opening igures  and  c Eternal laryngeal manipulation ay iprove the view in soe patients backwardupwardrightward pressure BP is applied to the larynx by an assistant d n young infants, gentle cricoid pressure “Sellick aneuver” on the anterior neck ay iprove glottis view PALS  no longer recoends routine use of cricoid pressure during intubation PEARL To ncrease xenation h Mean airway pressure especially PEEP h i2

1

To ncrease entiation h Respiratory rate RR h Tidal olume

iure 1 irect Larnoscope Bades

Epiglottis

Trachea

A Curved blade

B Straight blade

A Curved acintosh blade in vallecula B Straight iller blade underneath epiglottis ro Schoeld S, Sith  ndotracheal intubation n Caeron P, Browne G, itra B, et al, eds etboo of Paediatric mergenc Medicine rd ed lsevier 

1

iure 1 Cormac Leane rades

Grade II

Grade III

Grade IV

Resuscitation

Grade I

Grade  full view of glottis grade  partial view of glottis grade  only epiglottis seen grade  neither glottis nor epiglottis seen ro iller , Pardo  Basics of Anesthesia

1

th ed Philadelphia, PA Saunders 

 lacement o tube and conrmation a Conr tube placeent Box  b Secure  at a corner of the outh with strong adhesive tape Box 12

Verication of Endotracheal ue Placement a

•  irect laryngoscopy gold standard, condensation in ETT, eual cest moement ilaterally • STE ilateral reat sounds consider rigt mainstem intuation i reat sounds eard only on rigt; listen oer stomac or gastric insuation i ETT is esopageal • MTR Pulse oximetry, endtidal 2 monitoring capnograpy or colorimetric 2 detectors • ME est R, pointocare ultrasound a

o single method is niersall or completel reliale erication of ETT placement reires a mltiple method approach. ETT, Endotracheal te R, ra.

20

 ostintubation management a ngoing sedation, analgesia and paralysis as reuired b Positive pressure ventilation by hand until transferred to ventilator seek guidance, as needed, to establish appropriate tidal volues, pressure and rates on ventilator, igure  c or sudden deterioration of an intubated patient, address ost coon causes using the “P” neonic

PEARL PE mneumonic or causes o deterioration in an intuated patient isplacement o endotraceal tue ETT struction o ETT Pneumotorax Euipment ailure rom Pediatric danced ie Support. 21 merican eart ssociation uidelines or ardiopulmo nary Resuscitation and Emergency ardioascular are. Circulation. 21;122S–S.

Parameters

Guidelines

Servo I/U Mode Selection

SIMV PC + PS

PIP is set, Vt is variable PIP 15–25 cmH2O to achieve Vt 6–8 mL Commo moe or iitial vetilatio sort

SIMV PVC + PS

Resuscitation

iure 1 enera entiation nitiation uideines

Vt is set, PIP is variable Vt 6–8 mL Potetial or barotrama i atiets ith ecrease l comliace or siificat aira resistace   tbe lea reers this moe ificlt

CPPPS

Patiet triers all breaths Cotios PP ith PS set to obtai Vt –8 mL roriate or sotaeosl breathi atiets se l as a eai moe

RR (set)

• ebor term

25–5

se tC2  to otimie

• 1 mos–1 ear

2–

• 1 ear–6 ear

15–25

• 6 ears

15–2

Inspiratory: Expiratory time (sec)

he isiratoreirator I time ratio is ticall 12 or 1 I obstrctive cases sch as asthma the eirator time is set to be roortioall loer e, 1, 15, 16 he absolte isirator time ill ths ee o the resirator rate aroriate or ae a athohsiolo

PEEP (cmH)

o lmoar isease

5

e, cariac, seires, hea trama

Plmoar isease

5–15

e, brochiolitis, emoia, S, roi

bomial istetio

5–1

e, liver traslat, ascites, fli overloa

i

• aret SO2 rae eerall 2 or those ith ormal cariac aatom

PS

• eeral oo starti oit is 1 cmH2O rae 6–2 cmH2O

1

¨is is a general reference only ptial settings will vary with patient condition, available euip ent and institutional guidelines ABG, Arterial blood gas CPAP, continuous positive airway pressure PC, pressure control PIP, peak inspiratory pressure PRVC, pressure regulated volue control PS, pressure support SIMV, synchronied interittent andatory ventilation Vt, tidal volue © ¨e ospital for Sick Children Adapted fro ¨e ospital for Sick Children eference for the entilation of the Pediatric Patient, 

21

E Highrisk intubations  Risk actors on history a nown history of dicult ventilation or intubation b Present or ipending upper airway obstruction eg, epiglottitis, airway burns, soke inhalation, traua, tuor, abscess, angioedea PTALL igris intuations sould ideally ae an airway expert present. Tis includes specialties, suc as otolaryngology, anestesia, or anoter proider wit airway experience.

Resuscitation 1

22

 natomic risk actors a Atlantoaxial subluxation eg, own syndroe b acroglossia eg, own syndroe, Beckwithiedean c etrognathia or icrognathia eg, Pierreobin, reacher Collins, and Goldenhar syndroe d ther abnoralities in craniofacial developent eg, Apert, Crouon, and Pfeier syndroes e Liited outh opening, poor teporoandibular oint function eg, uvenile idiopathic arthritis f Liited cervical spine obility eg, lippeleil, ucopolysacchari doses or atlantoaxial instability eg, own syndroe g allapati classication see igure  i ay be useful in children . yrs not helpful in sall children ii Grades  and  ay predict a relatively easy intubation grades  and  ay predict a dicult intubation  Situational risks a Shock patients in shock are at high risk of further deterioration and cardiac arrest during intubation Provision of positive pressure ventilation a§er intubation can also severely ipair venous return and lead to circulatory collapse eodynaic status should be optiied before and a§er intubation ¯uids, vasoactive agents, as needed b sthma children with severe acute astha o§en have increased intrathoracic pressure fro alveolar obstruction and hyperin¯ation echanical ventilation of these patients is challenging and should be avoided unless absolutely necessary c leeding disorders children have large tonsilsadenoids that are susceptible to traua and bleeding, especially with repeated intuba tion attepts Children with bleeding disorders should have their coagulation prole optiied before procedures d iabetic ketoacidosis children with diabetic ketoacidosis use deep, fast breathing to decrease arterial carbon dioxide levels to copensate for their etabolic acidosis ntubation is typically

Resuscitation

avoided if possible in these patients as it reoves the ability of the body to selfregulate its breathing in response to its seru p echanical ventilation settings ust be adusted accordingly e ediastinal mass a ass ay result in distal tracheal copres sion, leading to coplete airway obstruction, and vascular collapse if thoracic tone is diinished by sedation or paralysis Sedation and intubation should be avoided and only perfored under controlled conditions in consultation with airway experts f alignant hyperthermia potentially fatal autosoal doinant inherited disorder that leads to a hyperetabolic crisis in susceptible patients exposed to volatile anesthetic or succinylcholine i Signs and syptos hypercarbia, tachycardia, tachypnea, uscle rigidity, hypertheria ii Laboratory ndings ixed respiratory and etabolic acidosis, hyperkaleia, raised creatine kinase, yoglobinuria, renal failure, disseinated intravascular coagulation iii reatent  Cease trigger drug  Adinister    antrolene  gkg  rapidly further doses of  gkg  ay be given until clinical iproveent, ie, decreasing teperature, heart rate, uscle rigidity up to a cuulative dose of  gkg—although ore ay be needed under adviseent of experienced practitioner  Active cooling ice bags, cold water lavage, cold  noral saline  onitor and correct ¯uid and electrolyte disturbances  reat acidosis, arrhythias, rhabdoyolysis, and coagulopa thy as reuired  Close onitoring intensive care unit, supportive ventilation

1

PALS ALGORITHMS igure  Pediatric cardiac arrest algorith igure  Pediatric bradycardia with a pulse algorith igure  Pediatric tachycardia with a pulse algorith igure  Pediatric septic shock algorith

23

iure 11 Pediatric Cardiac Arrest Aoritm 1 StartPR • Begin bag-mask ventilation and give oxygen • Attach monitor/defibrillator

Yes

CPR quality

o

Rhythm shockable?

2

 Asystole/PEA

VF/pVT

Resuscitation



Epinephrine ASAP

Shock



1 PR2min IV/IO access

Rhythm shockable?

o

PR2min • IV/IO access • Epinephrine every 3– min • onsider advanced airay and canograhy

Yes 

1

Rhythm shockable?

Shock

 PR2min • Epinephrine every 3– min • onsider advanced airay

Yes

No 11

Rhythm shockable?

o

CPR 2 min Treat reversible causes

Shock enery or deibrillation • irst shoc 2 / • econd shoc  / • ubseuent shocs ≥ / maimum 10 / or adult dose ru theray • inehrine  dose 001 m/ (01 m/ of the 01 m/m concentration) a dose 1m epeat ever 3– minutes f no / access ma ive endotracheal dose 01 m/ (01 m/ of the 1 m/m concentration) • Amiodarone  dose  m/ bolus durin cardiac arrest a repeat up to 3 total doses for refractor /pulseless T or idocaine  dose nitial 1 m/ loadin dose Advanced airway

Yes o

7

• Push hard (≥1/3 of anteroposterior diameter of chest) and fast (100–120/min) and allow complete chest recoil • inimie interruptions in compressions • hane compressor ever 2 minutes or sooner if fatiued • f no advanced airwa 12 compressionventilation ratio • f advanced airwa provide continuous compressions and ive a breath ever 2–3 seconds

Shock

Rhythm shockable?

• ndotracheal intubation or supralottic advanced airwa • aveform capnoraph or capnometr to confirm and monitor T tube placement

Yes

Reversible causes

 PR2min • Amioaroneor liocaine • reat reversible cases

• • • • • • • • • • •

12 • If no signs of retrn of sontaneos circlation O go to 1 • If O go to ost-ardiac Arrest are checklist

Go to 7.

Hpovolemia Hpoia Hdroen ion (acidosis) Hpolcemia Hpo/hperalemia Hpothermia ension pneumothora amponade cardiac oins hrombosis pulmonar hrombosis coronar

© 2020 merican eart ssociation

CPR, Cardiopulonary resuscitation IO, intraosseous IV, intravenous PEA, pulseless electrical activity pVT, pulseless ventricular tachycardia VF, ventricular brillation ro Pediatric Basic and Advanced Life Support  Aerican eart Association Guidelines for Cardiopulonary esuscitation and ergency Cardiovascular Care Circulation S–S Copyright

24

 Aerican eart Association, nc

iure 111 Pediatric Bradcardia it a Puse Aoritm Patient with bradycardia

Cardiopulmonary compromise? • Acutely ltered mentl sttus • Signs o shock • ypotension

No

Assessment and support • intin ptent iry • Assist brething ith positive pressure ventiltion nd oxygen s necessry • Crdic monitor to identiy rhythm monitor pulse B nd oximetry

• • • • •

Support ABCs Consider oxygen Observe 12-ed CG dentiy nd tret underlying cuses

Resuscitation

Yes

Strt C i  min despite oxygention nd ventiltion

1 radycardia persists?

No

Yes

• Continue C i  min • O ccess • Epinephrine • Atropine or incresed vgl tone or primry A block • Consider trnsthorcictrnsvenous pcing • dentiy nd tret underlying cuses

Yes

Check pulse every 2 minutes Pulse present? No Go to Pediatric Cardiac Arrest Algorithm

Doses/Details Epinephrine IV/I dose 1 mgkg 1 mkg o the 1 mgm concentrtion epet every – minutes  O ccess not vilble but endotrchel  tube in plce my give  dose 1 mgkg 1 mkg o the 1 mgm concentrtion Atropine IV/I dose 2 mgkg y repet once inimum dose 1 mg nd mximum single dose  mg Possible Causes • ypothermi • ypoxi • edictions © 22 Americn ert Assocition

ro Pediatric Basic and Advanced Life Support  Aerican eart Association Guidelines for Cardiopulonary esuscitation and ergency Cardiovascular Care Circulation  S–S Copyright  Aerican eart Association, nc

2

iure 112 Pediatric Taccardia it a Puse Aoritm Initial assessment and support • Maintain patent airway; assist breathing as necessary • Administer oxygen • Cardiac monitor to identify rhythm; monitor pulse, blood pressure, and oximetry • IV/I access • ead C if aailable Probable sinus tachycardia if • P waves present/normal • Variable RR interval • Infant rate usually 220/min • Child rate usually 180/min

Resuscitation

Search or and treat cause

Narrow (0.09 sec)

1

Yes

Evaluate S duration

Probable supraventricular tachycardia • P waves absent/abnormal • RR interval not variable • Infant rate usually ≥220/min • Child rate usually ≥180/min • istory of abrupt rate hane

• If IV/I aess is present ive adenosine O • If IV/I aess is not available or if adenosine is ineffetive perform synhronied ardioversion

Wide (0.09 sec)

Possible ventricular tachycardia

Synchronized cardioversion pert onsultation is advised before additional dru therapies

© 2020 merian eart ssoiation

Synchronized Cardioversion ein with 0–1 / if not effetive inrease to 2 / edate if needed but don’t delay ardioversion Drug therapy Adenosine IO/IV dose • irst dose 01 m/ rapid bolus maimum  m • eond dose 02 m/ rapid bolus maimum seond dose 12m

Evaluate rhythm ith lead EC or monitor

Cardiopulmonary compromise • utely altered mental status • ins of sho • ypotension

Doses/Details

No

arro  sec

Evaluate S duration

Probable supraventricular tachycardia • P waves absent/abnormal • RR interval not variable • Infant rate usually ≥220/min • Child rate usually ≥180/min • istory of abrupt rate hane

Consider vagal maneuvers

ide  sec

Possible ventricular tachycardia

If rhythm is regular and R monomorphic onsider adenosine

Epert consultation is recommended

If IV/I aess is present ive adenosine

ro Pediatric Basic and Advanced Life Support  Aerican eart Association guidelines for cardiopulonary resuscitation and eergency cardiovascular care Circulation S–S Copyright  Aerican eart Association, nc

26

iure 113 Pediatric eptic oc Aoritm Identify signs of septic shock (as below or per protocol) • Altered mental status (irritability or decreased level of consciousness) • Altered heart rate (tachycardia or, less commonly, bradycardia) • Altered temperature (fever or hypothermia) • Altered perfusion (prolonged or “flash” capillary refill; cool or very warm extremities; plethoric appearance, mottled color or pallor; possible ecchymosis or purpura; decreased urine output) • Hypotension ay or may not be present

irst hour

Initial Stabilization • onitor and support airway, breathing, and circulation • onitor heart rate, blood pressure, and pulse oximetry • stablish vascular access ( or ); draw blood for culture and additional laboratory studies, including glucose and calciumdo not delay antibiotic or fluid therapy • Antibiotics ive broadspectrum antibiotics • Fluid boluses ive  mg isotonic crystalloid boluses ( mg for neonates and those with preexisting cardiovascular compromise) Assess carefully after each bolus epeat as needed to treat shoc top if rales, respiratory distress, or hepatomegaly develops • ive antipyretics if needed Goals of therapy mproved mental status, normaliation of heart rate and temperature, adeuate systolic and diastolic blood pressure, improved perfusion (see box above)

Resuscitation

mmediate (– min)

o sins of shoc persist (see box above) onsider critical care consultation

• Obtain expert/critical care consultation • Initiate and titrate vasoactive drugs: • Cold extremities, delayed capillary refill, and/or low blood pressure : Epinephrine (use dopamine if epinephrine is not available) • Warm extremities, “flash” capillary refill, and/or low (typically diastolic) blood pressure: orepinephrine (use hiher dose of dopamine if norepinephrine is not available)

Initial stabilization Therapies below this line are intended for critical care environment and expertise

1 • Establish central venous and intraarterial pressure monitorin • ontinue epinephrine/norepinephrine (as above) and bolus fluid therap as needed to treat shoc • erif adeuate airwa oxenation and ventilation • Evaluate cortisol if at ris for relative adrenal insufficienc consider stressdose hdrocortisone Critical care goals of therapy: cvO ≥ adeuate  normalized  adeuate cardiac output/index and oran perfusion

Is cvO ≥

Scv  ith poor perfusion and cold extremities despite epinephrine administration

• • • •

rovide additional fluid boluses as needed Transfuse if b  /d ontinue epinephrine therap f  low: dd norepinephrine if diastolic  low consider additional inotropic and vasoactive dru therap as needed • f  adeuate: dd milrinone and/or additional vasodilator therap consider addin inotropic dru • upport oran function oals of care Improved cvO normalized  and  adeuate cardiac output/index and oran perfusion

Scv ≥ ith poor perfusion and warm extremities despite norepinephrine administration

Scv ≥ igns of shoc resolved

• rovide additional fluid boluses as needed • ontinue norepinephrine therap • dd additional vasopressor therap and inotropic therap as needed • upport oran function oals of care Improved cvO normalized  and  adeuate cardiac output/index and oran perfusion

• onitor in I • upport oran function • Treat infection source • Evaluate septic shoc prevention detection and therap

©  merican eart ssociation

(odified from rierle  arcillo  hoon  et al linical practice parameters for hemodnamic support of pediatric and neonatal septic shoc:  update from the merican ollee of ritical are edicine Crit Care Med :–)

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POST-RESUSCITATION CARE igure  anageent of shock a§er return of spontaneous circulation algorith iure 114 Post CardiacArrest Care Cecist Components of Post-Cardiac Arrest Care

Cec

Oxygenation and ventilation Measure oxygenation and target normoxemia 94%–99% (or child’s normal/appropriate oxygen saturation). Measure and target Paco2 appropriate to the patient’s underlying condition and limit exposure to seere hypercapnia or hypocapnia.

Resuscitation

Hemodynamic monitoring et speciic hemodynamic goals during postcardiac arrest care and reie daily. Monitor ith cardiac telemetry. Monitor arterial lood pressure. Monitor serum lactate urine output and central enous oxygen saturation to help guide therapies. se parenteral luid olus ith or ithout inotropes or asopressors to maintain a systolic lood pressure greater than the ith percentile or age and sex. Targeted temperature management (TTM) Measure and continuously monitor core temperature.

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Preent and treat eer immediately ater arrest and during rearming  patient is conatose apply M (2°–4°) olloed y (°–.°) or only M (°–°). Preent shiering. Monitor lood pressure and treat hypotension during rearming. Neuromonitoring  patient has encephalopathy and resources are aailale monitor ith continuous electroencephalogram. reat seiures. onsider early rain imaging to diagnose treatale causes o cardiac arrest. lectrolytes and glucose Measure lood glucose and aoid hypoglycemia. Maintain electrolytes ithin normal ranges to aoid possile liethreatening arrhythmias. edation reat ith sedaties and anxiolytcs. Prognosis lays consider multiple modalities (clinical and other) oer any single predictie actor. ememer that assessments may e modiied y M or induced hypothermia. onsider electroencephalogram in conunction ith other actors ithin the irst  days ater cardiac arrest. onsider neuroimaging such as magnetic resonance imaging during the irst  days.

ro Pediatric Basic and Advanced Life Support  Aerican eart Association Guidelines for Cardiopulonary esuscitation and ergency Cardiovascular Care Circulatin

28

S–S Copyright  Aerican eart Association, nc

Resuscitation

 oals o postresuscitation care  resere brain unction preent secondary hypoic inury acilitate transer to tertiary care center with appropriate personnel and euipent a eperature fever should be aggressively treated a§er return of spontaneous circulation SC Continuous teperature onitor ing and cooling should be used to aintain norotheria ¨era peutic hypotheria ay be indicated in certain cases discuss with local expert b xygen providers ay wean suppleental oxygen to target satura tions of  to  to avoid both hypoxeia and hyperoxia c Carbon dioxide liit exposure to severe hypercapnia or hypocapnia d Circulation use ¯uids andor inotropesvasoactive drugs to aintain systolic blood pressure higher than the percentile for age e Agitationseiures treat pain with analgesics opioids and keep child sedated benodiaepines reat clinical seiures and apply electro encephalogra onitoring for patients who reain coatose a§er resuscitation potential for subclinical seiures

FURTHER READING Aerican Acadey of Pediatrics etboo of Neonatal esuscitation th ed lk Grove illage, L Aerican Acadey of Pediatrics and Aerican eart Association  Aerican eart Association Pediatric Advanced Life Support Provider Manual allas,  Aerican eart Association  de Caen A, Berg , Chaeides L, et al Part  Pediatric Advanced Life Support  Aerican eart Association Guidelines pdate for Cardio pulonary esuscitation and ergency Cardiovascular Care Circulation S leinan , Chaeides L, Schexnayder S et al Part  Pediatric Advanced Life Support  Aerican eart Association Guidelines for Cardiopulo nary esuscitation and ergency Cardiovascular Care Circulation  S–S opian A, ayond , Atkins , et al Part  Pediatric Basic and Advanced Life Support  Aerican eart Association Guidelines for Cardiopul onary esuscitation and ergency Cardiovascular Care Circulatin S–S

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2

CHAPTER

2

Emergency Medicine Marie-Pier Lirette • Maya Harel-Sterling • Iwona Baran • Suzanne Beno

Common abbreviations Useful calculations and values Circulatory emergencies Respiratory emergencies Anaphylaxis Neurological emergencies Environmental emergencies Brief resolved unexplained event (BRUE) Acute psychosis Burns Approach to trauma pecic inuries in pediatric trauma

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COMMON ABBREVIATIONS 0.9% NaCl AC A AC A C  C C A N C A A    tA 

0.9% sodium chloride (normal saline) aira breathing and circulation arterial blood gas altered level of consciousness advanced pediatric life support extracorporeal membrane oxgenation endotracheal tube lasgo Coma cale intracranial pressure focused assessment ith sonograph for trauma hpertension level of consciousness pediatric advanced life support pediatric respirator assessment measure positive pressure ventilation rapid seuence intubation sstemic vascular resistance tissue plasminogen activator venous blood gas

USEFUL CALCULATIONS AND VALUES . Estimated weight in kilograms (APLS) a. – months 0. 3 (age in months) 1  b. – ears  3 (age in ears) 1  c. – ears  3 (age in ears) 1  . Normal ranges for heart rate, respiratory rate, and lood pressre () for dierent ages ee gure in back inside cover of book . ypotension in children (sstolic ) a. erm neonates (, month) ,0 mmg b. – months ,0 mmg c. –0 ears ,0 1  3 (age in ears) mmg d. .0 ears ,90 mmg . lid ols in emergenc situations 0 mkg of 0.9% NaCl (normal saline)

Emergency Medicine

Also see page xviii for a list of other abbreviations used throughout this book

2

CIRCULATORY EMERGENCIES SHOCK . enitions a. Shock phsiological state in hich the cardiovascular sstem fails to deliver adeuate oxgen and nutrients to meet tissue demand i. ompensated shock an earlier form of shock characteried b normal . Compensator mechanisms to maintain cardiac output tpicall include tachcardia (oen the most sensitive

31

marker) and increased sstemic vascular resistance (). ¡is is a reversible state but if unrecognied and untreated progresses to decompensated shock. ii. ecompensated shock shock ith hypotension. ¡is is oen a late nding in children because of the abilit to compensate as described earlier. f untreated decompensated shock rapidl leads to organ dsfunction cardiovascular collapse and arrest. . Etiology a. ee able . for general classication and common causes of shock Emergency Medicine

able 1

Types o Shoc

Type

Common Causes

Pathophysiology

HR

SVR

Hypovolemic (most common)

Fluid loss (Gastroenteritis, burns)

g Intravas-

h

h

Hemorrae

2

ardioenic

Distributive (Derangement in vascular tone)

32

cular volume (decreased preload)

Clinical Features

Treatment

Dry mucous membranes, cool extremities, delayed cap rell, decreased urine output, depressed mental status

Fluids (crystalloid) Hemorrae control, ps

h

h

ten similar to ypovolemic soc Features more specic to cardioenic soc - Gallop rytm - Dilated nec veins - Hepatomealy -  cardiomealy 1 pulmonary conestion

udicious uid use, Inotropic support,  as needed

I-mediated release o istamine (vasodilator)

h

g

ins o anapylaxis (urticaria, anioedema, eee, GI upset)

ee Fiure 

oss o sympatetic tone leads to vasodilation

g

g

nopposed vaal activity leads to bradycardia (or absence o reex tacycardic response to ypotension), as capillary rell

Fluids, vasoactive aents

rrytmia, yocarditis, ericarditis, ardiomyopaty, onenital eart disease, ostoperative complications o cardiac surery, Dru inestions metabolic deranements

g ardiac

napylactic soc

euroenic soc (pinal cord trauma)

output because o decreased myocardial contractility

Type

Types o Shoc—cont’ Common Causes

eptic

bstructive

ardiac tamponade ension pneumotorax

Dissociative

arbon monoxide poisonin, etemolobinemia (see apter 3 oisonins and oxicoloy)

Pathophysiology

Clinical Features

HR

SVR

evere inammatory reaction to inection leads to myocardial dysunction, capillary lea, and vasomotor instability

h

h (cold

ecanical obstruction to let ventricular ino or outo

h

h

ued eart sounds, distended nec veins

ericardiocentesis

h

h

raceal deviation aay rom side o pneumotorax, narro pulse pressure

eedle decompression cest tube drainae

 not released rom emolobin

h

yanosis, neuroloical symptoms, coma

xyen

shock)

g (warm shock)

h6

“old” soc mottled sin, cool extremities, proloned capillary rell, ea pulses ost common in pediatric patients

Treatment Fluids, antibiotics, vasoactive aents ee Fiure 1

Emergency Medicine

able 1

“arm” soc arm extremities, as cap rell, boundin pulses, ide pulse pressure ost common in adults

2

CXR, Chest x-ray; ECMO, extracorporeal membrane oxygenation; GI, gastrointestinal; HR, heart rate; IgE, immunoglobulin E; pRBC, packed red blood cell; SVR, systemic vascular resistance.

. linical manifestations a. All forms of shock involve some degree of absolute or functional hpovolemia (from ¢uid losses or decreased  respectivel) and impaired tissue perfusion b. arl clinical manifestations of shock include tachcardia tachpnea decreased urine output and nonspecic smptoms of poor feeding and irritabilit. ate clinical manifestations of shock include hpoten sion altered level of consciousness and eak pulses. c. ¡e various tpes of shock and aspects of their clinical presentation are shon in able .

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PTFA Hypotension is a late ndin in pediatric soc and indicates impendin cardiovascular collapse linicians sould suspect and immediately treat soc in cildren it onoin tacycardia and sins o impaired perusion

Emergency Medicine 2

34

. nestigations a. Laoratory stdies i. igns of infection CC 1 dierential blood culture 6 urine studies ii. igns of organ dsfunction liver inur (s coagulation prole) acute kidne inur (urea creatinine) metabolic disturbance (glucose calcium) hematological dsfunction (platelets) iii. igns of impaired tissue perfusion blood gas (p) lactate iv. epsis orkup as appropriate for age and presentation b. maging stdies C for respirator smptoms 6 other additional studies as guided b histor and phsical examination C echocardio gram for suspected cardiogenic shock etc.) . Management a. uccessful management relies on rapid recognition reversal of shock state and identication and treatment of underling cause. ¡e clini cian should immediatel address the ACs ensure a secure aira place supplemental oxgen and insert to large bore peripheral  lines for adeuate ¢uid resuscitation. All children should be on continuous cardiac and respirator monitoring. rine output should be recorded and patients should be monitored for signs of multiorgan failure. esscitation goals inclde restoration of normal heart rate, P, mental stats, and perfsion parameters (eg, cap rell #2 s) (see able .). SEPSS . enitions a. Systemic inammatory response syndrome (SS) in¢amma tor response ith to or more of the folloing criteria (one of hich must be abnormal temperature or leukocte count) i. Core temperature ..°C or ,°C ii. achcardia (or bradcardia if , ear) iii. achpnea iv. ncreased or decreased leukocte count b. Sepsis  ith suspected or proven infection c. Seere sepsis (sepsis with associated organ dysfnction) sepsis associated ith cardiovascular dysfunction (hpotension need for vasoactive agent metabolic acidosis impaired perfusion) or acute respiratory distress syndrome (ARDS)/need for mechanical ventilation or dysfunction in 2 other systems (hematological

thromboctopenia disseminated intravascular coagulation neurological change in mental status C # hepatic elevated bilirubin A renal increased creatinine) d. Septic shock sepsis ith cardiovascular dsfunction and an signs of impaired tissue perfusion such as altered mental status (irritabil it drosiness confusion letharg) skin capillar rell and pulse abnormalities (see able .) decreased urine output or hpotension

Goals o rst our resuscitation in septic soc are to 1 estoremaintain oxyenation and ventilation  estoremaintain circulation 3 dminister broad-spectrum antibiotics (see apter  Inectious Diseases)

. Management a. ee igure . and Chapter  nfectious iseases b. Clinical targets during resuscitation include improvement of heart rate and  mental status perfusion (cap rell etc.) lactate urine output

Emergency Medicine

PEAR

Figure 2 Emergency Room anagement o Septic Shoc  min

 min

Recognize decreased mental status and perfusion. Begin high flow O2, and establish IO/IV access according to P.

2

If no hepatomegal or rales/cracles, then push 2 m/g isotonic saline boluses and reassess after each bolus up to  m/g until improed perfusion. top for rales, cracles, or hepatomegal. orrect hpoglcemia and hpocalcemia. Begin antibiotics. Fluid refractory shock? hoc resistant to – cc/g IV fluids Begin peripheral IV/IO inotrope infusion, preferabl pinephrine .–. µg/g/min. se tropine / etamine IV/IO/I if needed for entral Vein or irwa ccess. itrate pinephrine .–. µg/g/min for old hoc. itrate central opamine – µg/g/min if pinephrine not aailable itrate central orepinephrine from . µg/g/min and upward to reerse arm hoc. itrate central opamine ≥  µg/g/min if orepinephrine not aailable

 min

Resuscitation goals include improvement of: • eart rate, blood pressure • ental status • Perfusion cap refill, etc. • rine output • erum lactate

Catecholamine-resistant shock? If at ris for bsolute drenal Insufficienc, consider drocortisone. danced hemodnamic management in PI.

IM ntramuscular IO intraosseous IV intravenous AS pediatric advanced life support. (odied from avis A Carcillo A Anea  et al. American College of Critical Care edicine Clinical ractice arameters for hemodnamic support of pediatric and neonatal septic shock. rit are Med 00.)

3

Emergency Medicine

HPERTESVE CRSS . enitions a. ee Chapter  Nephrolog and rolog for age and sex appropriate normal  values b. ypertensie rgency signicantl elevated  (tpicall   9th% 1  mmg for age sex height or .090 if age ears) ithout severe smptoms or evidence of endorgan inur c. ypertensie emergency signicantl elevated  (tpicall   9th% 1  mmg for age sex height or .090 if age  ears) ith evidence of severe smptoms or secondar organ inur (most commonl brain ees heart kidnes) . Etiology a. ee able . for causes of hpertensive emergencies based on age

able 

Common Causes o Hypertensie Emergencies ase on Age

Age

Cause

Inantyoun cild

enovascular causes (trombosis, stenosis) oarctation o aorta enal parencymal disease ndocrine cause (tyrotoxicosis)

cool aeadolescent

enal parencymal disease (H, H, poststrep G) enovascular (renal artery stenosis) ndocrine (tyrotoxicosis, peocromocytoma) edicationsrecreational drus ssential ypertension

2

HSP, Henoch-Schönlein purpura; HUS, hemolytic uremic syndrome; poststrep GN, poststreptococcal glomerulonephritis.

36

. linical manifestations a. Nerological dysfnction blurred vision headache confusion encephalopath seiures hemiplegiafacial pals b. ardiac dysfnction congestive heart failure palpitations shortness of breath peripheral edema eakabsent femoral pulse c. enal dysfnction reduced urine output edema d. Signs of endocrine disorder goiter abdominal striae headaches palpitations ¢ushing . nestigations a. Laoratory stdies i. Serm CC electroltes glucose urea creatinine. Consider  plasma renin (before initiating therap) as indicated. ii. rine urinalsis. oxicolog screen urinar catecholamines as indicated.

Emergency Medicine

b maging stdies C C 6 echocardiogram if cardiac patholog suspected C head for severe neurological dsfunction renal ultrasound () ith oppler for suspected renal arter stenosis . Management a. Adeuate  access should be obtained and cardiorespirator and  monitoring initiated b Eclde increased intracranial pressre (P) before loering  (need to have an adeuate mean arterial pressure to sustain cerebral perfusion pressure) c oal initial reduction in  to stabilie severel smptomatic patients then gradual decrease loer  b maximum % or to 9th percentile for age (hichever is lesser reduction) over rst  to  hours then gradual decrease to 90th to 9th percentile for age over next  to  hours d. ee able . for pharmacological management e. Children on  infusions should be monitored in an C setting. Consider arterial line or –0min noninvasive  monitoring. f. top infusions temporaril and ensure ¢uids (normal saline) are read for unanticipated large drops in 

2

able 3 eications

Pharmacological anagement o Acute Hypertension See rug Formulary or osing Class

Onset o Action

uration o Action

Comments

ntermittent osing in Acute Hypertension Hydralaine I

Direct vasodilator

10–30 min

–1 

– dustment in renal impairment – Adverse effects: associated it dru-induced lupus, may cause eadaces, tacycardia, increased I

abetolol I

a1 and b blocer

–10 min

– 

– ontraindicated in astma, peocromocytoma – Adverse effects: ausea vomitin, diiness, scalp tinlin, eart bloc, burnin troat sensation

iedipine 

alcium cannel blocer

0–30 min



– Do not use in neonates – Adverse effects: Diiness, usin, rebound ypertension Continued

3

able 3 eications

Pharmacological anagement o Acute Hypertension See rug Formulary or osing—cont’ Class

Onset o Action

uration o Action

Comments

Continuous nusion in Hypertensie Emergencies

Emergency Medicine

abetalol I

a1 and b blocer

–10 min

– 

– ontraindicated in astma, peocromocytoma – Adverse effects: ausea vomitin, diiness, scalp tinlin, eart bloc, burnin troat sensation

itroprusside I

Direct vasodilator

itin seconds

Durin inusion only

– void i h I or renal ailure – is o cyanide toxicity – Adverse effects: ausea vomitin, diaporesis, muscle titcin

smolol I

b1 blocer

itin seconds

10–0 min

– Adverse effects ay cause proound bradycardia

Once target  is reached options or oral maintenance medications include amlodipine or metoprolol.

2

BP, lood pressure; ICP, intracranial pressure; IV, intravenous PO, orally.

RESPIRATORY EMERGENCIES

38

ACTE ASTHA EACERATO Also see Chapter  espirolog for further information on asthma . enition a. Stats asthmatics severe asthma exacerbation refractor to appropriate medical therap . linical manifestations a. espirator failure h respirator rate cough ork of breathing accessor muscle use prolonged expiration heeing silent chest (heeing ma be absent in severe exacerbation because of poor air entr from bronchoconstriction) b. Cardiovascular h heart rate c. Central nervous sstem altered mental status if severe (hpoxia hpercarbia) . llness seerity a. PAM score (pediatric respirator assessment measure) can help determine level of severit (able .)

able 

Peiatric Respiratory Assessment easure PRA Score or Asthma Eaceration Seerity

PRA Score Peiatric Respiratory Assessment easure 



2



uprasternal retractions

bsent

resent

calene muscle retractions

bsent

resent

ir entry

ormal

Decreased at bases

idespread decrease

bsentminimal

eein

bsent

xpiratory only

Inspiratory and expiratory

udible itout stetoscopesilent cest it minimal air entry

xyen saturation



–



Score

Asthma Seerity

0–3 – –1

ild oderate evere

rom Chalut S ucharme  avis . he reschool espiratory ssessment easure  a responsive index o acute asthma severity. J Pediatr. ;–.

. Management a. Assess ACs and place on monitors (cardiac monitor and   sat monitor) b.  to maintain saturation 9% c. xclude other causes of heeing based on histor and phsical exami nation (anaphlaxis foreign bod aspiration pulmonar infection etc.) d. PAM score can help guide management (igure .) e. Medications i. nitial treatment involves administration of inhaled bronchodilators such as shortacting b2agonists (salbutamol) 6 anticholiner gics (ipratropium bromide) as ell as systemic corticosteroids ii. or insu¬cient response despite maximiing above therap contin os nelied b2agonist intravenous magnesim slfate intraenos b2agonist infsion or helio ma be needed f. Noninasie entilation (ileel positie airway pressre iPAPcontinos positie airway pressre PAP) ma be necessar for tiring patients ith impending respirator failure. ntubation should be avoided if possible and reserved for cases of impending respirator arrest. etamine (bronchodilator) is the preferred agent if sedation is needed. g. s are not rotinely indicated in cases of acute asthma exacerbations

Emergency Medicine

Signs

2

3

Figure 22 nitial Treatment Algorithm or Acute Asthma Eaceration Initial assessment - including history and physical examination (auscultation, accessory muscle use, heart rate, respiratory rate, oxygen saturation) Calculate PRAM score and determine severity of exacerbation

Emergency Medicine

Mild exacerbation (PRAM 0–3) • Keep O2 saturations ≥94% • alutamol 4– pus∞ ia Ispacer 2min × – doses • onsider oral corticosteroids

Moderate exacerbation (PRAM 4–7) • Keep O2 saturations ≥94% • Salbutamol 4–8 puffs∞ via MDI+spacer 2min ×  oses • Oral corticosterois • onsier ipratropium 4 puffs via MDI+spacer 2min ×  oses

Severe exacerbation (PRAMS –) • Keep O2 saturations ≥94% • Salbutamol 4–8 puffs an ipratropium 4 puffs∞ via MDI+ spacer 2min eac ×  oses • Oral sterois or consier I metlprenisolone • onsier I manesium sulfate • onsier continuous aerosolie β2 aonists • Keep patient O

Severe to impending respiratory faire • Keep O2 saturations ≥94% nonrebreater mas it % oen • ontinuous salbutamol nebuliation an nebulie ipratropiumφ ×  oses • I metlprenisolone • I manesium sulfate • onsier epineprine S  m of  concentration • I beta aonists if neee • i as neee • Keep O ensure I access an continuous monitorin

eassess patient an recateorie o severe is te astma eacerbation after initial treatment  

2

Mid exacerbation? Observe in merenc Department D for 2  If no furter treatment reuire iscare ome to continue inale corticosterois an folloup appointment it psician

Moderate exacerbation? • Keep O2 sats ≥94% • Salbutamol  • eassess in 2 

Moderate/Severe exacerbation? • Keep O2 sats ≥94% • Salbutamol 2min until able to tolerate  • eassess freuentl

Severe to impending respiratory faire • SAM AS ARR • If eterioratin consier rapi seuence intubation A P PSA

eassess patient an recateorie o severe is te astma eacerbation after 2  of treatment

Mid exacerbation? Observe in D for 2  If no furter treatment reuire iscare ome to continue inale corticosterois an folloup appointment it psician

Moderate exacerbation? • Keep O2 sats ≥94% • Salbutamol  • eassess If patient nees salbutamol more often tan 4 AM  SPA

Moderate/Severe exacerbation? • Keep O2 sats ≥94% • Salbutamol 2min until able to tolerate  • eassess freuentl AM  SPA

Severe to impending respiratory faire • SAM AS ARR • If eterioratin consier rapi seuence intubation A P PSA

∞ ilren 2  soul alas receive 8 puffsose

Oral sterois prenisone –2 ma  as or eametasone  m O ail  –2 as Inalational salbutamol  mm 2   m 2 m per ose mie it  m S 2   m  m per ose mie it  m S φ Inalational Ipatropium bromie 2 mm  m 2 m mie it  m S

iA ilevel positive aira pressure IV intravenous MDI metereddose inhaler O nothing b mouth S normal saline I pediatric intensive care unit RAM pediatric

40

respirator assessment measure. (odied from rtiAlvare  ikrogianakis A. Canadian aediatric ociet. aediatr hild ealth 0()–.)

. omplications a. otential complications include pneumothorax pneumomediastinum hpoxia cardiac arrest PEAR

FORE O ASPRATO ee igure . Figure 2 anagement o Suspecte Foreign oy Aspiration istor o possile orein od aspiration

Emergency Medicine

etered-dose inaler (DI) it a mas and spacer is te preerred ay to administer broncodilators ebulier sould only be used in cases o impendin respiratory ailure

eep patient  Obtain inspiratory and expiratory CXRs*

XRs Equivocal (i.e., minimal atelectasis or consolidation)

XRs ormal

Low clinical suspicion: Followup within 2 das, consider repeat XRs i an smptoms

tron histor o aspiration, smptomatic, or ocal indins on eam

uestive histor o F aspiration or ocal indins on eam

XRs suestive o F (i.e., air trappin, consolidation, or atelectasis)

2

 histor poor or aspiration and no ocal indins on eam, treat or alternate cause, and ollow-up as indicated

Bronchoscopy or orein od identiicationremoval (can consider  chest irst to clari dianosis in select cases)  and lateral decuitus chest -ras (aected side placed down) ma sustitute in ouner or uncooperative patients Focal indins include ocal wheee, decreased reath sounds

 Computed tomograph R chest xra  foreign bod A foreign bod aspiration O nothing b mouth. (Adapted from leisher  udig  eds. etoo of ediatric merency Medicine. th ed. hiladelphia A ippincott illiams  ilkins 0.)

CROP ACTERA TRACHETS A EPOTTTS efer to able . in Chapter  tolarngolog

1

ANAPHYLAXIS

Emergency Medicine 2

. enition a. lassic anaphylactic reaction lifethreatening immunoglobulin gmediated tpe  hpersensitivit reaction hich occurs upon reexposure to an antigen . Etiology a. ost commonl caused b food allergens (peanuts tree nuts shellsh dair products). ther common triggers include menoptera stings (aspsbees) drugs (antibiotics) immunotherap radiocontrast media and blood products. ¡e causative agent is unknon in some cases. . linical manifestations and diagnosis a. nset of smptoms can var from minutes to hours folloing exposure b. Anaphylais shold e diagnosed when two or more of the following for systems are aected i. Skinmcos memrane urticaria pruritis ¢ushing angioedema uvula selling ii. espiratory (upper or loer) stridor voice changehoarseness larngeal edema throat itching bronchospasm heeing coughing chest tightness shortness of breath iii. astrointestinal nausea vomiting abdominal cramping diarrhea iv. irclation tachcardia hpotension sncope arrhthmias cardiac arrest c. ther common smptoms include anxiet diiness and sense of impending doom d. p to onethird of patients have biphasic reaction (smptoms recur in – hours) . Management a. ee igure . for treatment algorithm b. ischarge planning i. Consider discharge once stable for  to  hours aer  epinephrine ithout recurrence of smptoms ii. Anaphlaxis education (including edicAlert bracelet) iii. pien teaching and prescription iv. olloup ith an allergist for further testing if not alread done PEAR Outpatient pediatric EpiPen prescriptions: pien r (01 m) or cildren 10–  pien (03 m) or cildren . 

42

Figure 2 anagement Algorithm or Anaphylais Signs and symptoms fitting clinical criteria for anaphylaxis pinephrine   ggdose  thigh –in R ( gdose) in 0 mgdose 0 mLdose ax 0 mgdose 0 mLdose mminent cardiac arrest at any point?

emove trigger if nown ssess s and L ttach monitors ive 00 oxygen to eep Sa2 ≥  stablish  access lace patient spine with legs elevated or on side if vomiting or decreased L

pinephrine  00 mggdose 0 mLgdose max 0 mLdose Signs of airway compromise or respiratory failre?

Circulation Signs of hemodynamic instability?

repare for apid Seence ntbation

Emergency Medicine

• • • • • •

ive 0NS 20 mLg bols  epeat as needed for hypotension Airway and Breathing Signs of pper airway involvement: pinephrine  B 3% difference between right hand and foot is BORDERLINE

≥9% in right hand or foot ND ≤3% difference between right hand and foot is NORL

8

Reeat screen in  hor

90% in right hand or foot is BNORL

FAILED creen LL the ost resonsibe heath care roider

90%–94% in right hand and foot OR >3% difference between right hand and foot is BORDERLINE

≥9% in right hand or foot ND ≤3% difference between right hand and foot is NORL

PASSED creen ontine with NORL newborn care

Data fro eper A, ahle , artin , et al Strategies for ipleenting screening for critical congenital heart disease Pediatrics  e

CARIAC CREEI BERE E  TIMAT MEICATI  ittle evidence that stiulant edications increase the ris of sudden death in pediatric patients with attention-decit hyperactivity disorder ADD

211

Cardiology 8

 Children and adolescents with ADD should undergo a careful history and physical eaination by their priary care physician to identify those at increased ris of sudden death  outine C assessent not recoended  or patients with nown cardiac disease followed by a cardiologist, the physician with epertise in ADD is the appropriate person to recoend edication  Discussion of treatent options with the cardiologist to for a consensus decision is appropriate  or patients with ADD and suspected cardiac disease or ris factors for sudden cardiac death, assessent by a cardiologist is recoended as with any non-ADD patient IPI CREEI  See able  for screening recoendations  See able  for interpretation of results f abnoral, refer to lipid specialist and dietitian Tale 821

iid creening Recoendations

irt–2 years

o liid screening

2– years

Targeted liid screening (o 83)

–11 years

niersal liid screening (o 8)

12–16 years

Targeted liid screening (see o 83)

17–21 years

niersal liid screening (see o 8)

ata rom xpert anel on Interated uidelines or Cardiovascular Health and isk eduction in Children and Adolescents: ummar eport. ediatrics.  s:s.

Tale 822

aorator Paraeters for iid creening Reslts Accetale (ol)

Borderline (ol)

Anoral (ol)

Total colesterol

,

–51

52



,285

285–33

335



.115

1–115

,1

Triglycerides (,1 years o age)

,85

85–11

115

,1

1–1

15

,31

31–37

375

aorator Paraeter

Triglycerides (1–1 years o age) on

HDL, Hihdensit lipoprotein LDL, lodensit lipoprotein.

212

ata rom xpert anel on Interated uidelines or Cardiovascular Health and isk eduction in Children and Adolescents: ummar eport. ediatrics.  s:s.

Box 8.

Targeted Lipid Screening

easure asting liid role tice (2–12 ees aart) and aerage te results i 1 arent grandarent auntuncle or siling it I angina stroe AGstentangiolasty at ,55 years in males and ,65 years in emales 2 arent it total colesterol 62 mmol or non dysliidemia 3 ild as diaetes yertension I 5t ercentile (2– years)85t ercentile (12–16 years) or smoes toacco roducts  ild as a moderate or igris condition (see Tale 823)

Box 8.

Universal Lipid Screening (Nonfasting or Fasting)

onasting liid screen calculate non colesterol 1 on colesterol 5 Total colesterol –  2 I non 375 or  ,15 mmol ten otain asting liid role tice (2–12 ees aart) and aerage te results asting liid screen 1 eeat asting liid role in 2–12 ees and aerage te results i any o a  335 mmol  on 375 mmol c  ,15 mmol d Triglycerides 115 mmol (# years) or 15 mmol (1 years)

Cardiology

BI, od mass index CABG, coronar arter bpass rat I, mocardial inarction. ata rom xpert anel on Interated uidelines or Cardiovascular Health and isk eduction in Children and Adolescents: ummar eport. ediatrics.  s:s.

8

HDL, Hihdensit lipoprotein LDL, lodensit lipoprotein. ata rom xpert anel on Interated uidelines or Cardiovascular Health and isk eduction in Children and Adolescents: ummar eport. ediatrics.  s:s.

Tale 823

Moderate or HighRis Conditions for iid creening

ModerateRis Conditions

HighRis Conditions

aasai disease it regressed coronary aneurysms

ronic idney diseaseendstage renal disease ostrenal translant

ronic inammatory disease (S A)

T1 and T2

I inection

ostortotoic eart translant

erotic syndrome

aasai disease it current aneurysms

HIV, Human immunodecienc sndrome JRA, uvenile rheumatoid arthritis SLE, sstemic lupus erthematosus TD, tpe  diabetes mellitus TD, tpe  diabetes mellitus. ata rom xpert anel on Interated uidelines or Cardiovascular Health and isk eduction in Children and Adolescents: ummar eport. ediatrics.  s:s.

213

PREPARTICIPATI CREEI I ATHETE  Aerican eart Association AA a Screening for high school and college athletes preparticipation and at  to  year intervals b Cardiac-focused history and physical eaination  uropean Society of Cardiology SC a outine preparticipation screening of athletes b Cardiac-focused history, physical eaination and resting -lead C Cardiology

ANTIARRYTHMIC MEDICATIONS See able  for coon antiarrythic agents Tale 82

Cardiac Antiarrthic Agents

Medication Class

Exales

ide Effects

Class I—a Channel Blocade Ia

 uinidine  rocainamide

lood dyscrasias cinconism (uinidine) deressed myocardial contractility druginduced luus (rocainamide) nausea and omiting rolonged S comle entricular arrytmias

I

 idocaine  enytoin  eiletine

Aniety drosiness yotension resiratory deression seiure soc

Ic

 lecainide

radycardia A loc diiness lurred ision dysnea nausea eadace increased  and S duration

8

Class II—Beta Blocade    

adolol roranolol smolol Atenolol

roncosasm  yotension nausea and omiting atigue deression in adolescents

Class III— Channel Blocade  Amiodarone  Sotalol

Amiodarone ataia corneal microdeosits eart loc eatotoicity yotension nausea and omiting otosensitiity ulmonary rosis tyroid dysunction Sotalol radycardia roncosasm cest ain deression diiness eart loc yoglycemia yotension T rolongation torsades de ointes

21

Tale 82

Cardiac Antiarrthic Agents—contd

Medication Class

Exales

ide Effects

Class I—Ca Channel Blocade  eraamil  iltiaem

Arrytmias diiness edema eadace nausea and omiting ontraindicated in second and tirddegree eart loc sinus node dysunction

 Adenosine  igoin  agnesium sulate AV, Atrioventricular CH, conestive heart ailure.

Cardiology

Class —ther Mechanis

HEART TRANSPLANTATION See Chapter  ransplantation

8

215

CHAPTER

9

Child Maltreatment Tanvi Agarwal • Rebecca Wang • Elodie April • Romy Cho • Jennifer Smith

Common abbreviations Reporting and documentation Red ags Physical abuse Sexual abuse Neglect and caregiver fabricated illness

216

COMMON ABBREVIATIONS Also see page xviii for a list of other abbreviations used throughout this book NAAT STI

nucleic-acid amplication test sexually transmitted infection

A. Reporting 1 If you suspect child abuse or neglect is occurring you have met the legal threshold to report to the appropriate child elfare agency  eports should be directly made by the concerned healthcare provider in most cases families should be informed about the report being made to child elfare agency  hild elfare agencies ill determine the need for police involvement  ealthcare professionals have an ongoing duty to report any ne concerns B. Documentation 1 edical records can be used for legal purposes document history and physical examination carefully and obectively rite legibly include times and dates  Include relevant statements in uotes draings measurements body diagrams igure 1 photographs consent reuired

Child Maltreatment

REPORTING AND DOCUMENTATION

9

Figure 9.1 Body Diagram

R

L

R

L

R

L

Name of patient:– Name of doctor:– Date:–

rom e aculty of orensic  egal edicine of the oyal ollege of hysicians Source httpsmacukpublicationspro-forma-body-diagrams

217

RED FLAGS 1 Inury not consistent ith history provided andor agedevelopmental stage of child  Inconsistentchanging history for a signicant inury  elay in seeking medical attention  ultiple inuries or inuries of dierent ages  vidence of neglect or failure to thrive  Absence of a history of trauma in a child ith obvious inury Child Maltreatment 9

PHYSICAL ABUSE Any act that causes inury or trauma to a child through bodily contact A. History 1 Sentinel injuries: relatively minor suspicious inuries eg frenulum tears or bruises in precruising infants that may identify those ho are at risk for suering more serious abusive inuries  Injury: detailed history of seuence of events leading up to inury nonleading open-ended uestions event details timing location response of childcaregiver itnessedunitnessed symptom progression hen the child last fedacted normally  ast medical history: previous traumainury chronic illnesses birth history administration of vitamin  at birth bleeding history andor predisposition to fractures  Deelopment: ambulation level of functioning ability to express pain  amily history: family members ith easy bleeding bruising fractures bleeding disorder collagen vascular diseases bone disorders metabolic or genetic disorders consanguinity  Social: caregivers discipline practices substance or alcohol abuse mental illness domestic violence stressors prior criminal or child protection agency involvement other children in the home ho may need to be assessed for maltreatment

B. hysical eamination A thorough examination is necessary to respond to life-threatening inuries consider the dierential diagnoses assess for other inuries including sentinel inuries onsider conducting the examination folloing consultation ith a clinician ho has expertise in the evaluation of suspected child maltreatment 1 Anthropometrics: groth parameters eight height head circumference and percentiles  Sin: undress and examine entire body including pinnae behind ears soles and palms genitals and buttocks escribe location distribution color shape sie of each skin nding 218  : frenula beteen lips and gums under tongue oral inuries dentition

 eurologic: level of consciousness fontanelle fundi  MS: skull fractures scalp selling bony tenderness limb selling

Child Maltreatment

C. Injury patterns 1 Bruising a annot be dated b eatures concerning or inicted injury: preambulatory infant located on areas that are padded by so tissue or less exposed eg buttocks neck genitalia ears chest back abdomen clustered or patterned eg looped or parallel linear bruises large or numerous causal mechanism does not correlate c Dierential: birthmarks congenital dermal melanocytosis café-au-lait macules cultural practices coining or cupping bleeding disorders  Bites a haracteristic pattern involves to opposing convex arcs of bruising or change in pigmentation may or may not have a central bruise b Concerning eatures: multiple bites no explanation for bites bites on inaccessible areas self-inicted are oen on the hands  Burns a Inury can be aected by multiple factors such as temperature mechanism duration of exposure presence of clothing rst aid 9 applied see urn anagement in hapter  lastic Surgery b Concerning eatures: burns in nonmobile infants clear delineation beteen burned and healthy skin eg immersion patterned burns eg clothing irons radiators burns on buttocksperineum glovestocking distribution Absence of splash marks does not provide additional information about mechanism of scald burns c Dierential: skin infections eg staphylococcus scalded skin syndrome dermatitis herpetiformis varicella impetigo drug eruptions insect bites photosensitive dermatitis allergicirritant contact burn chilblains or sunburn cultural practices eg moxibustion chemical burns from home remedies senna typically in diapered children  ractures a Concerning eatures: fractures in nonambulatory children multiple fractures dierent stages of healing delay in seeking treatment fracture does not t ith described mechanism specic locations—ribs especially posterior metaphyseal vertebral spinous process scapula sternum i Ri ractures: typical mechanism is anterior–posterior compression of rib cage ith forceful sueeing or shaking can also include direct 219 impact to chest igure 

Figure 9.2 Mechanism of Ri Fracures in Chid Mareamen

Child Maltreatment

Front

ii Metaphyseal ractures classic metaphyseal lesion corner fracture bucket-handle fracture caused by shearing of ne bone formation from ends of long bones typical mechanism is tractionaltorsional force eg yank pull tist applied to the limb igure  b Dierential osteopenia osteogenesis imperfecta metabolic and nutritional disorders eg vitamin  deciency rickets enkes disease copper deciency osteomyelitis neoplasia

9 Figure 9. Mechanism of Meahysea Fracures

220

Child Maltreatment

 raumatic head injury a Description: subdural hemorrhages may be caused by direct impact to head andor head acceleration-deceleration eg forceful shaking Symptoms range from nonspecic fussinesscrying to persistent vomiting to life-threatening symptoms eg coma seiures respiratory arrest b ther ndings retinal hemorrhages especially extensive and multilayered complex skull fractures other cutaneousskeletal visceral inuries c Dierential bleeding disorder birth trauma infection metabolic conditions glutaric aciduria type 1 tumor arteriovenous malformations  Adominal injury a eatures: solid organ such as liver pancreas and spleen hollo organs duodenal perforation transection hematoma

D. Inestigations etermine investigations based on patient age history physical examination dierential diagnoses xclude the presence of other traumatic inuries hich may be occult 1 aoratory a Bleedingruising: complete blood count  international 9 normalied ratio INpartial thromboplastin time TT peripheral blood smear brinogen on illebrand antigen and activity blood group factor III factor I liver enymes and renal function tests b ractures: bone health calcium magnesium phosphate alkaline phosphatase A parathyroid hormone T vitamin  c Sudural hemorrhagesretinal hemorrhages: coagulation orkup see bleedingbruising orkup actor III metabolic orkup urine organic acids plasma amino acids plasma acylcarnitines d Adominal trauma: liver enymes alanine transaminase AT andor aspartate transaminase AST .  concerning for occult inury pancreatic enymes lipase and amylase e Altered mental status: urine toxicology for overdose if relevant  Radiologic a Seletal surey see hapter 1 iagnostic Imaging children , years old index case and siblings and in children . years old based on clinical suspicion and developmental level epeat skeletal survey aer 1days to assess for healing fractures b euroimaging: head T andor I for facial inuries or concern of head inury Screening head T andor I for all children ,1 year of age head ultrasound is not su¬cient to assess for 221 traumatic brain inury

c Adominal imaging: T if abnormally elevated ASTAT pancreatic enymes or abdominal bruising abdominal ultrasound not su¬cient  phthalmologic dilated fundoscopic examination for retinal hemorrhages if subdural hemorrhages on neuroimaging should be completed by ophthalmologist

Child Maltreatment

. Management 1 Stabilie patient manage acute inuries and consult experts eg child maltreatment clinician hematology neurosurgery orthopedics  bectively document clinical information and report case to local child elfare agency  onsider hospitaliation for additional medical testing subspecialty consultation treatment of knon inuries at reuest from local child elfare agency

SEXUAL ABUSE

9

A. Approach 1 Sexual abuse is any sexual behavior or action that is unanted or exploitative  Sexual abuse does not have to involve direct touching or contact Shoing pornography to a child photographinglming a child in sexually explicit poses or encouraging a child to perform sex acts also constitutes as sexual abuse  See Age of onsent in hapter  Adolescent edicine  Sexualied behaviors that raise concern for possible sexual abuse a evelopmentally inappropriate knoledge of sexual activities b evelopmentally inappropriate playsexual acts c Sexual behavior that results in emotional distress or physical pain d Sexual behaviors associated ith other physically aggressive behavior e Sexual behaviors that involve coercion

B. History o not ask leading uestions avoid asking the child directly and avoid repeated uestioning 1 Injury: hat happened hen did it happen  enitourinary  symptoms: vaginal or anal symptoms bleeding pain discharge  ast medical history: medical infection ecema gynecologic vulvovaginitis STI foreign body  sychological: current level of distress suicidality 222  Social: living situation prior child elfare agency involvement

C. hysical eamination 1 Acute assault or painleedingdischarge urgent evaluation by a sexual assault specialist examiner  onacute asymptomatic cases in consultation ith sexual assault specialist examiner can likely defer examination until intervie has been completed by child elfare agency  imit unnecessary or multiple examinations ost genital examination ndings are normal or nonspecic this does not conrm or rule out the possibility of sexual abuseassault  eneral: other inuries eg skin inuries sexual maturity rating  : perform in the most comfortable manner Supine frog leg prone knee-chest or lithotomy positions to assess external genital hymen conguration rim edges inuries anal examination No role for speculum examination a edness in the anogenital region is not diagnostic for sexual abuse ther causes of anogenital redness include vulvovaginitis streptococcus infections yeast infections b If anogenital arts present obtain detailed history including family members or caregivers ith arts maternal ap smear results maternal history of genital arts Absence of maternal genital arts on history does not exclude the possibility of perinatal transmission onsider vertical transmission if child is , years of age c onsult sexual assault specialist examiner for interpretation of genital ndings

Child Maltreatment

 Special consideration: forensic intervie should be completed by police and child elfare agency

9

D. Inestigations aboratory tests and STI screening are based on the sexual abuseassault disclosed pubertal development of the child perpetrator risk factors and parentpatient concern or reuest for testing onsult sexual assault specialist examiner to assist ith these recommendations 1 Seual assault eidence it reuires patient consent and police involvement should be done in consultation ith a sexual assault specialist examiner  aoratory: serologies human immunodeciency virus I hepatitis  hepatitis  syphilis pregnancy testing  SI: consider sabs for STIs gonorrhea chlamydia trichomonas vaginal sabs technically di¬cult in symptomatic prepubertal females a onorrheachlamydia urine nucleic acid amplication test NAAT vaginalvestibuledischarge girls and meatus boys sabs for NAAT 223 andor culture pharynxrectum sabs for NAAT andor culture

b Trichomonas vaginalis vaginalvestibuledischarge girls or meatus boys culture c erpes simplex virus S viral lesion sab if ulcers present

Child Maltreatment

. Management 1 Report to child elare agency: if the child is under the age of 1years and any of the folloing applies a Suspected sexual abuse by a person in position of authority eg teacher employer  b If assault is by a stranger and parents are not believing or are unsupportive of disclosure c ngoing concerns of child safety  harmacological agents: consider STI prophylaxis risk of exposure based on history and ability to follo-up particularly in adolescents emergency contraception hepatitis  immuniation and immunoglobulin I prophylaxis for high-risk cases reuires expert consultation ith sexual assault specialist examiner andor infectious disease specialist

NEGLECT AND CAREGIVER FABRICATED ILLNESS 9

A. eglect 1 Denition omission in care that results in actual or potential harm a hysical: failure to provide necessities eg shelter food abandonment or inadeuate supervision repeated accidentstoxicities b motional: lack of nurturing or aection c ducational: child not involved in any educational programs d Medical: failure to seek timely care of illnessinury failure to comply ith medical recommendations  History evidence of harm eg inury ingestion indications that basic needs have not been adeuately met eg poor hygiene poor groth nature and pattern of neglect risk factors for neglect eg parental depression substance abuse violence  Assessment complete physical examination including groth mental status  Management target interventions at apparent medical concerns may need hospitaliation address risk factors report to local child elfare agency

B. Caregieraricated illness 1 Denition: hen a parent or adult simulates or causes disease in a child and falsely presents a child for medical attention sometimes called medical child abuse Munchausen syndrome by proxy 224 factitious disorder by proxy

Child Maltreatment

 Clinical maniestations presentation varies in nature and severity xamples include bleeding adding dyes to samples seiures fabricated history toxin induced apnea partial suocation toxin induced gastrointestinal symptoms forced ingestion of substances laxative use recurrent sepsis contaminating intravenous lines blood urine samples  ossile eatures suspect if signssymptoms occur only in the presence of a single caregiver failure of illness to respond to normal treatments caregiver not relieved if child improves andor caregiver insists on invasivepainful procedures and hospitaliations  Approach: revie all the child’s medical records look for discrepancies beteen obective and described ndings lose observation and detailed clinical documentation of events is essential  Management: ork collaboratively ith providers ith expertise in child maltreatment for treatment plan

9

225

CHAPTER

10

Dentistry Rodd Morgan • Jane Ho • Shonna Masse

Childhood dental care and caries Teething, natal/neonatal teeth, and early tooth loss Dental trauma Odontogenic infections/dental abscess Common dental problems Conditions associated with dental anomalies and periodontal disease Dental care of medically compromised patients

226

CHILDHOOD DENTAL CARE AND CARIES

B. Early childhood caries 1 . Diagnosis: may appear as chalky hite spots through to bronblack holes in enamel. arents may not be aare of cavities until teeth discolor or enamel “chips.” 2 . Risk factors: bottle feeding ith any liuid other than ater hile being put to sleep using bottle as pacier not brushing aer feeding breastfeeding on demand and special healthcare needs. Children on longterm medications containing sucrose or high caloric dietary supplements are at elevated risk. ugarfree formulations should be used hen possible. 3 . Prevention: dental care outlined earlier. Cessation of a bottle and use of a cup should be encouraged by age one.

Dentistry

A. Recommendations for dental care 1 . Children should have dental home and rst dental visit by age one 2 . Encourage parents to li the lip to check for tooth decay 3 . Wipe gums ith damp ashcloth until rst tooth erupts  . Children ,3 years should have their teeth brushed tice daily starting at rst tooth eruption. luoride toothpaste smear no larger than a grain of rice should be used if child is at risk of caries.  . Children aged 3 to  years should have adult assistance and use adult uoridated toothpaste  . onnutritive sucking habits should be discouraged as young as possible

10

PEARL A child’s rst dental check-up should occur by 1 year of age

TEETHING, NATAL/NEONATAL TEETH, AND EARLY TOOTH LOSS A. Teething 1 . Clinical featres  a. Children have to sets of teeth primary and permanent  b. eeth tend to follo a pattern of eruption although variance in timing is common. e earliest eruption is typically around  months igure 1.1 and able 1.1.  c. ome children may present ith drooling irritability and discomfort in the area of an erupting primary tooth 2 . anagement  a. Chilled teething rings and oral analgesics may help alleviate discomfort  b. void teething gels and topical anaesthetics because of risk of 227 methemoglobinemia aspiration and overdose

Figure 10.1 Identication System for Primary and Permanent Teet Permanent Teeth 10

Right

Left

11 12 13

Permanent maxillary 1 left seon premolar 1

Permanent maxillary right first molar Dentistry

1

1

Open mouth view

Permanent maniular 32 right thir molar 31

1 1 19

30

20

29 2 10

2

2 2 2 23

Primary Teeth    Primary maxillary right first molar 



21 22

Permanent maniular left anine

  

A



T

 

R  Primary maniular P right lateral inisor

Primary maxillary left seon molar

L  O



Primary maniular left anine

odied from enko ip . Emergency dental procedures. n Roberts and Hedges’ Clinical Procedures in Emergency Medicine and Acute Care. th ed. hiladelphia  Elsevier 21.

228

Cronoogy of Human entition

Teet

Erution (Exfoliation)

Primary Teet

aiary

andiuar

Central incisors ateral incisors Canines irst olars econd olars

6–1 onths 7–8 years  –12 onths 8–9 years  16–2 onths 11–12 years  11–1 onths 9–11 years  2– onths 9–12 years 

– onths 6–7 years  7–1 onths 7–8 years  16–2 onths 9–11 years  11–1 onths 10–12 years  2– onths 11–13 years 

Permanent Teet

aiary

andiuar

Central incisors ateral incisors Canines irst preolars econd preolars irst olars econd olars hird olars

7– years –9 years 11–12 years 1–11 years 1–12 years –7 years 12–1 years 17– years

6–7 years 7– years 9–11 years 1–12 years 11–1 years –7 years 12–1 years 17– years

Dentistry

able 11

Modied from AAPD Dental Growth and Development Resource 2018.

B. atalneonatal teeth 1 . Clinical featres  a. ay be present at birth natal or ithin 3 days postpartum neonatal and are most commonly in the region of mandibular incisors. ost are prematurely erupted primary incisors. e eact etiology is unknon.  b. igaede disease is a lesion on the ventral tongue caused by abra sion from neonatal teeth. ay be associated ith dehydration and insucient nutrient intake. 2 . anagement: natal teeth may arrant etraction if they interfere ith feeding present an aspiration risk or cause igaede disease.  dental consult is recommended.

10

C. Early tooth loss 1 . rimary teeth may be lost efoliate early because of advanced dental development of underlying permanent successors or less commonly because of underlying systemic causes such as immunological defects.  dental consult is recommended.

DENTAL TRAUMA 1 . Clinical featres  a. ee able 1.2 for description of common inuries

229



b. ultiple teeth and oral structures mucosa gingiva bone may be inured concurrently 2 . anagement  a. lease see able 1.2 for indications for dental referral. rauma management may include tooth replantation splinting etractions suturing root canal therapy and restorations.  b. Children may reuire advanced behavioral management including sedation and support of other services such as child life specialists able 12

escrition and anagement of enta Trauma

Dentistry

Tye of Inury

escrition

anagement of enta Trauma

Cron fractures

o pulpal eposure enael and dentine only

eranent and priary teeth referral to dentist ithin 2 h

ith pulpal eposure enael dentine pulp

eranent and priary teeth iediate referral to dentist

Concussion inury to tooth supporting structures  abnoral looseningdisplaceent

eranent and priary teeth referral to dentist ithin 2 h

ubluation inury to tooth supporting structures abnoral loosening but  displaceent

eranent teeth iediate referral to dentist

ondisplaceent inuries

10

riary teeth in signicantly loose iediate referral if inially loose referral ithin 2 h isplaceent inuries

Aulsion

trusion partial displaceent of tooth fro socket

eranent and priary teeth iediate referral to dentist

uation displaceent of tooth in direction other than aially

eranent and priary teeth iediate referral to dentist

ntrusion displaceent of tooth apically into aleolar bone

eranent and priary teeth iediate referral to dentist

Coplete displaceent of tooth fro socket

eranent teeth iediate replantation ithin  in if not store and transport in chilled ilk iediate referral to dentist riary teeth iediate referral if caregiers cannot nd tooth rule out intrusion aulsed tooth should not be replanted infection and ankylosis risk

PITFALL 230

ailure to reect the lips to assess the soft tissue surrounding teeth and the sulcus ay result in issed oral soft tissue inuries these ay be ore etensie than they appear

1 . Etiology: infection in the teeth and supporting structures may originate from a chronic or acute source typically from a tooth ith either decay a dental anomaly trauma or failed dental treatment. 2 . Clinical featres: pain selling erythema and suppuration localied to tooth 3 . anagement  a. ntibiotics  i. ral antibiotics for localied selling amoicillinclavulanate is typically the antibiotic of choice. Clindamycin is an alternative for children ith penicillin allergy.  ii.  antibiotics Clindamycin in cases of failed oral antibiotics rapid progression or signs of complications see later  b. rgent referral to dentist most cases ill reuire etraction pulp therapy root canal andor incision and drainage  . Comlications: untreated dental abscesses can spread to local so tissues facial cellulitis udig’s angina bone a osteomyelitis or spread hematogenously sepsis meningitis. Early management  antibiotics and dental evaluation is essential because of potential for rapid systemic involvement. PEARL

Dentistry

ODONTOGENIC INFECTIONS/DENTAL ABSCESS

10

A spreading odontogenic infection can lead to facial cellulitis hich is a dental eergency

COMMON DENTAL PROBLEMS A. o tisse lesions of the oral cavity 1 . Ertion cysthematoma: so tissue cyst oen found in the posterior mandible or here a tooth is about to erupt. Color varies from coral pink to blueblack depending on etent of blood ithin cyst. elf resolving lesion reuires no treatment unless infected. 2 . cocele: small bluishtranslucent hite if longstanding selling caused by etravasation of mucous from a severed minor salivary gland. sually midline of the loer lip. ometimes on inner cheek surface ventral tongue and oor of mouth. ay spontaneously resolve. Ecision of nonresolving lesions and associated gland. 3 . Ranla: painless selling that occurs as a result of etravasation of mucous from a severed sublingual gland. arger bluishtranslucent selling on the oor of the mouth lateral to the tongue. Consult for ecision or marsupialiation. B. lcers 1 . inor recrrent ahthos lcers i.e. canker sores: cause not clear may be genetic or related to stress trauma hematinic deciency

21

Dentistry

12 folate iron. mall ulcers ith a red halo and yellogrey oor. elflimiting lesions that do not scar. anagement is symptomatic topical anesthetic mouthash e.g. lidocaine may reduce discomfort. 2 . Acte heretic gingivostomatitis 1: selflimited manifesta tion of primary herpes simple virus  infection lasting  to 1 days. Characteried by gingivitis inamed friable gums and stomatitis oral and lip ulcers. esicles rupture leaving painful pseudomembranous lesion ith adacent erythema. atients typically demonstrate malaise cervical lymphadenopathy headache and fever during prodromal phase. anagement is symptomatic so diet good oral hygiene ensure ade uate hydration and analgesia acetaminophen ibuprofen morphine in severe cases. ntibiotics contraindicated. Consider antivirals in immu nocompromised patients.

CONDITIONS ASSOCIATED WITH DENTAL ANOMALIES AND PERIODONTAL DISEASE

10

umerous syndromes have associated dental and or craniofacial anomalies. nherited dental anomalies may be epressed in an ecess or reduced number of teeth sie or shape of teeth or ualitative defects of the formation of tooth structure. everal systemic conditions are also associated ith periodontal disease able 1.3. able 1

Systemic Conditions it anifestation of Periodonta isease

Systemic Condition

entaPeriodonta Features

ypophosphatasia

reature loss of priary teeth

hler-anlos syndroe

reature loss of priary teeth gingial recession periodontal disease

eutropenia

iery red gingia periodontal disease increased susceptibility to oral ulcerations and infections

eukeia

Acute oral infections spontaneous gingial bleeding oral ucosal petechiae periodontal disease

eukocyte adhesion deciency syndroe

reature loss of priary teeth periodontal disease linear gingial erythea recurrent oral infections oral ulcerations

Chediak-igashi syndroe

eere gingial inaation periodontal disease oral ulceration increased tooth obility

apillon-efère syndroe

reature loss of priary teeth periodontal disease

DENTAL CARE OF MEDICALLY COMPROMISED PATIENTS  riskbenet analysis should be performed before performing elective 232 dental care on patients ho are medically compromised

B. Cardiac atients 1 . Consultation and denitive dental management caries and periodontal should be performed before elective cardiac surgery 2 . Considerations hen planning care include hemodynamic stability anticoagulants and specic anesthesia reuirements including the availability of the etracorporeal membrane oygenation EC team 3 . Cardiac dental patients may or may not reuire antibiotic prophylais before dental procedures and need is dependent on cardiology guidance and endocarditis guidelines see Chapter  Cardiology

Dentistry

A. mmnocomromised atients 1 . deally dental assessment and management should be performed before initiation of oncology care immunosuppression or organ transplanta tion to eliminate potentially lifethreatening infection sources 2 . Ecellent oral hygiene is reuired as is regular dental folloup. ral hygiene should be maintained and monitored by parents. 3 . ecause of epediency of oncology management nonemergent dental care may need to be delayed until cell counts are adeuate for it to be performed  . or patients ith mucositis hen use of a so toothbrush is not possible because of bleeding a sodium bicarbonate rinse may be used as a rinse or on damp clean ashcloth  . oam sabs are inferior to toothbrushes and should only be used hen toothbrush is contraindicated  . ines of communication should be kept open beteen dentistry and the physician managing the child regarding precautions and health changes

10

USEFUL WEBSITES merican cademy of ediatric entistry http.aapd.orgpolicies Canadian ental ssociation http.cdcadc.ca

2

CHAPTER

11

Dermatology Laura Morrissey • Kimberly Tantuco • Rebecca Levy

Common abbreviations Morphology Neonatal/infantile eruptions Neonatal/infantile vascular lesions Dermatitic eruptions Papulosquamous eruptions Acne vulgaris Cutaneous bacterial infections Cutaneous viral infections Childhood exanthems Fungal eruptions nfestations Alopecia Acute blistering reactions enodermatoses genetic sin conditions pidermolysis bullosa  opical steroids

234

COMMON ABBREVIATIONS AD BSA CM CNI CS AS S  C SSN SSSS ung 

atopic dermatitis body surface area capillary malformation calcineurin inhibitors corticosteroids group A streptococcus herpes simplex virus potassium hydroxide polymerase chain reaction Stevens ohnson syndrometoxic epidermal necrolysis staphylococcal scalded skin syndrome ointment varicella oster virus

Dermatology

Also see page xviii for a list of other abbreviations used throughout this book

MORPHOLOGY  Macule (,1 cm in diameter) and patch (.1 cm): A at nonpalpable change in the normal color of the skin  Papule (,1 cm) and plaque (.1 cm): An elevated ellcircumscribed palpable change above the skin surface  Vesicle (,1 cm) and bulla (.1 cm): A raised elldemarcated uid lled change in the skin uid can be clear yello andor hemorrhagic  Pustule: A , cm in diameter lesion raised above the skin surface lled ith seropurulent uid  Nodule: A . cm in diameter lesion ith deeper involvement than a plaue extending into the deep dermis and subcutaneous tissue  Purpura: Nonblanchable erythematous to violaceous discoloration of the skin represents extravasated blood

11

NEONATAL/INFANTILE ERUPTIONS VESICULOPUSTULAR ERUPTIONS See able  able 

Differential Diagnosis of Infantile Vesicopustular Eruptions

Diagnosis

Clinical Presentation

Acropustulosis of infancy

Pustules/vesicles on palms and soles; presents/recurs birth to 3 years

Erythema toxicum neonatorum

Discrete vesicles papules pustules on erythematous base; spares palms and soles; ne lesions up to  days

nfantile acne

Presents at 3– months; erythematous papules and pustules primarily on face/upper chest Continued 235

able 

Differential Diagnosis of Infantile Vesicopustular Eruptions—cont’

Diagnosis nfections acterial mpetio neonatorum unal onenital candida

Dermatology

iral eonatal  eonatal varicella iliaria

Clinical Presentation

laccid elldemarcated bullae; leaves erosions crust collarette of scale sually systemically unell; eneralied erythematous papules vesicles pustules erythroderma; presents on rst day of life rouped vesicles on erythematous base acules and papules evolvin to vesicles that crust over; presents ithin 5 days of birth locae of eccrine ducts associated ith excess heat/humidity iliaria crystillina –2 mm supercial vesicles ithout sin erythema iliaria rubra “heat rash” small erythematous papules and pustules

11

eonatal cephalic pustulosis

ormerly non as “neonatal acne”; Presents in rst 4 ees of life

cabies

Papules vesicles pustules burros in axillae nec eb spaces palms and soles

ucin blister

olitary ovoid blister or erosion on noninamed sin

ransient neonatal pustular melanosis

Pustules that rupture and leave a collarette of scale pimented macules; more common in blac infants; presents at birth

enerally resolves spontaneously; severe cases may reuire topical etoconaole to treat alaseia furfur

HSV, Herpes simplex virus.

SUCUTANEOUS AT NECROSIS O THE NEORN SCN  Etiology: anniculitis of unknon etiology thought to be related to hypoxiahypothermiatrauma at birth  Clinical presentation nset in rst fe eeks of life benign selflimited rythematous plauesnodules over cheeks arms trunk buttocks andor legs May be tender to palpation  Management esolves spontaneously ithout scarring ithin eeks Monitor serum calcium for up to  months PEARL ypercalcemia is common in infants ith subcutaneous fat necrosis of the neborn and may be severe and lifethreatenin erum calcium levels should be monitored for up to  months

DIAPER DERATITIS 236 See able 

able 2

Etiolog an anageent of Diaper Deratitis Etiolog

Clinical Presentation

Inestigations

anageent

rritant contact dermatitis

Proloned contact  riht red erythema  scale erosions ith urine and  onvex surfaces often spares the folds feces friction

one necessary

 Decrease contact time beteen sin and et diaper  inc oxide  opical corticosteroids

andidiasis

Candida albicans

 ntense erythema ith desuamation/ supercial erosions; satellite pustules  avors folds enitalia

f dianosis uncertain  preparation unal culture

opical antifunal e clotrimaole

eborrheic dermatitis

Malassezia furfur  ebum over production

 elldened pin to erythematous patches; thin plaues ith ay or reasy yelloish scales  ntertriinous areas scalp

one necessary

 ood sin hyiene  etoconaole cream or  ombination of topical antifunal 1 corticosteroids

ullous impetio

Staphylococcus aureus

 laccid bullae vesiculopustules supercial erythematous erosions ith collarette of scale

ram stain and bacterial culture

Antibiotics

Acrodermatitis enteropathica

Autosomal recessive inherited disorder

 riad of acral and periorocial sin lesions erythem atous scalin crusted psoriasiform ecematous or vesiculobullous eruption diarrhea and alopecia  ocalied around body orices buttocs and extensor surface of maor oints

inc levels 5 mc/d or loer

inc supplementation

 in biopsy  hec hematoloic bone mar ro pulmonary hepatosplenic renal seletal  systems

or sinlesystem sin disease  opical corticosteroids  opical antimicrobials  arroband  PA

anerhans lonal proliferative  Pin to sin colored papules pustules vesicles cells histiocytosis disorder erosions and/or ulcers  Petechiae/purpura  issures in perineal area

CNS, Central nervous system; KOH, potassium hydroxide; PUVA, psoralen and ultraviolet A; UVB, ultraviolet B.

11

23

Dermatology

Dermatology

SEORRHEIC DERATITIS  Clinical maniestations a esions erythematous plaues ith yello greasy scales b Sites scalp axilla trunk andor exural areas  Management a ducation and reassurance because it resolves spontaneously in most infants b Application of gentle emollient or baby shampoo folloed by careful removal of scales using a so toothbrush or comb c If extensive or resistant may try limited course of lo potency topical steroid  hydrocortisone or topical antifungal aole etoconaole 

NEONATAL/INFANTILE VASCULAR LESIONS

INANTILE HEANIOA  Denition Benign tumor of endothelial cells  Clinical maniestations a Supercial bright red papular deep bluish nodular and mixed b resents at birth or in eeks thereaer as macular lesion c apid proliferation phase continues until average  months then groth plateaus d Subseuent slo involution over several years complete involution 11 in  by  years  by  years and  by  years  Complications: lceration functional impairment depending on location airay involvement aesthetic complications and complex associations see belo  Management a No interention for maority b Pharmacological treatment indicated for functional impairment potential poor cosmetic outcome ulceration i opical timolol maleate  gel tice daily for supercial lesions not reuiring systemic therapy ii ral beta blockers nadolol or propranolol for all other lesions reuiring treatment • Contraindications include extreme prematurity signicant reactive airay disease infants , kg bradycardiaheart block pheochromocytoma • Monitor vitals regularly Discuss signs hypoglycemia • Average course of treatment  year continued until complete resolution or lack of e¤ect and then tapered sloly to prevent rebound groth iii lastic surgery or laser as needed for cutaneous residua aer involution is complete 238  Prognosis Maority completely resolve ithout complication or residua

CAPILLAR ALORATIONS C  Neus simple salmon patchstork bite  Epidemiology  of all neborns  Clinical maniestations inkred patches ith poorly dened borders common sites include nape of neck glabella and eyelids  nestigations: umbosacral lesions accompanied by other stigmata of spinal dysraphism should undergo spinal imaging S , months MI . months  Prognosis reuently fade or disappear by  to  years of age

Dermatology

 ssociations a PCE() syndrome: Posterior fossa brain malformations large segmental facial hemangioma arteriopathy cardiac anomalies eye abnormalities sternal defects reuires MIMA of the head and neck ophthalmology evaluation and echocardiogram b PEV syndrome: Perineal hemangioma large segmental external genitalia malformations lipomyelomeningocele vesicorenal abnormalities imperforate anus skin tag c Neonatal hemangiomatosis: multiple cutaneous hemangiomas oen small . associated ith visceral hemangiomas liver most common I tract brain rarely Screen all infants ith . heman giomas ith abdominal ultrasound to look for liver hemangiomas others endoscopy echo only if symptoms monitor thyroid function hen liver lesions present associated ith hypothyroidism

11

 Neus ammeus (portine stain)  Epidemiology:  of neborns  Clinical presentation: Blanchable red to pink patch oen segmental unilateral and respecting midline resent at birth gros proportionately ith child  ssociations a Sturgeeber syndrome in  of facial  distribution facial CM 1 leptomeningeal angiomatosis 1 eye involvement b lippelrénaunay syndrome CM 1 venous and lymphatic malformations 1 so tissue overgroth asymmetry c MacrocephalyCM syndrome  Management: aser improves redness and prevents nodularity thickening from occurring in adulthood

DERMATITIC ERUPTIONS ATOPIC DERATITIS AD  is actors: amily or personal history of atopy gene mutations

23

Dermatology 11

 Clinical maniestations a Cutaneous eatures: ruritis and dry skin are maor features Appear ance and distribution otherise dependent on age and chronicity i # years scaly erythematous crusted plaues to extensor surfaces cheeks andor scalp esicles and signicant exudate may be presents Diaper area is typically spared ii Childhood more chronic lichenied plaues to exural surfaces b ggraating actors Cold eather concurrent illness seating contact sensitivity andor secondary infection c Course o disease: Chronic illness that axes and anes Many remit by adulthood but family history of AD persistence in childhood more severe disease are risk factors for persistence in adulthood  Management a in care i Moisturiers minimum  to  times daily during ares and as maintenance ointments and creams preferred to lotions and oils ii egular bathing – minutes lukearm ater – 3 daily ith immediate application of emollient prescribed topical medicines to active areas and moisturiers to other areas iii Cotton clothing keep room cool humidier iv Avoid irritants use mild soaps detergents double rinse avoid bleaches fabric soeners ool andor synthetic bers v etroleum elly around mouth to avoid irritation from contact hen eating vi ack of evidence for food triggers avoid elimination diets because of risk of malnutrition PEARL elect your vehicle for topical steroids thouhtfully intment provides optimal absorption of medication reams and lotions may stin hen applied to open sin otions or oils may be useful for hairy surfaces scalp

240

b opical medicines i opical corticosteroids CS See able  otency and vehicle dependent on age severity and location cover all a¤ected areas ith thin layer and treat to complete resolution rstline option for infants andor mild to moderate disease in childhood • ace and folds  hydrocortisone ointment to a¤ected areas tice daily until clear • Body  betamethasone valerate ointment to a¤ected areas tice daily until clear

Dermatology

ii opical calcineurin inhibitors CNI alternative to topical steroids as steroidsparing agents may sting on application • imecrolimus  cream o¤label use in , years tice daily until clear mild to moderate AD • acrolimus  o¤label use in , years or  o¤label use in , years ointment tice daily until clear moderate to severe AD iii Maintenance therapy topical CS or CNI  to  timeseek to areas that commonly are during periods of remission increases duration beteen ares compared ith moisturiing alone c dunctie measures i Bleach baths in patients ith recurrent skin infections ii et raps over petroleum elly or mild topical CS for severe lichenied areas iii ral antihistamines hydroxyine or diphenhydramine for severe pruritis resulting in loss of sleep iv hototherapy option for older patients ith moderate to severe disease  Complications a acterial superinection most oen Staphylococcus aureus i Clinical presentation eeping ooing honeycrusting pustules ii reatment topical mupirocin  cream tice daily for localied disease empiric oral cephalexin for more extensive infection b Ecema herpeticum superinfection ith S or  i Clinical presentation punchedout erosions hemorrhagic crusts vesicles oen unell ith fever and lymphadenopathy ii Investigations sab for viral C andor culture

11

PITALL Ecema herpeticum involvin the nasal tip can lead to ocular complications ie herpes erato conunctivitis because of innervation by  branch of trieminal nerve

iii reatment I acyclovir for extensive involvement periorbital involvement younger patients  acyclovir for milder mucocu taneous involvement Avoid topical steroids to a¤ected areas until all lesions are crusted over c ther complications extensive molluscum mental health issues anemia of chronic disease

24

Dermatology 11

ALLERIC CONTACT DERATITIS  Etiology a Delayed type hypersensitivity reaction Allergen specic and reuires prior exposure b Common allergens urshiol in poison ivyoak latex nickel  Clinical maniestations: rythematous pruritic plaues  vesicles and bullae in areas of allergen contact but may spread more di¤usely  Management: Avoid contact allergen Antihistamines for itch opical CS or CNI oeek course of systemic steroids ith slo taper for more extensive cases ith blistering involvement oen reuired for poison ivyoak

PAPULOSQUAMOUS ERUPTIONS PSORIASIS  Clinical presentation a esions elldemarcated erythematous plaues ith adherent silvery scale  itch 1 oebner phenomenon the appearance of skin lesions in areas of trauma b Distribution extensor surfaces scalp umbilicus exuresfolds inverse psoriasis nail involvement variants include palmar plantar pustular guttate may follo AS infection PITALL Psoriasis can be confused ith other papulosuamous sin conditions includin ecematous dermatitis seborrheic dermatitis tinea corporis and pityriasis rosea

 Management a Comorbidity screening: er American Academy of ediatrics guidelines for associated obesity diabetes dyslipidemia hypertension nonalcoholic fatty liver polycystic ovary syndrome inammatory boel disease IBD arthritis mood disorders b opical C i lo potency ie desonide ung tice daily for face and folds mid to highpotency ie betamethasone valerate  ung tice daily for body ii Incorporate topical vitamin D analog calcipotriene tice daily or mixed ith potent CS Dovobet iii May add keratolytics CS compounded ith  salicylic acid iv or scalp tar shampoo and uocinolone acetonide oil daily 242

ACNE VULGARIS  Pathogenesis ollicular hyperkeratiniation increased sebum production inammation Cutibacterium acnes (formerly Propionibacterium acnes) groth  Clinical maniestations a Noninammatory open and closed comedones b Inammatory papules pustules nodules c ostinammatory erythema and hyperpigmentation d Scarring ith nodulocystic lesions e ocation predominantly located on the face may involve the upper chest and back  nestigations a sually not indicated unless clinical signs of hyperandrogenism b All acne presenting beteen  and  years of age reuires bloodork total and free testosterone DAS hydroxyprogesterone bone age endocrinology referral

Dermatology

c opical CN useful as steroidsparing agent for facefolds tacrolimus  ung tice daily to a¤ected areas d Phototherapy e ystemic agents i No systemic CS cause erythroderma on ithdraal ii Methotrexate cyclosporine retinoids acitretin biologics ustekinumab iii uttate psoriasis throatperianal sab for Streptococcus treat appropriately

11

PEARL emale patients ith acne and oliomenorrhea should undero orup for polycystic ovary syndrome

 Management: Depends on primary morphology severity clinician experience and patient preference Combination therapy oen preferred able  a opical retinoids tretinoin adapalene taarotene b opical antimicrobials erythromycinclindamycin not recom mended as monotherapy dapsone benoyl peroxide c ral antibiotics tetracyclinedoxycyclineminocycline not recommend for use under the age of  years old alternatives are aithromycin trimethoprimsulfamethoxaole 243

Dermatology

d ral isotretinoin  mgkgday 3  month then  mgkgday Continue until cumulative dose beteen  and  mgkg i ests needed before initiation and monthly s urinalysis CBC cholesterol triglycerides bC ii ighly teratogenic avoid conception during and  month posttreatment need to forms of contraception during active treatment iii Side e¤ects cheilitis xerostomia xerophthalmia conunctivitis myalgia headache teratogenicity hepatitis hypercholesterol emia hypertriglyceridemia pseudotumor cerebri exacerbation of IBD ares premature epiphyseal closure depression e ormonal therapy oral contraceptive pill spironolactone  Complications: ermanent scarring mental health issues able 3

irstLine Treatent for Acne

il Coeonal or Inaator

11

opical retinoida OR enoyl peroxide Pb OR opical combinationc

oerate opical combinationc ith or ithout systemic antibioticsd OR ral contraceptive females

Seere ScarringNoulocstic ral isotretinoin

a

Example: adapalene 0.1 el HS. Example: enoyl peroxide  el daily.  ptions inlude topial retinoid 1 B, topial lindamyin 1 B, or topial retinoid 1 lindamyin 1 B. d Example: doxyyline 100 m  dailyB 3 – months. 

CUTANEOUS BACTERIAL INFECTIONS See able  Also see Chapter  Infectious Disease PEARL mpetio presents ith yelloold or “honey” crusts

244

Cutaneous acterial Infections

Diagnosis

Clinical eatures

Inestigations

anageent

ellulitis

 nfection of dermis and subcutaneous tissue  Poorly demarcated erythema armth induration  tenderness fever Streptococcus pyogenes and taphylococcus aureus most common

mpetio

 upercial infection  Erythematous papules vesicles pustules ith thic honeycrustin  ullous variant involves proression to lare accid bullae that may rupture S. aureus or S. pyogenes are most common

ab for  if  opical antibiotics dianosis uncertain if mild/focal e mupirocin ointment  f more severe or extensive then P antistaphylococcal coverae e cephalexin cloxacillin   antibiotics if systemically unell

taphylococcal scalded sin syndrome

S. aureus exfoliative toxin causes sin exfoliation at ranular layer  ender erythematous eruption of central face nec axillae roin torso folloed by accid bullae eneralied desuamation; typically spares conunctivae and mucous membranes

ab potential foci of infection nares conunctivae umbilicus neonates throat and perianal collect blood culture abs of bullae are sterile

 sually benin selflimited course  inimie handlin  Petroleum elly to affected sin  nonstic aue   cefaolin  cloxacillin omplete – days course ith P cephalexin after clinical im provement and no ne lesions

oxic shoc syndrome

 Acute toxinmediated illness caused by S. aureus or S. pyogenes  Extensive macular erythema/ erythroderma involvin mucous membranes fever hypoten sion multioran involvement; late desuamation particularly palms and soles  hair loss and nail sheddin

aboratory testin reective of shoc and oran dysfunction in sabs positive in maority of cases rarely positive blood culture

 3–5 mortality  upportive care includin hemody namic support  Empiric  ceftriaxone P vancomycin P lindamycin antitoxin effect  onsider 

ral cephalexin or  cefaolin dependin on severity

Dermatology

able 4

11

C&S, Culture and suseptiility; IV, intravenous; IVIG, intravenous immunoloulin; PO, y mouth.

245

CUTANEOUS VIRAL INFECTIONS Also see able  for childhood exanthems

Dermatology 11

HSV UCOCUTANEOUS INECTIONS Also see Chapter  Dentistry herpetic gingivostomatitis and Chapter  Infectious Diseases  Etiology: S and   Clinical maniestations: rimary eruption most severe folloed by recurrences at same site a erpes labialis i ain burning tingling or itchiness may precede eruption ii rouped vesicles on an erythematous base n pustules n erosions and crusts ith scalloped border involving mouth and lips iii eals ithin  to  eeks b erpetic hitlo grouped vesicles pustules erosions crusts and paronychia involving the digits  nestigations: anck smear from early lesion multinucleated giant cells sab for viral culture and C bacterial culture to rule out alternative diagnosessecondary bacterial infection  Management: Antivirals initiated ithin  to  hours of eruption onset reduce viral shedding and shortens duration a opical acyclovir   times daily 3  days may hasten resolution b ral acyclovir or valacyclovir c Chronic suppressive therapy may be considered for freuent recurrences OLLUSCU CONTAIOSU  Etiology: oxvirus  Clinical maniestations leshcolored domeshaped umbilicated papules  Management: Spontaneous regression aer  years maority reuire no treatment ptions for extensive disease or signicant distress cantharidin podophyllin cryotherapy andor curettage VERRUCAE ARTS  Etiology  human papilloma virus  Epidemiology ertical transmission ie contracted from mother ith genital arts possible up to  years of age Infection via direct skin contact during diapering  Clinical maniestations: ulgaris anyhere on body plantar palms and soles accuminata genitals and at sites of trauma face or extremities

246

HAND OOT AND OUTH H DISEASE  Etiology Coxsackievirus ecaloral route of transmission  Clinical maniestations a Classic M ever diarrhea oral vesiclesulcers and erythematous macular or papulovesicular rash to palms soles and groinbuttocks b Atypical M Coxsackievirus A More severe skin involvement than classical M papular vesiculobullous andor erosive lesions that extend beyond the palms and soles can be accompanied by petechial and purpuric eruption may favor sites of skin trauma ie ecema

Dermatology

 Management  ulgaris and plantar  salicylic acid under duct tape for  eek debride and continue ith eaker overthecounter preparations Cryotherapy in older childrenadolescents  Accuminata topical eregen tice daily or imiuimod  times per eek until resolution Consider child abuse if . years lack of household contact ith arts or risk factors present

PEARL nychomadesis sheddin of the nails may occur –2 months after infection ith coxsacievirus infection ails rero normally thereafter

11

 nestigations ule out S as needed sab for CS if suspicion for secondary bacterial infection can do stool enterovirus C if diarrhea but not necessary in most cases  Management: Supportive

CHILDHOOD EXANTHEMS See able  PITALL aricella is hihly contaious and is transmitted via direct contact or aerosolied droplets of nasopharyneal secretions Airborne precautions are indicated hen infection is suspected

24

Classic Ciloo Eantes

Diagnosis

Infectious Agent

Incuation Perio

easles

easles virus

–4 days

ubella

ubella virus

– days

Ras Appearance

Oter eatures

Dermatology

11

248

able 5

Inestigations

anageent

Coplications

 Prodrome ever Erythematous macules and couh corya papules that rst appear on conunctivitis head/face and spreads ceph  opli spots ray alocaudally and fade after hite papules on 5 days in order of the buccal mucosa appearance durin prodrome

eroloy   P for virus isola tion throat or naso pharyneal sabs blood urine

 recommends itamin A once daily for 2 days to de crease morbidity and mortality

easlesassociated im munosuppression diar rhea pneumonia acute disseminatin encepha lomyelitis subacute sclerosin panencepha litis – years later

 Prodrome fever Erythematous macules and headache upper papules that bein on the respiratory face spreadin cephalocau symptoms dally and fade after 2–3 days  orchheimer spots in order of appearance petechial macules on the soft palate

eroloy   and P throat or nasopharyneal sabs preferred; blood and urine also possible

upportive

 Arthralia and arthritis  arely hepatitis myocarditis pericar ditis hemolytic ane mia and thrombocy topenia encephalitis

Erythema infectiosum ifth Disease

uman parvovirus 

4–4 days

 Erythematous macules on the chees spares the nasal bride and perioral area “slapped chee”  econd stae occurs –4 days later erythema tous macules and papules in lacy reticular pattern on the trun and limbs for –3 ees may ax and ane  Papular purpuric loves and socs syndrome petechial and purpuric eruption on acral sites and exures

Prodrome occurs – days before exanthem appears lorade fever myalia headache

P testin for par vovirus  pre ferred test; erum seroloy  

oseola infantum

 

– days

Exanthem appears after fever  Prodrome ih fever  P NOT resolution rosepin macules occurs for 3–5 days routinely needed and papules on the nec  aayama spots trun proximal extremities red papules on the and occasionally the face soft palate and uvula  haracteristic ndin uvular and palatolos sal unctional ulcers

upportive

ransient aplastic crises thrombocytopenia neu tropenia and pancyto penia; fetal hydrops

upportive care

ebrile seiures common

Continued

11

24

Dermatology

Classic Ciloo Eantes—cont’

Diagnosis

Infectious Agent

Incuation Perio

carlet fever

A

aricella chicenpox

aricella oster virus

Dermatology

11

250

able 5

Ras Appearance

Oter eatures

Inestigations

anageent

Coplications

– days

 ed nely textured sandpaper rash preads from face to rest of body  Perioral pallor straberry tonue  Pastia’s lines in antecubi tal and axillary folds

    

hroat sab for A

 Antibiotic treatment of A if throat sab positive  upportive care

 heumatic fever very rare  Arthritis  Poststreptococcal lomerulonephritis

–2 days

rops of pruritic macules papules and vesicles that leave crusts and erosions after rupture esions present at various staes concurrently

Prodrome of fever malaise

iral P for  from vesicles and/or blood

 upportive for healthy patients  antivirals for immunocompro mised  Prevention ith vaccine and/or  for exposed atris patients ee hapter 23 mmunoprophy laxis

ncreased ris of invasive roup A streptococcal infections encephalitis eye syndrome pneumonia hepatitis

ever ore throat eadache ausea omitin

GAS, roup A streptoous; HHV, human herpesvirus; Ig, immunoloulin; PCR, polymerase hain reation; VZV, variella oster virus; VZIG; variella oster immune loulin; WHO, orld Health raniation.

FUNGAL ERUPTIONS See able  Tinea Capitis Versus Corporis Tinea Capitis Hea

Tinea Corporis o

Etioloy

 Trichophyton tonsurans or Microsporum canis

M. canis, T. mentagrophytes, T. tonsurans, T. rubrum

linical features

 5 linical patterns diffuse scalin circumscribed alopecia ith scale blac dot broen hairs erion boy mass pustular

anaement

 crapin for  and funal culture  crapin for  and funal culture  ystemic treatment only erbinane  ystemic treatment rarely needed P for 4– ees ,2   opical terbinane ciclopirox 25 m daily; 2–4  25 m clotrimaole etoconaole all efcacious applied D daily; .4  25 m daily  esolution may tae up to 4 ees

 Pruritic annular scaly plaues ith active border and central clearin

Dermatology

able 

BID, ie daily; KOH, potassium hydroxide.

INFESTATIONS See able  Cutaneous Infestations

able  Diagnosis

Clinical eatures

Treatent

cabies

 Sarcoptes scabiei var hominis mite  ntensely pruritic papules/ burros nodules dermatitis  avors abdomen dorsa of hands eb spaces enitalia sin folds

 reat all household contacts  5 permethrin cream/lotion from nec don include head in infants for –4 h then ash off; repeat after  days  ash beddin/clothin in hot ater  reat pruritus ith antihistamines topical midpotency corticosteroid if needed

Pediculosis lice

 Pediculosis louse 3–4 mm lon and mobile; es  mm and rmly adherent to hair shaft  an be capitis Pediculus capitis or pubis Pthrius pubis; crab louse

 ash and toeldry hair; apply permethrin  for  min and rinse; repeat in  days  inearandater  soas and netooth comb  f eyelash involvement apply petroleum elly D to D for  days  oa combs hair accessories in permethrin shampoo or boil for  min; ash clothin/ beddin in hot ater

11

PITALL Persistent pruritis after treatment does not necessarily indicate treatment failure; postscabetic pruritis may persist for up to 4 ees and can be manaed ith topical corticosteroids

ALOPECIA See igure  for general approach to hair loss

25

11

Dermatology

252 igure 111 Approac to Nonscarring Hair Loss

Alopecia

Pattern

Diffuse

Clues

• Stress • Medications • Dietary restriction

Diagnosis

elogen effluium

Localized

• Chemotherapy

nagen effluium

Patterned

• Eclamation pt hairs • ail pitting •  autoimmune disease

• Pruritis • Scale • Lymphadenopathy • erion

• Irregular patches • Eyelash/bro inolement •  aniety

lopecia areata

inea capitis

richotillomania

• hinning of erte • ther signs of hyperandrogenism •  male pattern” balding

ndrogenetic alopecia

ACUTE BLISTERING REACTIONS See Box  and able 

• • • • • • • • •

tevens ohnson syndrome/toxic epidermal necrolysis ycoplasmainduced rash and mucositis Erythema multiforme ullous impetio/staphylococcal scalded sin syndrome Autoimmune blisterin diseases linear immunolobulin A/chronic bullous dermatosis of child hood; pemphius; pemphioid; bullous cutaneous lupus Dermatitis herpetiformis ullous xed dru eruption riction blisters urns

able 

Dermatology

o 111 Differential Diagnosis of Most Common Acute Bullous Eruptions

Coparison of Ertea ultifore coplasa Inuce Ras an ucositis Steensonson Snroe an Toic Epieral Necrolsis Ertea ultifore E

coplasa Inuce Ras an ucositis IR

Steensonson Snroe SS an Toic Epieral Necrolsis TEN

Etioloy

 Mycoplasma pneumoniae drus

Mycoplasma pneumoniae

Drus ADs antibiotics anticonvulsants

Prodrome

ay have precedin herpes labialis

ever couh precede mucosal and sin eruption

ever sin tenderness precedes the sin eruption by –3 days

linical features

 ypical taret lesion  Prominent mucosi  ender erythematous dusy purpuric patches n three distinct concen tis 2 mucosal accid bullae n erosions tric ones ith dusy sites involved most n desuamated plaues erythematous center often eyes and  ypically develops mouth  Extent of sin detachment over the extremities  ariable cutaneous    A upper . loer involvement; vesic  E overlap ulobullous taretoid face and/or trun –3 A EM minor mild to no or morbiliform  E .3 A mucosal symptoms 2 mucosal surfaces limited systemic oral ocular enital symptoms  ystemic manifestations EM maor severe fever lymphadenopathy mucosal involvement hepatitis cytopenia and fever malaise cholestasis arthralias

linical course

Appears ithin 24–2 h and usually clears ithin –2 ees ecur rences possible

in lesions heal ithout seuelae complete resolution ithin 2 ees

11

apid proression of disease; hiher mortality

Continued 253

Coparison of Ertea ultifore coplasa Inuce Ras an ucositis Steensonson Snroe an Toic Epieral Necrolsis—cont’

able 

Ertea ultifore E

Dermatology 11

coplasa Inuce Ras an ucositis IR

anaement  Antiseptic/local anes  Antiseptic/local thetic solutions for anesthetic solutions oral lesions for oral lesions  Antihistamines or  Antihistamines topical  for itch for itch  Antivirals if conrmed  reat underlyin  infection mycoplasma  onsider oral steroids infection to prevent for severe/extensive other complications disease but does not alter  phthalmoloy if course of cutane ocular involvement ous disease  phthalmoloy if ocular involvement

Steensonson Snroe SS an Toic Epieral Necrolsis TEN   or burn unit admission for supportive care infection uid and electrolyte instability respiratory distress multioran failure  Early identication and ithdraal of offendin dru  Early involvement of der matoloy ophthalmoloy and uroloy/ynecoloy  Potential systemic aents  cyclospo rine systemic steroids a inhibitors

BSA, Body surae area; CS, ortiosteroids; HSV, Herpes simplex virus; ICU, intensive are unit; IVIG, intravenous immunoloulin; NSAID, nonsteroidal antiinammatory dru; TNF, tumor nerosis ator.

GENODERMATOSES (GENETIC SKIN CONDITIONS) See able 

254

ost Coon Neurocutaneous Snroes

Neuroroatosis N1 an N

Tuerous Sclerosis

Sturgeeer Snroe

nheritance Autosomal dominant hih rate of de novo mutations

Autosomal dominant hih poradic rate of de novo mutations

utaneous   afe au lait macules ndins .5 mm before puberty .5 mm after puberty  reclin axilla and roin  eurobromas cutaneous subcutaneous plexiform 2  utaneous and subcutaneous tumors sin plaues

 ypomelanotic macules 5 mm “ashleaf spots”  Aniobromas  Periunual bromas  ibrous plaue of forehead  hareen patch  “onfetti” sin lesions  Dental enamel pits

emental facial capillary malformation/ port ine stain

ther ndins

  isch nodules iris hamartomas macrocephaly learnin disabilities seiures sphenoid sysplasia pseu doarthrosis and bone dysplasia scoliosis conenital heart disease hypertension 2  ataracts retinal hamartomas

eiures/infantile spasms developmental delay cortical dysplasia retinal hamartomas

psilateral lepto menineal ani oma seiures laucoma hemiparesis and hemihypertro phy intracranial A develop mental delay

umors

  Peripheral neurobromas cutaneous plexiform nodular optic liomas malinant peripheral nerve sheath tumors astrocytomas pheochromo cytoma leuemia astrointestinal stromal tumor rhabdomyosarcoma 2  channomas vestibular other cranial peripheral nerve neuro bromas cranial/spinal meniniomas liomas spinal cord ependymomas

EA lioneuronal hamartomas cortical tubers subependymal nodules cardiac rhabdo myomas renal anio myolipomas lymphani oleiomyomatosis

Dermatology

able 

11

AVM, Arteriovenous malormation; NF, neuroromatosis; SEGA, suependymal iant astroytoma.

EPIDERMOLYSIS BULLOSA (EB) PEARL Alays consider epidermolysis bullosa on the differential of conenital blisterin ote that severity of disease in the neborn is not reective of overall subtype or lonterm pronosis

 Denition: roup of heterogeneous mechanobullous diseases charac teried by extreme skin fragility usually present at birth or in infancy

255

 eerity: anges from more benign nonscarring B simplex to more severe scarring ith multisystem involvement hich is oen fatal unctional D dystrophic B  Management: Multidisciplinary support including education symp tomatic management support special attention to temperature control airay involvement nutrition uid balance secondary infections functional impairments

TOPICAL STEROIDS  Potency a See able  for topical CS potencies Dermatology 11

able 

Classes of Topical Corticosterois

Classroup

Representatie Eaples

uperhih potency/roup 

clobetasol propionate 5 un

ih potency/roup 2

uocinonide 5 un

ih potency/roup 3

betamethasone valerate  un mometasone furoate  un

edium potency/roup 4

hydrocortisone valerate 2 un mometasone furoate  cream

oermid potency/roup 5

betamethasone valerate 5 un hydrocortisone valerate 2 cream

o potency/roup 

desonide cream

east potent/roup 

hydrocortisone  un

ung, intment.

 ide eects: Increase ith potency and duration atrophy striae hypertrichosis perioral dermatitis delayed ound healing exacerbation of skin infections  Vehicle a intment most potent greasy and hydrating use on thick dry skin avoid on hairy areas or face b Creams less potent than ointment can be used on face may sting on open skin c otions oils and gels more drying much less potent good for scalp  uantity a ingertip unit  used to estimate uantity reuired b ne  5 amount of cream sueeed out of tube onto tip of index nger from DI to distal aspect 5 , g c ne  treats the sie of  palm ie for isolated bilateral hand involvement 5   3 BID 5  day 3  days 5   256 month 5  g for  month

Darro D reene A Mancini A Nopper A Diagnosis and management of infantile hemangioma Pediatrics e–e icheneld  rakoski AC iggott C et al videncebased recommen dations for the diagnosis and treatment of pediatric acne Pediatrics suppl S–S icheneld  Bogunieic M Simpson  et al ranslating atopic dermatitis management guidelines into practice for primary care providers Pediatrics – aller AS Mancini A Hurwitz Clinical Pediatric Dermatology th ed hiladelphia A lsevier 

USEFUL WEBSITES Dermnet Ne ealand dermnetnorg cema Society of Canada ecemahelpca

Dermatology

FURTHER READING

11

25

CHAPTER

12

Development Audrey Tilly-Gratton • Claire Nguyen • Jenna Doig • Amber Makino

Common abbreviations Normal patterns of development Developmental red ags Developmental assessment Behavior issues Early developmental impairment and intellectual disability Autism spectrum disorder Cerebral palsy Attention decit/hyperactivity disorder Specic learning disorders Speech and language disorders Developmental coordination disorder

258

COMMON ABBREVIATIONS ABA ADHD ADOS ASD OO  DS D S D   S 

applied behavior analysis attention decit/hyperactivity disorder autism diagnostic observation schedule autism spectrum disorder cognitive orientation to daily occupational performance cerebral palsy Diagnostic and Statistical Manual of Mental Disorders, th dition early developmental impairment gross motor function classication system intellectual disability inborn error of metabolism individual education plan picture exchange communication system rapid eye movement

Development

Also see page xviii for a list of other abbreviations used throughout this book

NORMAL PATTERNS OF DEVELOPMENT  Developmental domains a Gross motor: movements using the large muscles b Fine motor: movements using the hands and smaller muscles oen involving selfhelp skills c Communication: receptive and expressive language speech nonverbal communication d Cognitive: reasoning memory problemsolving skills e Social-emotional and behavioral: attachment selfregulation interaction ith others  Developmental milestones: see able  for average developmental milestones from birth to  years

12

259

Development

12

260

able 2

Emerging eelomental Milestones From irt to  ears

Age

Gross Motor

Fine Motor

Social

Language and Communication

Self-Hel

Birth

Kicks legs and thrashes arms; Moro, stepping, placing and grasp reexes present

Looks at obects or aces

esponds positiel to eeding and comorting

ries; startled b lod sdden sonds

lert interested in sights and sonds

 month

aises head and chest hen ling on stomach

ollos moing obects ith ees

ocial smile; becomes actie hen sees hman ace

ries in distinct a hen hngr

cks ell; responds to oices trns head toard oice

2 months

entral sspension head sstained in plane o bod; pll to sitting head lags; holds head stead hen held sitting

olds obects pt in hand; hand regard; ollos moing obect 8 degrees

ecognies motherprimar caregier; listens to oice and coos

Makes sonds “ah,” “eh,” “gh”; laghs

eacts to sight o bottle or breast

 months

entral sspension lits head and chest, arms extended; tonic neck postre predominant; pll to sitting head lag partiall compensated; earl head control ith bobbing motion; back ronded

hakes rattle; reaches toard and misses obects; aes at to

ecognies most amiliar adlts

as “ahh,” “ngah”

ncreases actiit hen shon to

 months

rns arond hen ling on stomach; in prone position, lits head and chest— head in approximate ertical axis, legs extended; pll to sitting no head lag; head stead, held orard; sitting ith ll trncal spport; hen held in standing erect position, pshes ith eet

ts tos or other obects in moth

nterested in on image in mirror—smiles, plal; laghs ot lod; ma sho displeasre i social contact is broken; excited at sight o ood

eals, “ah-goo” sonds

eaches or larger obects; sees small obects bt makes no moe to them

5 months

olls rom stomach to back

icks p obects ith one hand

eacts dierentl to strangers stranger anxiet

Makes raing sonds—gies “raspberries”

 months

olls rom back to stomach

ransers obects rom one hand to another

eaches or amiliar persons

Babbles; trns to on name

Looks or obect ater it disappears rom sight

 months

its ithot spport; ma spport most o eight hen standing; bonces actiel

olds to obects one in each hand at same time; grasps sing radial palm; rakes at small obect

ets pset and araid i let alone

Makes sonds, sch as “da,” “ba,” “ga,” “ka,” “ma”

nticipates being lited b raising arms

8 months

rals on hands and knees

ses orenger to poke, psh, or roll small obects

las “peek-a-boo”

Makes sonds like “ma-ma,” “da-da,” “ba-ba” to-sllable babbling

eeds sel cracker or cookie

9 months

lls sel to standing position

icks p small obects sing onl nger and thmb pincer grasp

esists haing to taken aa

mitates speech sonds

 months

idestepsalks arond rnitre hile holding on

icks p to small obects in one hand

las “pat-a-cake”

 months

tands alone ell

ts small obects in cp or other container

hos or oers to to adlt

ses “Mama” or “ada” specicall or parent

icks p spoon b handle

2 months

limbs p on chairs or other rnitre; alks ith one hand held; “crises”

rns pages o books a e at a time

mitates simple acts, sch as hgging or loing doll; plas simple ball game; makes postral adstment to dressing

as one ord clearl; points in response to ord

emoes socks

 months

alks ithot help

Bilds toer o to or more blocks

las ith other children

hakes head to express “no”; hands obect to o hen asked

Lits cp to moth and drinks Continued

12

2

Development

Development

12

262

able 2

Emerging eelomental Milestones From irt to  ears—cont’d

 months

toops and recoers

Marks ith pencil or craon

ies kisses

sks or ood or drink ith sonds or ords

nsists on eeding sel

5 months

ns

cribbles ith pencil or craon

reets people ith “hi” or similar; hgs parents

as to ords besides “Mama” or “ada”; makes sonds in seences that sond like sentences

eeds sel ith spoon

8 months

its on small chair; alks pstairs ith one hand held; kicks a ball—good balance and coordination; moes tos into and ot o container

Bilds toer o or or more cbes; imitates ertical strokes; dmps small obect rom bottle

ometimes sas “no” hen interered ith; kisses parent ith pckering o lips; exhibits shared attention points to share interesting obseration ith another

ses e or more ords as names o things ie, ater, cookie, clock; ollos a e simple instrctions; nderstands phrases sch as “ie me that” hen gestres are sed; recognies names o common obects; identies one or more parts o bod

eeds sel; eats ith a ork; seeks help hen in troble; ma complain hen et or soiled; knos se o toothbrsh and comb

2 months

ns ell; alks p and don stairs, one step at a time; opens doors; climbs on rnitre; thros and kicks ball

Bilds toer o six cbes; perorms circlar scribbling; imitates horiontal stroke; olds paper once imitatiel

ells immediate experiences; listens to stories ith pictres

ts to to three ords together; knos “”; points to appropriate pictre hen someone sas “ho me the dog”; has expressie ocablar o 5–25 ords

andles spoon ell; helps to ndress

 months

mps

Bilds toer o eight cbes; makes horiontal and ertical strokes bt generall ill not oin them to make a cross; imitates circlar stroke, orming closed gre

retends in pla

eers to sel b pronon “”; knos ll name

elps pt things aa

 months

oes p stairs alternating eet; rides triccle; stands momentaril on one oot

Bilds toer o nine cbes; imitates constrction o “bridge” o three cbes; copies circle; imitates cross

las simple games in “parallel” ith other children

Knos age and sex, conts three obects correctl; repeats three nmbers or sentence o six sllables; expressie ocablar o oer  ords; remembers some recent past eents

oilet trained; helps in dressing nbttons clothing, pts on shoes; ashes hands

8 months

ops on one oot; thros ball oerhand; ses scissors to ct ot pictres; climbs ell

mitates constrction o “gate” o e cbes; copies cross and sare; dras person ith to or or parts besides head; can name longer o to lines

las ith seeral children— beginning o social interaction and role-plaing

onts or pennies accratel; tells stor; asks man estions; ses or- to e-ord sentences; ses plrals; can repeat three or or nmbers; knos or colors

ses toilet alone

 months

kips

opies triangle; can name heaier o to eights

sks estions abot meaning o ords; participates in domestic role-plaing

epeats sentence o  sllables; conts  pennies correctl; ollos three-part instrctions; can name penn, nickel, and dime; ses pronons properl

resses and ndresses

Modied from Parker S, Zuckerman B, eds. Behavioural and Developmental Pediatrics: A Handbook for Primary Care. Boston, MA: Little, Brown; 199:–1.

12

2

Development

DEVELOPMENTAL RED FLAGS See able  able 22

Development

Time Period

Language Cognitie

Gross Motor Fine Motor and Self Care

Social-Emotional

eonatal period

nant does not respond to lod sonds

Mscle tone too lo to eed

aregier shos indierence or disinterest in inant

 months

ot starting to coo, does not trn toards oices

ot holding head and sholders p ith good control hen ling on tmm, not holding head ith control in spported sitting, does not bring hands together at midline

oes not smile, lagh or interact ith people

9 months

Lack o babbling ith consonants

ot rolling, not sitting independentlithot spport, does not pass obect rom one hand to another

ot sharing enoment ith others sing ee contact or acial expression

2 months

o babbled phrases that sond like talking, no response to amiliar ords eg, bottle, dadd

o orm o independent mobilit eg, craling, commando craling, bottom she, not plling to stand independentl and holding on or spport, does not eed sel nger oods or hold on bottlecp, nable to pick p small items sing index nger and thmb

oes not notice someone ne, does not pla earl trn-taking games

8 months

o clear ords, not able to nderstand short reests eg, “here is the ball”

ot standing independentl, not attempting to alk ithot spport

Lacks interest in plaing and interacting ith others, absence o pointing to sho interest or shoing gestres

2 ears

Lack o ords and not ptting ords together, inabilit to ollo simple commands

ot able to alk ell, does not attempt to eed sel sing a spoon andor help ith dressing

oes not imitate actions or ords o caregiers, tends to bang, thro or drop tos rather than se tos or their prpose

 ears

peech diclt or amiliar people to nderstand, not sing simple sentences eg, “Big car go”

ot able to alk p and don stairs independentl, not able to rn or mp, does not attempt eerda sel-care skills sch as eeding or dressing

o interest in pretend pla or interacting ith other children, diclt noticing and nderstanding eelings in themseles and others

 ears

peech diclt to nderstand, does not anser simple estions, not able to ollo directions ith to steps

ot toilet trained b da, not able to dra lines and circles, not able to alk, rn, climb, mp and se stairs condentl, not able to catch, thro or kick a ball

nilling or nable to pla cooperatiel

12

264

eelomental Red Flags

eelomental Red Flags—cont’d

Time Period

Language Cognitie

Gross Motor Fine Motor and Self Care

Social-Emotional

5 ears

ot able to anser estions in a simple conersation, inabilit to recognie shapes, letters, colors

oor balance, concerns rom teacher abot school readiness

la is dierent than their riends, nsall earl, sad, sh, angr

n age

Loss o preiosl acired skill Lo tone or high tone impacting on deelopment and nctional motor skills Lack o response to sond or isal stimli ierences beteen right and let sides o bod in strength, moement or tone Lack o or limited ee contact oor interactions ith adlts or other children arental concerns

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Development

able 22

DEVELOPMENTAL ASSESSMENT  History a resenting issue parental concerns b Pregnancy and birth history i Antenatal investigations bloodork ultrasounds ii aternal illness including pregnancy related eg hypertension 12 gestational diabetes exposure to teratogens eg alcohol nico tine other drugs OH infections iii Delivery gestational age type of delivery birtheight resuscitation complications iv eonatal history eg neonatal intensive care unit stay establish ment of feeding issues encountered and treatment needed eg aundice hypoglycemia seiures infection c edical history: previous interactions ith healthcare hospitaliations including surgeries emergency room primary care attendance subspeci ality consultations and investigations medications vaccinations d Developmental history: see Box  e Day careschool history: grades individual education plan  special supports eg educational assistant extracurricular activities f Supports and services: rehabilitation services eg physiotherapy occupational therapy speech and language therapy funding euipment g Family history: threegeneration pedigree consanguinity miscarriages/ stillbirths presence of developmental disability neurological and psychiatric conditions h Social history: home environment parental education and employ ment involvement of child protection services i evie of systems including vision hearing sleep diet dental 25 diapering/toileting feeding activities of daily living

PEARL ollateral inormation rom teachers, therapists, and other practitioners is alable

o 121

Development 12

266

Developmental History

 or each domain otlined in able 2 determine hen milestones ere achieed, crrent leel o nction, presence o an regression 2 ssessment o a ross motor eg, head control, sitting, alking, rnning, stairs, sports b ine motor eg, handedness, pincer grasp, tensil se, ippersbttons, printing skills c ommnication langages to hich the child is exposed, expressie langage eg, babbling, age o rst ords, nmber o ords, grammar, conersational abilities, receptie langage eg, response to name, olloing simple and mltistep commands, speech articlation, enc, and nonerbal eg, ee contact, gestres, pointing d ocial and pla eg, oint attention, interest in peers, pretend pla, aorite tos and actiities e daptiesel-help eg, eeding, dressing, toileting, hgiene  Behaior eg, aggression, sel-inr, hperactiit, implsiit, repetitie behaiors or mannerisms, temper tantrms, discipline strategies, sensor interests or aersions g ognitie eg, case-and-eect, ples, bod parts, colors, shapes, letters, nmbers, academic perormance, attention

 Physical eamination a nformal observation parental and professional interaction ith child observe skills in each developmental domain oys crayons and books can be helpful b roth parameters c Dysmorphology examination see hapter  enetics and eratology d eurological examination cranial nerves tone strength reexes sensory coordination gait e Head to toe head and neck eg cle palate strabismus cardiore spiratory eg murmur abdominal eg organomegaly genitouri nary eg inguinal hernia undescended testes musculoskeletal eg scoliosis contractures skin eg caféaulait macules hypomelanotic macules bromas  nvestigations: depends on specic clinical context  anagement a ultidisciplinary teambased care including input from physiothera pists occupational therapists psychologists behavioral therapists registered dietitians paediatricians psychiatrists and social orkers is a mainstay of care b Diagnosisspecic early intervention to optimie infant motor and cognitive plasticity prevent secondary complications and enhance caregiver ellbeing

BEHAVIOR ISSUES  ight terrors 1. Description: non sleep disorder presents as an abrupt aaken ing from sleep typically ithin  to  hours of falling asleep oen asso ciated ith screaming and agitation child may be ushed seating and tachycardic child does not respond to attempts at comforting by the caregiver and does not remember the episode in the morning 2. pidemiology: common in preschoolers family history common 3. anagement: supportive parental reassurance and education sleep hygiene avoid aking the child during an episode if occurring on a nightly basis may consider scheduled aakenings  ehavioral insomnia o childhood  Denition a Sleep-onset association type: diculty initiating sleep indepen dently child associates falling asleep ith certain conditions or circumstances eg caregiver presence feeding from a bottle b imit-setting type: child delays bedtime caregiver has diculty setting limits c Combined type: both of the aforementioned  anagement: parent education sleep hygiene see hapter  ental Health consistent routines gradual extinction C emper tantrums  Denition: extreme episodes of frustration or anger xamples of mani festations can include shouting screaming crying and falling to the oor  pidemiology: freuency peaks around  years of age typically a transient developmental stage  ssessment a Distinguish developmentally normative behaviors from atypical behaviors explore freuency intensity and duration b eatures hich indicate problematic disruptive behaviors include atypical behavior for the child’s developmental age that persists for

Development

c onsider educational needs including an  d amily support including counselling about diagnosis and progress system navigation access to necessary supports eg alloances respite e onsideration of comorbid conditions eg seiure disorder sleep constipation mental health f lear communication ith primary care providers is an important aspect of continuing care

12

2

at least  months occurs across situations and impairs functioning causes signicant distress for the child and family  anagement a Anticipatory guidance for positive parenting and eective discipline strategies regular praise for good behavior reducing triggers distraction/ redirection ignoring the tantrum as long as the child is safe b irstline management are parent behavior training programs

Development

EARLY DEVELOPMENTAL IMPAIRMENT AND INTELLECTUAL DISABILITY

 Description a arly developmental impairment D: failure to meet expected developmental milestones in at least to areas of functioning eg motor speech/language cognition social adaptive functioning in an individual under  years of age euires reassessment aer a period of time Also referred to as global developmental delay DD b ntellectual disability all three criteria must be met adapted from DS i Decits in intellectual functions such as reasoning problem solving planning abstract thinking udgment academic learn ing and learning from experience conrmed by both clinical 12 assessment and individualied standardied intelligence testing ii Decits in adaptive functioning that result in failure to meet developmental and sociocultural standards for personal inde pendence and social responsibility ithout ongoing support the adaptive decits limit functioning in one or more activities of daily life such as communication social participation and inde pendent living across multiple environments such as home school ork and community iii Onset of intellectual and adaptive decits occur during the developmental period  pproach to investigations see igure  a hen a more specic diagnosis is suspected folloing clinical evaluation investigations to conrm that etiology should be ordered rst b or unexplained D chromosomal microarray and ragile  testing are rstline investigations c hen no etiological diagnosis has been identied folloing history physical examination and initial genetic tests ragile  chromosomal microarray consider metabolic testing for diagnosis of treatable inborn errors of metabolism  even in the absence of red ags for a metabolic condition able  6 brain imaging see also 268 treatableidorg

Figure 121 An Aroac to nestigation of Earl eelomental mairment and ntellectual isailit 1 istory and hysical eamination 2 udiology  hthalmology or otometry  f susected seiures 

Confirmatory testing

Diagnosis established?

Yes

Diagnosis suspected?

No

No

• Chromosomal microarray • Fragile-X testing • Laboratory investigations (Table 12 • rain R if abnormal neurologic eam seiures micro macrocehaly • Consider C2 in girls ith moderate-to-severe  or suggestive clinical course

Development

Referral to rehabilitation services hile aiting for results of investigations

Yes Yes

• Family counselling • Referrals as needed

Diagnosis established?

No

12

• rain R • Consult geneticsmetabolics for • Further metabolic testing (Tier 2 of T rotocol • ene anels (eg XL Rett syndrome variants • Consider neurology referral

Yes

Diagnosis established? No eriodic reevaluation

EEG lectroencephalogram GDD/ID global developmental delay/intellectual disability MECP2 methyl p binding protein  MI magnetic resonance imaging IDE reatable ntellec tual Disability ndeavor https//tidebcorg/resources/arlydentaediatrhildHealth pdf ID linked intellectual disability odied from Bélanger SA aron  valuation of the child ith global developmental delay and intellectual disability Paediatr Cild ealt –

29

able 2

Laorator nestigations for nelained Earl eelomental mairmentntellectual isailit

Development

looda

rinea

-

-

omplete blood cont lcose Blood gas rea, creatinine lectroltes to calclate anion gap , L  reatine kinase mmonia Lactate mino acids clcarnitine prole, carnitine ree and total omocsteine opper, cerloplasminb Biotindasec erritin, itamin B2 hen dietar restriction or pica are present - Lead leel hen risk actors or exposre are present

rganic acids reatine metabolites rines, primidines lcosaminoglcans

a

Perform testin after –  of fastin. ecommended tier1 test in te reatale ntellectual isailit ndeaor  rotocol, ut not  te American Academ of Pediatrics AAP, te American Academ of eurolo AA. onsider as a rstline inestiation wen eatomeal, dstonia, anormal lier function ndins are resent. c linical eert recommendation onl. onsider iotinidase testin wen seere otonia, seiures are resent. ALT, Alanine aminotransferase; AT, asartate aminotransferase; TH, troidstimulatin ormone. 

12

rom Bélaner SA, aron . aluation of te cild wit loal deelomental dela and intellectual disailit. Paediatr Child Health. 1;:–1.

 anagement: early intervention programs ith family education and support routine health evaluations anticipate and plan for future level of independent functioning PEARL reschool deelopmental scales are not alas predictie o tre intelligence otient   cannot be accratel determined beore 5 ears o age

AUTISM SPECTRUM DISORDER  Description: persistent decits in social communication and social in teraction across multiple contexts and restricted repetitive patterns of 270 behavior interests or activities see Box  for diagnostic criteria

DSM-V Diagnostic Criteria for Autism Spectrum Disorder

 ersistent decits in social commnication and social interaction across mltiple contexts, as maniested b the olloing, crrentl or b histor xamples inclde  ecits in social-emotional reciprocit, eg, abnormal social approach, and ailre o normal back-and-orth conersation 2 ecits in nonerbal commnicatie behaiors sed or social interaction, eg, poorl integrated erbal and nonerbal commnication, abnormalities in ee contact  ecits in deeloping, maintaining, and nderstanding relationships, eg, diclties adsting behaior to sit arios social contexts seerit is based on social commnication impairments and restricted, repetitie patterns o behaior B estricted, repetitie patterns o behaior, interests, or actiities, as maniested b at least to o the olloing, crrentl or b histor xamples inclde  tereotped or repetitie motor moements, se o obects, or speech eg, simple motor stereotpies, lining p tos or ipping obects, echolalia, idiosncratic phrases 2 nsistence on sameness, inexible adherence to rotines, or ritalied patterns o erbal or nonerbal behaior eg, extreme distress at small changes, diclties ith transitions, rigid thinking patterns, greeting ritals, need to take same rote or eat same ood eer da  ighl restricted, xated interests that are abnormal in intensit or ocs eg, strong attachment to or preoccpation ith nsal obects  per- or hporeactiit to sensor inpt or nsal interest in sensor aspects o the enironment eg, apparent indierence to paintemperatre, aderse response to specic sonds or textres, excessie smelling or toching o obects, isal ascination ith lights or moement  mptoms mst be present in the earl deelopmental period bt ma not become ll maniest ntil social demands exceed limited capacities or ma be masked b learned strategies in later lie  mptoms case clinicall signicant impairment in social, occpational, or other important areas o crrent nctioning  hese distrbances are not better explained b  or   and  reentl cooccr; to make comorbid diagnoses o  and , social commnication shold be belo that expected or general deelopmental leel

Development

o 122

12

AD, Autism sectrum disorder, D, earl deelomental imairment, D, intellectual disailit. ata from Dianostic and tatistical anual of ental Disorders, t dition.

 utism spectrum disorder SD red ags a educed/atypical eye gae sharing of emotion social interest/ connectedness response to name babbling language comprehension and production gestures and imitation b xcessive/unusual manipulation or visual exploration of obects repetitive actions under/overreaction to sensory stimuli or delayed ne/gross motor skills  pproach to diagnosis a ull development assessment see the Developmental Assessment section b Diagnosis can include standardied assessments eg autism diagnostic 2 observation schedule

Development 12

 anagement a onsider investigations for underlying etiology especially if cooccurring D or regression see igure  and able  b ultidisciplinary teambased care i arly intervention is important and can include particular methodologies such as parent coaching of social communica tion skills and behavior therapy ie applied behavioral analysis ii Occupational therapy to improve ne motor decits and improve academic and selfcare skills Addresses issues ith the integration of sensory of information iii Speech and language pathology to improve verbal and nonverbal communication ay include sign language and augmentative communication techniues such as picture exchange communi cation system S c At higher risk for comorbid conditions including i Sleep disorders constipation nutritional deciencies from restricted diet ii ental health anxiety attention decit/hyperactivity disorder ADHD obsessivecompulsive behaviors disruptive behaviors

CEREBRAL PALSY

 Denition a A group of permanent disorders of the development of movement and posture causing activity limitations b Attributed to nonprogressive disturbances that occurred in the developing fetal or infant brain c Oen accompanied by disturbances of sensation perception cogni tion communication and behavior by epilepsy and by secondary musculoskeletal problems  Diagnosis a ull developmental assessment see the Developmental Assessment section b Standardied tools helpful in identifying infants at risk for cerebral palsy eg Hammersmith infant neurological evaluation rechtl’s general movements assessment  Classication systems a See igure  for classication of subtypes b See able  for dierent  classication tools i ross motor functional classication system S gross mo tor grading system describing selfinitiated movement emphasis on sitting transfers and mobility elatively stable aer  years of 272 age can be used for prognosticating and goal setting

Figure 122 Classication for Sutes of Cereral Pals Is there persisting increased muscle tone in one or more limbs? Yes

No

Are both sides of the body involved? Yes

Yes

No

No Is there generalied hypotonia ith signs of ataia?

Dysinetic CP

Spastic unilateral CP

Yes Reduced activity (tone tends to be increased)

Increased activity (tone tends to be decreased)

Dysinetic CP

Choreoathetotic CP

No

Ataic CP

onclassifiable

Development

Spastic bilateral CP

Is the tone varying?

CP erebral palsy S ollaborative roup Surveillance of cerebral palsy in urope a collaboration of cerebral palsy surveys and registers Developmental edicine and Child euroloy –

able 2

Functional Classication Sstems sed in Cereral Pals

GMFCS

MACS

CFCS

EACS



alks ithot limitation

andles obects easil and sccessll

ectie sender and receier

ats and drinks sael and ecientl



alks ith limitations no mobilit aid b  ears

andles most obects ith redced speedalit

ectie bt slo paced sender and receier

ats and drinks sael bt ith some limitations to ecienc



alks ith handheld mobilit deice

andles obects ith diclt, help to prepare or modi actiit

ectie sender and receier ith amiliar partners

ats and drinks ith some limitations to sael; there ma also be limitations to ecienc



el-mobilit ith limitations, ma se poer

andles limited nmber o obects in adapted setting

nconsistent sender and receier ith amiliar partners

ats and drinks ith signicant limitations to saet



ransported in manal heelchair

oes not handle obects

eldom eectie sender and receier ith amiliar partners

nable to eat or drink sael, consider eeding tbe

CC, ommunication, unction lassication Sstem; DAC, atin and rinkin Ailit lassication Sstem; C, ross Motor unctional lassication Sstem; AC, Manual Ailit lassication Sstem. rom Paulson A, arusAdams, . eriew of four functional classication sstems commonl used in cereral als. Children Basel. 1;:.

12

2

Development 12

ii anual ability classication system AS used to classify a child’s typical use of both hands and upper limbs for children . years of age iii ommunication function classication system S assesses everyday communication alloing all methods of communica tion eg vocaliations manual signs eye gae pictures communication boards speech generating devices to be included during classication iv ating and drinking ability classication system DAS assesses eating and drinking safety aspiration and choking as ell as eciency amount of food lost and time taken to eat in children . years of age Addresses the amount of assistance a person needs independent reuires assistance or dependent for eating and drinking  anagement a arly multidisciplinary team input b ompensatory and environmental adaptation approaches including environmental modications or specialied euipment eg orthotics alkers specialied seating c Surveillance and management of common musculoskeletal complica tions including pain contractures hip subluxation aacpdmorg/ publications/carepathays/hipsurveillanceincerebralpalsy scolio sis and osteoporosis aacpdmorg/publications/carepathays/ osteoporosisincerebralpalsy d one management may reuire oral medications eg baclofen or botulinum toxin A inections to manage hypertonia if it is causing pain limiting function or participation or impacting caregiving pathaysniceorguk/pathays/spasticityinchildrenandyoung people for care pathay for spasticity and aacpdmorg/publications/ carepathays/dystoniaincerebralpalsy for care pathay for dystonia e Siallorhea care pathay aacpdmorg/publications/care pathays/sialorrheaincerebralpalsy

ATTENTION DEFICIT/HYPERACTIVITY DISORDER  Description: a persistent pattern of inattention and/or hyperactivity impulsivity that interferes ith functioning or development and nega tively impacts directly on social and academic/occupational activities  pproach to diagnosis: see able 

274

able 25

Clinical Process and iagnosis of Attention ecit Heractiit isorder

Ste

Considerations

chedle mltiple oce isits to complete the diagnostic ealation btain detailed inormation on prenatalperinatal eents, medical and mental health histor n preschool children, impaired attention and hperactiit ma also be eatres o a nerodeelopmental disorder

erorm thorogh phsical, nerological, and dsmorpholog assessments alate amil medical and mental health, amil nctioning and coping stles o primar caregiers alate or comorbid disorders pschiatric, nerodeelopmental and phsical

o comorbid smptoms meet criteria or a separate disorder that is the main diagnosis,  exist in tandem ith  as the main diagnosis,  are the secondar smptoms stemming rom the 

eie academic progress eg, report cards, sample assignments, look or smptoms o a learning disorder

linical impressions and se o standardied scales are the most eectie tools or diagnosis

btain standardied behaior rating scales that ealate M- criteria rom primar caregiers, teachers, and the adolescent being assessed ind screening tools and rating scales at https cpscaentools-otilscondition-specic-screeningtools-and-rating-scales

ating scales are not diagnostic o  bt help anti the degree to hich a behaior ma deiate rom the norm and can be sed to ealate the eects o interentions in home or school

Development

btain deelopmentalbehaioral histor motor, langage, social milestones, and behaior, inclding temperamentemotional reglation and attachment

12

nless indicated b histor and phsical examination, do  - rder laborator tests, genetic testing, , or neroimaging - rder pschological, neropschological, or speech-langage assessments - se pschological tests or measres o exectie nction to diagnose  andor as a means to ealate the eects o interentions Continued

25

able 25

Development 12

Clinical Process and iagnosis of Attention ecit Heractiit isorder—cont’d

Ste

Considerations

eer to M- criteria or core smptoms and characteristics  mptoms are seere, persistent ie, present beore 2 ears o age and contining . months, and inappropriate or the patient’s age and deelopmental leel 2 mptoms are associated ith impairment in academic achieement, peer and amil relations, and adaptie skills   there is a discrepanc o smptoms across settings, it is important to identi h the discrepanc exists  peci the tpe o  presentation as per the M- i ombined presentation criteria are met or inattention, hperactiit-implsiit ii redominantl inattentie presentation criteria are met or inattention iii redominantl hperactie-implsie presentation criteria are met or hperactiit-implsiit 5 peci crrent seerit mild, moderate, or seere based on the smptoms and degree o nctional impairment

onsider the demands and expectations being placed on the child and hat the child’s innate capabilities are to meet these expectations hat ill this child look like oer time

he abilities to sel-control attention, actiit and implses emerge in a deelopmental process he M- does not proide or deelopmental leel dierences, hich ma lead to oerdiagnosis o  in preschoolaged children mpairment implies greater seerit and reenc o smptoms that interere ith the abilit to nction across maor lie domains

ADHD, Attention deciteractiit disorder; DSM-V, Dianostic and tatistical anual of ental Disorders t dition; EEG, electroencealoram. Modied from Belaner SA, Andrews , et al. A in cildren and out: art 1—etiolo, dianosis, and comoridit. Paediatr Child Health. 1;:–.

 anagement a Children , years o age i arent behavior training ii oor evidence for psychostimulants in this age group b Children . years o age i onparmacological interventions psychoeducation parent behavior training classroom management daily report card behavioral peer interactions organiational skills training cognitive training exercise ii Parmacological interventions see able 

276

  

eserve medications for those diagnosed ith ADHD hose learning or academic performance are impaired by attention diculties or hose behaviors and social interactions are impaired by lack of impulse control and hyperactivity n addition to nonpharmacological interventions extended release stimulants are recommended as rstline therapy See hapter  ardiology for cardiac screening before stimulant use hen initiating pharmacological therapy use standardied checklists to monitor treatment response

PEARL  medications are controlled sbstances n ntario, prescriptions or controlled sbstances mst be prescribed as the total antit to be dispensed ells are permitted i the rell antit and rell date or interal beteen rells is inclded in the prescription onsider haing the interal as st less than the antit to be dispensed to gie amilies time to pick p the medication Example: italin 2 mg ake one tablet 2 mg b moth once dail otal antit 9 tablets ispense as  tablets eer 25 das

Development



12

2

12

Development

278

able 2 Medication

Pscostimulant and on-Pscostimulant Parmacological Treatment for AH Formulations

elier

uration of Actiona

Starting ose

ose Titrationc

Ametamine-ased Pscostimulants First Line tions dderall 

apsles 5, , 5, 2, 25,  mg

ranles can be sprinkled

2 h

25 mgkg or 5– mg am

h5– mg at eekl interals Usual dose range: – mgkg Maximum dose/day: hildren 5  mgkg or  mg dolescents and adlts 5 2– mg

anse

apsles , 2, , , 5,  mg

apsle content can be dilted in liid or sprinkled

– h

5 mgkg or 2– mg am

h–2 mg b clinical discretion at eekl interals Usual dose range: 8–5 mgkg Maximum dose/day: 2 mgkg or  mg

heable ablets , 2, , , 5,  mg

heable tablets shold be cheed thoroghl

Metlenidate-ased Pscostimulants First Line tions Biphentin

apsles , 5, 2, , , 5, , 8 mg

ranles can be sprinkled

–2 h

5 mgkg or –2 mg am

h5– mg at eekl interals Usual dose range: 8–5 mgkg Maximum dose/day: hildren and adolescents 5 2 mgkg or  mg dlts 5 8 mg

oncerta

xtended elease ablets 8, 2, , 5 mg

smotic-ontrolled elease ral elier stem 

2 h

5 mgkg or 8 mg am

h9–8 mg at eekl interals Usual dose range: 8–5 mgkg Maximum dose/day: hildren and adolescents 5 2 mgkg or 5 mg dlts 5 2 mg

oest

apsles 25, 5, 5, 55, , 85,  mg

ranles can be sprinkled

 h

25 mg am

h–5 mg in interals o no less than 5 das Maximum dose/day: hildren and adolescents 5  mg dlts 5  mg

p to 2 h

– ears and  kg 5 5 mgkg

Maintain dose or a minimm o – das beore adsting hildren and adolescents h b  mgkg interals dlts or  kg h b 2 mg interals Usual dose range: 5–2 mgkg Maximum dose/day: 2 mgkg or 8 mg

on-Pscostimulant–Selectie oreinerine Reutae niitor trattera tomoxetine

apsles , 8, 25, , , 8,  mg

apsle needs to be salloed hole to redce  side eects

dlts or  kg 5  mg dail on-Pscostimulant–Selectie Ala-2A Adrenergic Recetor Agonist ntni  anacine 

xtended elease ablets , 2, ,  mg

ills need to be salloed hole to keep delier mechanism intact

p to 2 h

 mgda morning or eening

Maintain dose or a minimm o  das beore adsting b no more than  mg increment eekl Usual dose range: 5–2 mgkg Maximum dose/day for monotherapy: –2 ears 5  mg – ears 5  mg Maximum dose/day as adjunctive therapy with stimulants: – ears 5  mg To discontinue: aper dose in decrements o  mg eer – das

a

Parmacokinetic and armacodnamic resonses ar from indiidual to indiidual. AA recommends startin wit te lowest dose aailale. c ose titration er roduct monora. or secic details on ow to start, adust and switc A medications, clinicians sould refer to te anadian A Practice uidelines www.caddra.ca. ildren refers to –1 ears of ae. Adolescents refers to 1–1 ears of ae or . k. ata from tts:www.caddra.cawcontentuloadsinalLaminateard1991.df and eldman et al. A in cildren and out: Part   treatment. Paediatr Child Health 1, :–. 

12

29

Development

SPECIFIC LEARNING DISORDERS  Description: see Box   ssessment: diagnosis reuires psychoeducational assessment Box   anagement:  ith childspecic accommodations and modications o 12

Development 12

Types and Diagnosis of Specic Learning Disorders

 iclt in at least one o the olloing areas, despite targeted help a naccrate or slo and eortl ord reading eg, reads single ords alod incorrectl or slol and hesitantl b iclt nderstanding the meaning o hat is read eg, ma read text accratel bt not nderstand the deeper meanings o hat is read c iclties ith spelling eg, ma add, omit, or sbstitte oels or consonants d iclties ith ritten expression eg, makes mltiple grammatical or pnctation errors ithin sentences e iclties mastering nmber sense, nmber acts, or calclation eg, has poor nderstanding o nmbers  iclties ith mathematical reasoning eg, has seere diclt appling mathematical concepts 2 cademic skills are sbstantiall belo hat is expected or the child’s age and impair eerda nction  Learning diclties begin dring school-age ears ma not ll maniest ntil later hen demands are greater  Learning diclties are cased b other conditions sch as intellectal disabilities, ncorrected isal or aditor acit, other mental or nerological disorders, pschosocial adersit, lack o procienc in the langage o academic instrction, or inadeate edcational instrction Modied from te Dianostic and tatistical anual of ental Disorders, t dition.

o 12  2  

Components of a Psycoeducational Assessment

Cognitie Assessment eg, eschler ntelligence cales or hildren, tanord-Binet, Leiter Academic Assessment eg, oodcock ohnson, ide ange chieement est Emotionaleaioral Assessment onners, hild Behaior hecklist Adatie Assessment eg, ineland daptie Behaior cale, daptie Behaior ssessment stem

SPEECH AND LANGUAGE DISORDERS  Description a anguage disorder: persistent diculties acuiring/using language limited vocabulary sentence structure discourse ith linguistic abilities belo hat is expected for age b Speech sound disorder: early onset persistent diculty ith speech sound production aecting intelligibility of communication 280 and interfering ith social academic or personal accomplishment

DEVELOPMENTAL COORDINATION DISORDER  Description: acuisition and execution of coordinated motor skills is substantially belo that expected for the patient’s age and opportunities for learning otor skills decit signicantly and persistently interferes ith activities of daily living appropriate to chronological age ¦e onset of symptoms is in the early developmental period  ssessment a Developmental coordination disorder uestionnaire b eferral to trained occupational therapist and/or physiotherapist for standardied assessment of motor abilities eg ovement Assessment Battery for hildren  anagement: individualied and taskoriented approach focusing on direct teaching of functional skills such as OO techniue typically delivered by occupational or physiotherapist

Development

c Child-onset uency disorder stuttering: sustained disturbance speech uency and time pattern repetitions prolongations or broken ords out of keeping ith age and language skills causing signicant distress d Social pragmatic communication disorder: persistent dicul ties in use of verbal/nonverbal communication for social interaction including using language in the appropriate context responding to verbal/nonverbal signals or understanding metaphors/multiple meanings  pidemiology: children ith speech/language disorders are at increased risk for later learning diculties  ssessment and management a arly introduction of reading and provision of a language rich environment b nvolve a speech language pathologist and/or psychologist to help ith diagnosis and ruling out alternative diagnoses eg D ASD hile providing therapy c Alternative or augmentative communication may be used sign language S or computeried devices

12

28

CHAPTER

13

Diagnostic Imaging Alisha Jamal • Jerey Traubici

Common abbreviations General principles Approach to imaging Radiographic appearance of lines and tubes

282

COMMON ABBREVIATIONS AP AXR C1 CT C2 CT C1MR CTA CXR ETT GA MRA MRCP NEC NG N PA PCC R R G  CG

anterior to posterior abdominal x-ray contrast-enhanced computed tomography non-enhanced computed tomography contrast-enhanced magnetic resonance imaging computed tomography angiography chest x-ray endotracheal tube general anesthesia magnetic resonance angiography magnetic resonance cholangiopancreatography necrotiing enterocolitis nasogastric nasoeunal posterior to anterior peripherally inserted central catheter right loer uadrant right upper uadrant upper gastrointestinal series ultrasonography oiding cystourethrogram

Diagnostic Imaging

Also see page xiii or a list o other abbreiations used throughout this boo

13

GENERAL PRINCIPLES  Imaging modalities a ee Table   Contrast agents a enition agents used to increase the contrast o so tissue structures and o uids to aid in dierentiating adacent structures as ell as characterie abnormal lesions in the body during medical imaging b ee Table  or description o arious contrast agents and their modes o administration

283

able 3

Imaging Modalities

Diagnostic Imaging

Modality

Advantages

Disadvantages

Contraindications

Plain lm x-ray

- Inexpensive, noninvasive - Readily available

- Radiation exposure - Poor at distinguishing soft tissues

Pregnancy



- xcellent delineation of bones and soft tissue - piral  fast data acuisition - ay allo 3 reconstruction -  angiography less invasive than conventional catheter angiography

- igh radiation exposure - edation may be needed - Relatively expensive - aution ith contrast in renal failureallergy - etal causes artifact

- Pregnancy - ontraindication to contrast agents

RI

- xcellent soft tissue resolution and discrimination - o ioniing radiation involved - R angiography provides noninvasive assessment of vessels and o

- laustrophobia - edation may be needed - etal causes artifact - ay be less readily available

- erromagnetic metal - oreign bodies - ardiac pacemaer



- Inexpensive, noninvasive - o ioniing radiation involved - etermines cystic vs solid - Real-time imaging useful for interventions

- ighly operator dependent - ir in boel may prevent imaging of midline abdominal structures

uclear medicine

unctional imaging data

- Radioactive substance inectedingestedinhaled - reuently needs I 6 sedation urine - ody uids radioactive

Pregnancy

P-

xcellent tumor detection

igh radiation dose 6 I sedation

Pregnancy

luoroscopy I

- Real time - Rarely reuires sedation

- Radiation involved - ladder catheteriation for 

Pregnancy

13

3D, Three-dimensional; CT, computed tomography; GA, general anesthesia; GI, gastrointestinal; GU, genitourinary; MRI, magnetic resonance imaging; PET, positron emission tomography; US, ultrasound; VCUG, voiding cystourethrogram.

284

Types and ses o Contrast in Imaging

Modality

Agent

Routes and Study Type

Contraindications

-ray

arium

PPR—routine I studies

Perforation, toxic megacolon

Iodinated contrast

PPR—suspected perforation in I studies, suspected boel obstruction, delineate stulasinus tract

- llergy consult radiology regarding alternative options consider premedication in select cases - naphylactoid-lie reactions have been reported in I imaging

ia urinary catheter— studies, , retrograde urethrogram 

Iodinated contrast

I—highlights vessels, enhances solid and hollo viscera, inammation

- Renal failure - llergy discussion ith radiologist regarding potential alternative modality or pretreatment

RI

adolinium

I—specic angiographic seuences, enhances solid and hollo viscera, inammation

Renal failure ris of nephrogenic systemic brosis—

Diagnostic Imaging

able 32

CT, Computed tomography; GI, gastrointestinal; GU, genitourinary; MRI, magnetic resonance imaging; VCUG, voiding cystourethrogram.

13

APPROACH TO IMAGING HEAD  X-ray skull: most common indications include assessing trauma orup o syndromes metabolic disorders and dysplasias abnormal sull shape craniosynostosis or ocal cranioacial lesion including occasionally metastatic disease  Ultrasound a Preterm babies and term neonates used to ealuate intraentricular hemorrhage  entricular enlargementmacrocephaly hite matter abnormalities ie perientricular leuomalacia and as an initial screening test or pathologies o the central nerous system b Transcranial oppler used to measure cerebral blood o noninasiely ie sicle cell disease  Computed tomography a C– CT typically the initial imaging modality or acute trauma and emergent situations to assess or sull ractures traumatic brain inuries hemorrhages or masses 28

Diagnostic Imaging 13

286

b C1 CT typically used or the ealuation o head and nec masses intracranial inection inammatory diseases c Computed tomography angiography CTA used to ealuate blood essel patency ie or pediatric stroe asculitis or ascular malormations  Magnetic resonance imaging a Most sensitie and specic noninasie test to proide detailed imaging o the brain oen used as the imaging modality o choice or CN pathologies bypassing CT b MRA used to ealuate blood essel patency similar to CTA CERICA SPIE  Also see Chapter  Emergency Medicine or C-spine trauma  Plain radiographs (anterior to posterior P cross tale lateral and hen obtainable open mouth odontoid ie are typically the initial imaging modality o choice to detect ractures or dislocations a Inury location: axial occiput to C cerical spine inuries are most common in children , years lder children tend to sustain subaxial C–C inuries b pproach to C-spine -ray interpretation: see igure  and Table  c Pseudosuluation o C on C can be dierentiated rom true subluxation by ealuating ischu’s line line dran rom the spinolaminar point o C to spinolaminar point C True subluxation should be suspected i this line misses the spinolaminar point o C by . mm ee igure  d dontoid ies: Examine dens or ractures and ensure lateral aspects o C are symmetric hae eual amounts o space on both sides o the dens lateral atlantodental interal and that lateral masses align e leionetension -rays are ery useul to detect ligamentous inury  C C-spine is the most sensitie modality to detect bony inuries a sed to conrm suspicious or abnormal C-spine x-rays or hen high suspicion or inury despite normal x-rays or instead o x-rays in cases o seere head trauma or acute neurological decits b C1 CT useul hen associated ascular inury suspected  MI spine is the most sensitie modality to detect inuries to so tissues o spinal column and spinal cord ndicated or patients ith neurological decit or ongoing symptoms despite negatie x-raysCT can hae signicant spinal cord inury TT radiological abnormalities

Diagnostic Imaging

igure 131 ormal ateral Ray o CSpine

5

4

3

2 1

Line 1 Preertebral so tissue line runs along the posterior border o airay through the rst

13

e ertebral bodies then idens around laryngeal cartilage and parallels the remaining cerical ertebrae Line 2 Anterior spinal line demarcates anterior border o cerical ertebral bodies Line 3 Posterior spinal line demarcates posterior border o cerical ertebral bodies Line 4 pinolaminar line connects unction o lamina and spinous process Line 5 pinous process line oins the tips o spinous processes ¦ese lines should run smooth and parallel ith no abrupt step-os

able 33

ACDS or Reading CSpine Rays

Alignment

oo for continuous lines ith smooth contour and no step-offs - nterior vertebral body spinal line - Posterior vertebral body spinal line - acet line - pinolaminar line - pinous process line

Bones

hips or fractures

Count

ust visualie all seven cervical vertebral bodies in entirety

Dis spaces

oo for consistent distance beteen each vertebral body

Soft tissue

oo for selling, especially prevertebral

28

igure 13 ateral Cervical Spine Ray it Siscu’s ine

Diagnostic Imaging 13

emonstration o pseudosubluxation physiological displacement o C on C A true subluxation should be considered i spinolaminar point o C is deiated . mm rom this line see text aboe

CHEST  Plain -rays are initial imaging study or nearly all suspected thoracic disease a tandard -ray ies: upright PA and le lateral supplemental ies may be obtained as needed obliue lordotic le or right lateral decubitus b asic CX interpretation: see Table  c X-ray nding o common pulmonary pathologies: see Table   U is the most sensitie modality or pleural eusions ie detecting pleural uid extent loculations septations and pleural thicening  C chest: used or assessment o complex pleural disease ascular disease congenital malormations complex chest inections trauma inammatory diseases interstitial lung diseases and or oncological staging  MI chest: used or detailed assessment o the mediastinum cardioascular system chest all and pleura ess useul or ealuation o the 288 lung parenchyma

Identication

ate, patient name, indications, techniue of examination

xposure

horacic dis spaces should be ust visible through heart

Rotation

- edial ends of clavicles should be euidistant from spinous process - nterior and posterior aspects of the ribs should appear symmetric

Inspiration

- Poor inspiration poor aeration, vascular croding, compressed and idened central shado - lder children six anterior and eight posterior ribs normally seen general rule - yperination lucent lungs, attened diaphragm, small heart

oft tissues

- oo for air in the soft tissues - elling, calcication, foreign bodies

bdomen

- oo for free air under diaphragm in upright R - isplacement of the gastric air bubble medially suggests splenomegaly

ones

hec cervical and thoracic spine, shoulder girdle, ribs, sternum

ediastinum

- rachea, heart, great vessels, thymus - oo for mediastinal or tracheal shift, idened mediastinum

ila

Pulmonary vessels, mainstem and segmental bronchi, lymphadenopathy

ungs

ung parenchyma, pleura, diaphragm

inestubes

Presence 1 positioning of , s, , feeding tubes

CVLs, Central venous lines; CXR, chest x-ray; ETT, endotracheal tube; UAC, umbilical artery catheter; UVC, umbilical vein catheter.

able 3

13

Patologic indings on Cest Ray

inding

Patology

Pulmonary opacity

ells or uid in the bronchoalveolar airspace results in a conuent density ie, pneumonia, mass, pulmonary edema, hemorrhage, atelectasis

ilhouette sign

pacity, by virtue of its presence, obscures a normal anatomic landmar Right middle lobe Right loer lobe ingula eft loer lobe

-

Diagnostic Imaging

asic Approac to Cest Ray

able 3

oss of right heart border oss of right hemidiaphragm oss of left heart border oss of left hemidiaphragm

ir bronchograms

Radiolucent branching bronchi visible through opacied airspace disease ie, pneumonia, edema, infarct, hemorrhage

Peribronchial cufng

Interstitial inltrate, edema, andor bronchial inammation ie, bronchiolitis, asthma

olume lossatelectasis

- Indicates collapse of a portion of the lung - ay see rib croding - Indirect signs hilarmediastinal shift toard collapse, 6 hemidiaphragm elevation - ften difcult to differentiate volume loss from other pulmonary opacity in children Continued 28

able 3

Diagnostic Imaging 13

290

Patologic indings on Cest Ray—cont’d

inding

Patology

Pleural effusion

lunting of costophrenic angle, uid in horiontal or obliue ssures an see meniscus in uncomplicated effusions

Pneumothorax

- ir enters pleural space ie the space beteen visceral and parietal pleura, separating partially collapsed lung from the chest all - ediastinal shift aay from the pneumothorax and diaphragmatic inversion indicate tension pneumothorax

ADME  Plain -rays are initial imaging modality o choice or many abdominal conditions especially NEC boel obstruction boel peroration radioopaue renal tractbiliary tract calculi and oreign bodies a tandard -ray ies: AP supine PA erect others include lateral decubitus dorsal decubitus PA prone b asic X interpretation: see Table  and igure 

able 3

asic Approac to Adominal Ray

Identication

ate, patient name, indications, techniue of examination

xposure

hole abdomen should be visible from diaphragm to pelvis

tomach, small and large boel

- tomach seen left of the midline, belo hemidiaphragm ith variable amount of air - mall boel - sually more central ith the large boel creating a “frame” around the periphery - ucosal folds are seen across the full idth of the small boel valvulae conniventes - arge boel - he ascending and descending colon ill be in xed positions laterally and posteriorly in the retroperitoneum transverse and sigmoid positions can vary each on a “mesocolon” - oo for haustra folds that protrude only part ay through the lumen and the spaces beteen the haustra - oo for the presence of gas to the level of the rectum and for signs of obstruction ie, airuid levels, dilated loops of boel, transition one, as ell as intramural gas and intraperitoneal gas

ther organs

- ungs–chec lung bases for pathology ie, consolidation as a source of abdominal pain - iver seen in R - allbladder often not seen, but can see calcied gallstones - idneys often visible if sufcient perirenal fat, the right ill be loer than left because of liver - pleen seen in 

able 3

asic Approac to Adominal Ray—cont’d

ones

hec ribs, lumbar vertebrae, sacrum, coccyx, pelvis, proximal femurs

alcicationsartifacts

oo for renal, ureteric, bladder stonescalcications, gallstones R

inestubes

Presence and positioning of  tubes,  tubes, -tubes,  tubes, femoral lines, etc

igure 133 Posteroanterior Adominal Ray

Diaphragm Splenic flexure

Liver

Small intestine

Diagnostic Imaging

AXR, Abdominal x-ray; GI, gastrointestinal; GJ, gastro-jejunal; LUQ, left upper quadrant; NG, nasogastric; NJ, nasojejunal; RUQ, right upper quadrant.

Transverse colon

Descending colon

13

L5 vertebrae Iliac crest

Rectum

 U: generally used as a rst-line screening test or ealuation o lier spleen boel intussusception appendicitis etc biliary tract and gallbladder and renal pathologies  C adomen: used sparingly because o radiation ris but aluable or oncologic orup abdominal trauma and surgical planning  MI adomen: useul or detailed ealuation o solid organ pathology intra- and extraluminal boel pathology oncologic orup  Magnetic resonance cholangiopancreatography: used as a nonin2 asie techniue to assess the biliary tree and pancreatic duct system

Diagnostic Imaging

ie choledocholithiasis congenital anomalies o the bile and pancreatic ducts choledochal cysts primary sclerosing cholangitis  Meckel scan Tc-pertechnetate scintigraphy generally used as a rstline screening test or a Mecel dierticulum ¦e radiopharmaceutical has an a©nity or gastric mucosa and can be used to identiy ectopic gastric mucosa  Upper I (UI 6 small oel ollo-through: oral contrast administered under uoroscopic isualiation arious indications including orup o abdominal pain omiting bleeding ailure to thrie n neonates oen used in the orup o congenital obstructionmalrotationmidgut olulus  arium enema: contrast administered per rectum ia a rectal catheter under uoroscopic isualiation arious indications include orup o chronic constipation irschsprung disease or bleeding n neonates oen used in the orup o congenital obstruction ie distal boel atresia irschsprung disease meconium ileus MSCSEETA SSTEM

 Also see Chapter  rthopedics or urther description o ractures hip disorders and bone and oint inections  Plain -rays are initial imaging modality o choice or most musculoseletal complaints trauma inection congenital abnormalities and malignancy 13 a tandard -ray ies or ractures AP and lateral x-ray obliue ies may be helpul in select cases b one age: obtain single P -ray o le hand and rist radiologist then compares x-ray ith images in standard atlas o bone deelopment c keletal surey:  x-ray ies sull spine chest abdomen extremities Mainly perormed in seletal dysplasias metabolic disorders histiocytosis and child abuse see Chapter  Child Maltreatment  C scan a seul to ealuate certain ractures eg pelic and select anle ractures or treatment planning b seul or detailed ealuation o congenital bone malormations tumors oreign bodies and osteochondral lesions c en used hen MR is unaailable  MI a Most sensitie and specic noninasie test or ealuation o bone cartilage so tissue and ligamentous changes b Most useul modality to ealuate extent o bony tumors and metastases c est modality to diagnose osteoarticular inections such as osteomyelitis septic arthritis x-ray relatiely insensitie in the early stages o 292 osteomyelitis

d est modality to assess or muscle pathologies and inammatory conditions ie dermatomyositis e est modality to assess marro disorders EITRIAR TRACT ee Chapter  Nephrology and rology

ee Table  able 3

Appropriate Positioning o ines and Tues on Ray

ineTue

Imaging Study

Appropriate Position



R P and lateral

iday beteen thoracic inlet and carina

hest tube

R P and lateral

- Pneumothorax anterior and apical - Pleural ffusion posterior and inferior to the uid to enable drainage

PI

R P 6 lateral

- rm PI end should be in the  –, avoid right atrium R unction at  or interspace abovebelo in 3 of patients - eg PI in I, – above renal veins , 2, avoid right atrium



hestabdominal P 6 lateral

- igh – preferred, o 3– - void maor branches of abdominal aorta celiac axis to inferior mesenteric artery



hestabdominal lateral 6 P

- hould be beteen the level of the diaphragm and the IR unction, lateral radiograph very useful for localiing tip



hestabdominal P 6 lateral

- hould overlay the gastric bubble

Diagnostic Imaging

RADIOGRAPHIC APPEARANCE OF LINES AND TUBES

13

AP, Anteroposterior; CXR, chest x-ray; ETT, endotracheal tube; IVC, inferior vena cava; NG, nasogastric; PICC, peripherally inserted central catheter; RA, right atrium; SVC, superior vena cava; UAC, umbilical artery catheter; UVC, umbilical vein catheter.

FURTHER READING Coley  Caey’s Pediatric Diagnostic Imaging. 13th ed Canada Elseier Canada 

USEFUL WEBSITE Resource or additional inormation and radiographic examples httpsradiopaediaorg

23

CHAPTER

14

Endocrinology Joju Sowemimo • Julia Sorbara • Jonathan D. Wasserman

Common abbreviations Diabetes mellitus Hypoglycemia Thyroid disorders Calcium disorders Puberty Disorders of sex development Adrenal disorders Short stature Disorders of water balance

294

COMMON ABBREVIATIONS 17-OHP ACTH ADH AI ATD  CAH CHO DDAP DHA DI DA DD D H AD  H nH HA1c hC IAI iCa I AI H  OTT PCO AI T1D TD TDD TPO TH TI

17-hydroxyprogesterone adrenocorticotropic hormone antidiuretic hormone adrenal insuciency antithyroid drugs lood glucose congenital adrenal hyperplasia carohydrate desmopressin 1-deamino-8-D-arginine vasopressin dehydroepiandrosterone sulate diaetes insipidus diaetic etoacidosis disorders o sex deelopment emergency department ollicle-stimulating hormone glutamic acid decaroxylase glomerular ltration rate groth hormone gonadotropin-releasing hormone hemogloin A1c human chorionic gonadotropin intermediate-acting insulin ionied calcium intrauterine groth restriction long-acting insulin luteiniing hormone multiple endocrine neoplasia oral glucose tolerance test polycystic oarian syndrome rapid-acting insulin type 1 diaetes mellitus type  diaetes mellitus total daily dose o insulin thyroid peroxidase thyroid-stimulating hormone thyroid-stimulating immunogloulin TH receptorstimulating antiodies

Endocrinology

Also see page xiii or a list o other areiations used throughout this oo

14

295

DIABETES MELLITUS 1 Diagnosis: see ox 11 Box 14.1

Diagnostic Criteria for Diabetes

Fasting BG 7.0 mmol/L (i.e., no caloric intake for 8 h OR BG . mmol/L 2 h folloing G (75 g oral carohrate OR anom BG . mmol/L BG, Blood glucose; OGTT, oral glucose tolerance test.

Endocrinology 14

296

TYPE 1 DIABETES MELLITUS 1 Denition: hyperglycemia resulting rom asolute or relatie insulin deciency  Presentation a Polyuria polydipsia polyphagia eight loss  Adominal pain nausea omiting c Asymptomatic incidental nding d ay present in diaetic etoacidosis DA  Onset: peas in early childhood – years and around puerty 1–1 years  Diagnosis a ymptomatic random hyperglycemia 111 mmol diagnostic no conrmatory testing reuired  Asymptomatic perorm second conrmatory test on a dierent day c I high suspicion conrmatory testing should not delay treatment d HA1c not useul or diagnosis—may e normal in early Type 1 diaetes mellitus T1D  Investigations a Blood: hyperglycemia see ox 11 6 etonemia  Urine: glucosuria 6 etonuria c Electrolyte abnormalities: may hae hyponatremia hypoalemia d Additional tests: not reuired to estalish diagnosis ut may e helpul i etiology unclear i oundetectale asting insulin and C-peptide ii Positie pancreatic b-cell autoantiodies anti-AD anti-insulin islet cell antiodies A. Management o ye 1 diabetes mellits itot DA 1 Monitor blood glcose B: eore meals and eore ed a Additional checs i symptoms o hypoglycemia see later  Chec oernight i concern or nocturnal hypoglycemia

ale 4.

Cararisis o Insulin Praraions

Insulin (Trad a in Parnss

Ons

Pa

Duraion

UlraRaidAin sart (Fias

4 min

0–90 min

–5 h

RaidAin (RAI Lisro (malog sart (oolog, oorai Gllisine (ira nslin reglar (mlin 

0–5 min 0–5 min 0–5 min 0 min

–2 h –2 h –2 h 2– h

–5 h –5 h –5 h .5 h

InrdiaAin (IAI sohane (mlin , oolin

– h

5–8 h

 to 8 h

LonAin (LAI Glargine (Lants, Basaglar, oeo etemir (Leemir eglec (resia

90 min 90 min 90 min

o eak o eak o eak

 to 24 h –24 h 42 h

 Mltile daily inection basalbols regimen a ie hal TDD as AI asal insulin administered once daily and hal as AI olus insulin diided eually among three meals g  g child TDD  unitsgday 5  units Hal as asal insulin 5 1 units gien as AI h Hal as olus insulin 5 1 units diided y  daily meals 5  units gien as AI eore each meal may distriute dierently relatie to sies o meals eg  units at reaast  at lunch  at dinner  BID or ID regimens a sed i a child cannot administer their on insulin and no caregier is aailale to administer pre-lunch insulin at school  ie to-thirds TDD in morning one-third in eening

Endocrinology

c ay use continuous or ¡ash glucose monitors to augment capillary  monitoring d arget B i  to 1 mmol in initial education period andor younger patients ii  to  mmol or estalished patients  Inslin initiation a Initial total daily dose TDD  to  unitsgday  Anticipate decreasing reuirements during rst year a£er diagnosis ecause o “honeymoon period” c Inslin rearations: see Tale 11

14

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Endocrinology 14

298

c Diide each dose to-thirds IAI one-third AI g  g child TDD  unitsgday 5 1 units orning dose ⅔ o 1 5 1 units ⅔ 7 units as IAI eore reaast ⅓  units as AI eore reaast ening dose ⅓ o 1 5  units ⅔  units as IAI eore dinner toddler or eore ed schoolage child ⅓ 1 units as AI eore dinner  ontinos sbctaneos inslin insion inslin m a Deliers AI as continuous inusion asal insulin ith oluses eore each meal  olus calculated using carohydrate CHO ratio and insulin sensitiity actor c DA eoles uicly i insulin deliery interrupted  ongterm management: education and multidisciplinary team inolement essential a Treatment can egin as outpatient proided patient is medically stale and there are no arriers prohiiting ollo-up B. yoglycemia in ye 1 diabetes mellits 1 amilies should receie hypoglycemia education and eep glucagon its at home  Presentation a ild seating eaness tachycardia tremors eelings o nerousness hunger  eere irritaility conusion lethargy anormal ehaior seiure otundation c ay e asymptomatic hypoglycemia unaareness  Management o mild yoglycemia a Treat  , mmol een i asymptomatic  Oral treatment preerred in an alert child ho is otherise ell 1 to 1 g CHO eg 1 m uice – dextrose talets  m mil or  teaspoons o sugar c echec  in 1 to 1 min i , mmol repeat 1 to 1 g CHO d ay reuire mini-dose glucagon i unale to tolerate ¡uids see later  Management o severe yoglycemia a ie glucagon immediately sucutaneous C or intramuscular I  Aoid oral CHO until the child is aae and alert c Continue monitoring  or seeral hours a£erard glucagon hal-lie –1 minutes

 ED management: igure 11 reer i a Persistent hypoglycemia despite treatment  uspected prolonged hypoglycemia AI oerdose c Ongoing hypoglycemia and not tolerating oral therapy

History Recent hypoglycemic event requiring treatment with oral CHO or SC/IM glucagon

and/or

Clinical signs • Altered level of concioune • Seiure • ocaliing neurological ign

Investigations • Capillary  • lectrolyte and lood ga uually not neceary

Tolerating oral fluids

ncourage CHOcontaining drin at leat at hourly maintenance fluid rate

Observation and monitoring • ry to determine caue of hypoglycemia • ecreae all inulin doe y  for the net  hour • Arrange followup • rovide glucagon precription if ued

Yes

lert

Maintenance I  detroe/ aCl regardle of 

Endocrinology

iur 14.1 Ern Darn Manan o Holia in T 1 Dias Mllius

No

olu I  detroe  m/g followed y maintenance  detroe/ aCl

14

Continue maintenance IV dextrose until • eurological eam normalie • o longer retle confued drowy or irritale hen change to CHOcontaining oral fluid May tae up to  hour if hypoglycemic encephalopathy i preent Aim to maintain   mmol/ throughout

Discharge when child is • ully alert • olerating oral fluid • At neurological aeline

BG, lood glucose CHO, carohydrate IM, intramuscular IV, intraenous NaCl, sodium chloride SC, sucutaneous

 emind amilies to carry glucagon en route to D 7 ecurring hypoglycemia may arrant insulin dose adustment y diaetes team 299

. Management o intercrrent illness in 1DM 1  may e high or lo in the setting o illness  Chec  and urine etones h including oernight  nsure ¡uid and calorie intae ideally 1 g CHO per hour can e proided as ¡uid  Insulin doses may reuire adustment Tale 1 ut should not e omitted

Endocrinology

ale 4.2 B (olL heck 4 orl

Insulin Adusn or Illnss ,

–4

.4

.4

Urin ons

egatie or ositie

egatie or ositie

egatie or small (1

oerate or large (11

Aion

ecrease   20 ntil BG –

ontine to monitor, gie inslin as sal

Gie inslin as sal ls 0  as 

Gie inslin as sal ls 20  as 

BG, Blood glucose; RAI, rapid-acting insulin; TDD, total daily dose.

14

 Consider mini-dose glucagon or mild or recurrent hypoglycemia  , mmol a , years old  units  mcg   years 1 unityear o age eg -years-old 5  units to maximum 1 units 1 mcg c Doule dose i initial administration does not result in  . mmol  ED management: igure 1 reer i a  omits ithin  to  hours  eusing oral intae c ultiple doses o glucagon reuired

300

iur 14. Ern Darn Manan o Inrurrn Illnss in T 1 Dias Mllius Initial assessment istory, physical eamination, and inestigations

*Investigations • apillary glucose • erum glucose • enous lood gas • lectrolytes, creatinine • rinary ketones • urther orkup as directed y clinical indings

see panel on right Yes

omiting No

I emesis ≥2 in 4 hours, keep  or 4– hours

olerating oral luids

Yes

No IV fluids • I seere dehydration 0 al 10 mL/kg olus oer 1 hour • B 20 mmol/L 0 al maintenance • B 20 mmol/L  detrose/0 al maintenance • dd l once oiding

ncourage containing luids

Hyperglycemia • o not omit insulin • se  insulin unless acidotic see igure 14 • I B 11 mmol/L and ketonuria gie usual insulin  I q4 hourly at 10–20 

Monitoring • Intake and output • Blood glucose q2–4 hourly, aim 4–10 mmol/L • rine ketones q4 hourly

Hypoglycemia • o not omit insulin • ecrease  insulin dose y 10–20 • I not tolerating  manage as per igure 141 • I tolerating  encourage containing luids

Criteria for discharge • olerating oral luids • o other indication or hospitaliation • eplace usual meals ith containing luids

BG, lood glucose CHO, carohydrate Cl, potassium chloride NaCl, sodium chloride NO, nil per os nil y mouth O, per os y mouth I, rapid-acting insulin SC, sucutaneous DD, total daily dose

14

0

Endocrinology

Endocrinology

D. Managing yerglycemia on inslin m teray 1 Administer inslin bols via m: rechec a£er  hours lac o response suggests insulin pump malunction  ec m: empty reseroir expired insulin error messages dead attery contact manuacturer or replacement i necessary  ec tbing: leas craced tuing air ules change inusion set reprime  ec m site: leaage dislodged catheter cellulitis change site  onvert to inslin inections: until pump is unctioning relialy a I amily has IAI gie ⅔–¾ total daily asal insulin as single IAI dose at edtime  I amily has AI gie total daily asal insulin as single AI dose c Total daily asal insulin 5 aerage asal pump rate 3  hours determine y interrogating pump settings d ie AI ith meals and to correct hyperglycemia according to ratios set in pump  Once pump issue resoled restart asal rate 1 to  hours a£er AI PEARL ssme herglcemia ith ketonria reects m failre ntil roen otherise

14

E. Perioerative management o ye 1 diabetes mellits 1 chedule as rst case o the day to minimie duration o asting  onitor  closely eore and during procedure a yerglycemia: correct ith insulin especially i associated etosis  yoglycemia: correct ith oral ¡uids during indo or clear ¡uids  I id selection a  dextrose aCl i  in target range o  to 1 mmol  1 dextrose aCl i concerns aout hypoglycemia c  aCl i  .1 mmol d Choice o I ¡uid may reuire adustment intraoperatiely ased on   Insulin adustments or surgery are ased on the patient’s insulin regimen specic recommendations should e proided y the patient’s primary diaetes team or electie surgeries

DIABETIC ETOACIDOSIS 1 Denition: hyperglycemia  .11 mmol detectale etones urinelood acidosis  lassication a Mild: pH ,7 or HCO ,1 mmol  Moderate: pH ,7 or HCO ,1 mmol 302 c evere: pH ,71 or HCO , mmol

 Presentation and management: see igure 1 iur 14. Manan o Pdiari Diai oaidosis (DA

kg

Endocrinology

10–15 L/min via non-rebreather mask (if signs of shock)

lf hoerfuse (tachcaria ca refill  sec cool etremities) give 10 mL/kg 0 NaCl bolus over 0 minutes reassess after bolus an reeat  1 if ersistent hoerfusion iscuss further flui management ith eiatric eferral Centre

0 NaCl

14

Repeat 10 mL/kg 0.9% NaCl bolus over 30 min. Reassess after eac bolus an repeat if persistent poperfusion. iscuss it eiatric Referral Centre.

ehrate ith  0 NaCl Change to 10 etrose/0 NaCl hen glucose is 15mmol/L OR glucose is 5 mmol/L an ecreases b 5 mmol/L/hr

Neurovitals (GC uils)  an 

BCs, airay reathing circulation B, lood pressure C, computeried tomography CG, electrocardiogram D, emergency department GCS, lasgo coma scale H, heart rate IV, intraenous Cl, potassium chloride NaCl, sodium chloride , respiratory rate odied ith permission rom Translating mergency noledge or ids

 Usel calclations a ectie osmolarity 5  3 a mmol 1 glucose mmol  Corrected sodium 5 measured a 1  3 glucose –  TYPE  DIABETES MELLITUS 1 Denition: hyperglycemia see ox 11 associated ith insulin resis0 tance and relatie impairment in insulin secretion

 Presentation a Polyuria polydipsia  Incidental nding asymptomatic hyperglycemia c Occasional clinical oerlap ith T1D distinguished y presence o oesity signssymptoms o insulin resistance ox 1 and strong amily history d ay present ith DA less liely than T1D or hyperosmolar hyperglycemic state HH

Endocrinology

Box 14.

Risk Factors for Type 2 Diabetes Mellitus

esit (B 95th ercentile for age an gener ighrisk ethnic gro (original, frican, sian, isanic, acic slaner, or oth sian escent Famil histor of e 2 iaetes mellits an/or in utero eosre to herglcemia linical eience of inslin resistance  canthosis nigricans  ertension  sliiemia  onalcoholic fatt lier isease (L .3 er limit of normal, or fatt lier on ltrason  olcstic oarian snrome ALT, lanine aminotransferase BMI, o mass ine.

14

304

 Investigations: as in T1D a Additional inestigations useul to dierentiate rom T1D high asting insulin high C-peptide leels asence o pancreatic autoantiodies  DM screening: Diaetes Canada recommends screening  yearly using asting  in children ith any o a  ris actors in pre-puertal children or  in puertal children see ox 1  Impaired asting glucose  1– mmol or impaired glucose tolerance  7–11 mmol  hours post-oral glucose tolerance test OTT c se o atypical antipsychotic medications  Management a onarmacological: eight reduction dietary interention increased physical actiity to improe insulin sensitiity  Metormin: decreases hepatic glucose production increases insulin sensitiity i Alternatie oral antihyperglycemic agents not yet approed or use in children c  inslin: as in T1D particularly i HA1c . d Mltidiscilinary aroac: as in T1D

OTHER ORMS O DIABETES A. Monogenic diabetes 1 Preiously non as maturity-onset diaetes o the young OD  are autosomal dominant deect in pancreatic eta-cell genes  lassic caracteristics: , years o age at diagnosis aerage eight asence o eatures o insulin resistance asent b-cell autoantiodies o£en multi-generational amily history  reatment: depends on specic gene aected a ome orms reuire no treatment others reuire liestyle and diet modications as ell as sulonylureas or insulin B. Medicationindced yerglycemia 1 any medications impair glucose tolerance y a Decreasing insulin secretion  Increasing hepatic glucose production c Promoting insulin resistance d xerting cytotoxic eects on pancreatic b-cells e nhancing counter-regulatory responses  ommon cases: corticosteroids immunosuppressants atypical antipsychotics  reatment: monitoring alone ersus C or I insulin depending on degree o hyperglycemia and anticipated duration o medication use a Adust insulin dose as doses o hyperglycemia-inducing medications are altered

Endocrinology

A. yerosmolar yerglycemic state 1 Denition: sustantially eleated  . mmol and hyperosmolarity . mOsmg o£en ithout signicant etosis or acidosis a etaolic emergency in the context o seere insulin resistance o£en reuires IC-leel care  Presentation a Polyuria polydipsia seere dehydration  Altered leel o consciousness seiures c Clinical oerlap ith DA usually ithout hyperentilation omiting or adominal pain  Management a Aggressie ¡uid resuscitation  Correct electrolyte anormalities reuent electrolyte monitoring c Insulin administration once  no longer improing ith ¡uids alone  omlications: rhadomyolysis renal ailure deteriorating mental status entricular arrhythmias thromosis malignant hyperthermialie picture rising C and eer death

14

05

HYPOGLYCEMIA 1 eonatal yoglycemia: see Chapter 7 eonatology  onneonatal yoglycemia: see Chapter  etaolic Disease PEARL etermining the resence or asence of ketones is central to etermining the etiolog of ho glcemia. rine ketones ma remain etectale seeral hors after hoglcemia, ths a rine samle is alale, een if a serm samle cannot e otaine (see etails of critical samles reire for BG ,2.7 oglcemia section, hater 2 etaolic isease.

Endocrinology

THYROID DISORDERS

COEITAL HYPOTHYROIDISM 1 ay e permanent or transient depending on etiology  Asymptomatic at irth ecause o transplacental passage o maternal T  I ntreated may lead to a eurodeelopmental delay  Prolonged neonatal aundice c eeding diculties constipation adominal distension umilical hernia d acroglossia ide anterior ontanelle 14 e Hypotonia hypothermia lethargy apneic episodes  eborn screening B: allos identication eore symptoms deelop a All anormal results reuire conrmatory serum thyroid unction tests TH ree T  B  .4 mIU: reuire prompt physician assessment initiate treatment ithout delay i conrmatory testing remains anormal c B  1–4 mIU: perorm conrmatory las suseuent management determined y results   programs using TH alone may miss congenital central hypothyroidism a end TH ree T i clinical suspicion or hypothyroidism despite normal   rter investigations: TH ree T 6 thyroid radionuclide scan ideally eore or ithin 1 ee o starting treatment 7 reatment: commence thyroxine replacement promptly once diagnosis conrmed—do not delay to accommodate imaging a evotyroine: once daily as talet Tale 1  Dose adstment: i TH . mI or , mI c Cognitie unction unliely to e impacted i treatment initiated y 306 1 days o age

ale 4.

Dos (da

0– months

0–5

–2 months

–0

–5 ears

5–7

–2 ears

–5

.2 ears

2–

lt

.7

 Monitoring: ree T TH and groth a To ees a£er starting treatment  At     1 months o age c ery  months rom 1 to  years o age d early a£er  years e Chec TH  to  ees a£er any dose adustment

Endocrinology

A

Loroxin Sarin Dos Rondaions

PEARL tale ssensions of leothroine o not eist. o not rescrie lii—crsh talets an mi ith reastmilk/formla instea.

ACUIRED HYPOTHYROIDISM 1 Primary yotyroidism a Hashimoto lymphocytic thyroiditis most common cause in orth American youth i emalemale ratio 1 ii High ris ith trisomy 1 Turner Dieorge and illiams syndromes other autoimmune diseases eg T1D celiac disease rst-degree relaties ith autoimmune thyroiditis  Iodine deciency most common cause orldide c Iodine excess d edications eg lithium amiodarone antiepileptic drugs tyrosine inase inhiitors e ³yroid inury eg olloing therapeutic radiation  entral yotyroidism a Hypothalamicpituitary tumors  Congenital ariant eg septo-optic dysplasia c Inltratie processes eg angerhans cell histiocytosis d Central nerous system C traumasurgery e euelae o craniospinal radiation

14

07

 Presentation: see ox 1 Box 14.

Clinical Manifestations of Hypothyroidism

Letharg, somnolence onstiation Linear groth failre elae or recocios ert ol intolerance cool, r etremities

Endocrinology 14

iffse goiter ins racaria roimal mscle eakness elae relaation of ee tenon reees r skin, hair, meema

 Investigations a : sole recommended screening test or primary hypothyroidism  ree 4: reuest i TH outside reerence range congenital hypothyroidism or suspicion or central hypothyroidism c AntiPO antibodies: i suspecting autoimmune thyroid disease d MI brain: i ne central hypothyroidism in child ithout non ris actors e yroid U: not routine consider only i thyroid asymmetry on exam cerical lymphadenopathy or palpale nodule  Diagnosis a Primary yotyroidism: h TH ith g ree T  bclinical yotyroidism: h TH ith normal ree T i epeat mildly h TH alues –1 mI a£er at least  ees as spontaneous normaliation is common ii Typically does not merit treatment unless clearly attriutale symptoms c entral yotyroidism g or inappropriately normal TH g ree T or progressiely declining ree T olloing C insult  reatment: leothyroxine see Tale 1 a Initiate hen TH .1 mI and lo ree T consider earlier ased on ree T leel and presence o attriutale symptoms 7 oals o treatment a Primary yotyroidism: normalie TH  entral yotyroidism: ree T ithin upper hal o reerence range not useul to ollo TH

THYROTOICOSIS 1 Etiology a raes disease most common cause  Hashitoxicosis Hashimoto thyroiditis ith early thyrotoxic phase c uacute thyroiditis usually iral d uppuratie thyroiditis acterial or iral e Toxic adenoma autonomously unctioning solitary nodule  Amiodarone-induced g Toxic multinodular goiter rare in children 308  Presentation: see ox 1

Clinical Manifestations of Hyperthyroidism

niet alitations ertension Fatige, eakness eating, heat intolerance remor

nsomnia ncrease aetite Freent stools eight loss ohthalmos Goiter

 Investigations a g TH h ree T  T not part o initial orup consider i ree T normal in the setting o g TH c ³yrotropin receptor antiody TA or thyroid stimulating antiody TI titers d adioactie iodine uptae and scan consider i TATI not eleated or palpale nodule present i High diuse uptae raes ii ocalied uptae toxic nodule iii oasent uptae Hashitoxicosis or other orms o thyroiditis  Medical management a Metimaole: rst-line anti-thyroid drug ATD adust initial dose ased on ree T or T leels  Proyltioracil: may e useul in thyroid storm lac ox arning in children ecause o ris o ulminant hepatic ailure c Proranolol: i signicant symptoms particularly at onset o ATD therapy d emission rate: up to  a£er  years ith medical management loer than adults  Monitoring a eore starting ATDs CC AT  ide eects o oth ATDs idiosyncratic include agranulocytosis lupus-lie syndrome aundice rashes c Chec CC i patient is erile or deelops pharyngitisstomatitis hile on ATDs

Endocrinology

Box 14.4

14

RAES DISEASE 1 Atoimmne atogenesis: TIs ind to TH-receptors leading to autonomous production o thyroid hormone associated ith other orms o autoimmunity  Presentation: see ox 1 a Associated ophthalmopathy in ⅓ children usually less seere than in adults ithout ision loss improes hen remission achieed 09  igest incidence: adolescent emales

Endocrinology

 Medical management: as earlier a I lo-dose methimaole is eectiely controlling thyroid unction ithout side eects prolonged ATD therapy may e considered  Denitive management a 131I (radioactive iodine) therapy: remission may tae ees months ongoing ATD o£en reuired until hypothyroidism achieed i Aoid ,1 years o age or in pregnancy  yroidectomy: immediate control o hyperthyroidism i urgical riss hypocalcemia ecause o hypoparathyroidism recurrent laryngeal nere palsy

CALCIUM DISORDERS HYPOCALCEMIA 1 ormal calcim range: see Chapter  aoratory eerence alues  elevant istory: see Tale 1 ale 4.4

14

310

Rlan Hisor in Hoalia

aurs ro Hisor

Dianosis o Considr

ongenital anomalies eelomental ela

iGeorge (22.2 eletion snrome

eck srger/trama

rgical/tramatic hoarathroiism

ietar limitations, ecrease snlight eosre

itamin  ecienc

Gastrointestinal, heatoiliar, or ancreatic isease

ecrease calcim or itamin  asortion

enal isease

ncrease calcim ecretion

ositie famil histor

ocalcemic genetic isorers

 Presentation a Tetany muscle cramps carpopedal spasm Trousseau sign acial titch Choste sign acral paresthesias numness seiures papilledema  aryngeal stridor c C anormalities eg prolonged T idened T-aes radycardia d Psychiatric maniestations  Dierential diagnosis: see Hypocalcemia in Chapter 7 eonatology neonates and ox 1 inants and children

oarathroiism  Genetic, e.g., late resentation of iGeorge snrome  toimmne olglanlar snrome te   nr to arathroi glans ecase of srger, iron eosition, raiation  ioathic efect in calcimsensing recetor (atosomal ominant seohoarathroiism, i.e., enorgan resistance to arathroi hormone itamin  ecienc, resistance or imaire snthesis erhoshatemia, e.g., tmor lsis snrome omagnesemia rgs, e.g., ishoshonates, some chemotheraetic rgs, calcimimetics ancreatitis Modied from Sperling M. Pediatric Endocrinology. 4th ed. Philadelphia, PA: B Saunders; 4.

 Investigations a Ca alumin iCa PO g AP PTH -OH-itamin D spot urine calciumcreatinine ratio renal unction  1-OH-itamin D should not e perormed during initial orup  Diagnosis: see Tale 1

ale 4.5

Endocrinology

Box 14. Differential Diagnosis of Hypocalcemia in Infants and Children

14

Laoraor indins in Hoalia

Dianosis

Ca

PO4

(OHD

1(OHD

PTH

ALP

itamin  ecienc

g

 or g

g

 or h

h

hhh

oarathroiism

g

h



g

ga

 or h

seohoarathroiism

g

h



g

h

h

a

r inappropriately normal in the setting of hypocalcemia

ALP, Alaline phosphatase; Ca, calcium; N, normal; PO4, phosphate; PTH, parathyroid hormone. ata from Alario A. Practical Guide to the Care of the Pediatric Patient. St. ouis, M: Mosy; .

7 Management a Oral calcim slementation: calcium caronate i ectie monotherapy in mild hypocalcemia ii ay reuire doses exceeding recommended daily intae  olecalcierol: -OH-itamin D i Treat itamin D deciency een i not thought to e the cause o hypocalcemia ii ay reuire I ormulation i concern or at malasorption



Endocrinology

c Alacalcidol 1a-OH-itamin D or alcitriol 1-OH itamin D actie orm o itamin D i euired in conditions associated ith deects in itamin D actiation eg renal disease hypoparathyroidism ii O£en used in settings o seere hypocalcemia een i deectie itamin D actiation not suspected d alcim insion: or seere andor symptomatic hypocalcemia eg seiures arrhythmia or inaility to use oral treatment i Central enous access preerred due to ris o seere extraasation inuries eg tissue necrosis urns enous thromosis loer ris ith calcium gluconate than calcium chloride ii eer gie I calcium olus ecause o ris o reound hypocalcemia iii onitor leels h until stale aim or total Ca . mmol and or iCa .1 mmol ean as tolerated repeat loodor  to  hours a£er any change in rate i During inusion monitor cardiac rhythm I site  Incompatile solutions sodium icaronate and ce£riaxone neer inuse ce£riaxone ith calcium een in dierent I lines ecause o ris o potentially atal calcium-ce£riaxone precipitates in lungs and idneys

14

PEARL lasma calcim concentration falls  0.2 mmol/L for eer 0 g/L fall in lasma almin— measre ia if serm rotein or  is anormal.

HYPERCALCEMIA 1 Denition: total calcium aoe upper limit o reerence range or age once corrected or alumin ie Ca . ,1 year . 1–1 years or iCa .17 mmol  Presentation a onspecic eaness hypotonia mental status changes  Adominal pain one pain c Hematuria nephrolithiasis polyuria d eonates poor eeding ittery increased urine output assess or sucutaneous at necrosis especially i history o instrument-assisted deliery  Dierential diagnosis: see Tale 1  Investigations a erum total Ca alumin iCa PO  312  PTH -OH-itamin D renal unction

c pot urine calciumcreatinine ratio d Consider 1-OH-itamin D leel i PTH suppressedlo-normal or i suspicion or systemic in¡ammatory process eg sarcoidosis or malignancy Dirnial Dianosis o Hralia

h Insinal Ca Asorion

h Bon Rsorion

Or

ncrease a intake eritaminosis  Granlomatos isease  arcoiosis  ctaneos fat necrosis (neonates

aternal hoarathroiism (neonates erarathroiism  arathroi aenoma/ herlasia  Genetic, e.g., , cnelright snrome erthroiism eritaminosis  alignanc  araneolastic relate etie roction  Bon metastases

rgs  hiaie iretics  Lithim hoshate eletion ioathic infantile hercalcemia Familial hocalciric hercalcemia rimar arenal insfcienc enal failre haomolsis mmoiliation otal arenteral ntrition

Endocrinology

ale 4.

MEN, Multiple endocrine neoplasia; PTH, parathyroid hormone.

 Management a Hold itamin D calcium supplements  ormlaed inants: sitch to lo-calcium ormula c yerydration: I ¡uids at  to  times maintenance i normal renal unction d I no imrovement in 1 to 4 ors: consider isphosphonate 6 sucutaneous calcitonin use limited to – days ecause o tachyphylaxis e teroids: inhiit hydroxylation o -OH-itamin D may e useul in circumstances o increased 1-OH -itamin D production eg sucutaneous at necrosis sarcoidosis

14

PUBERTY ORMAL PUBERTAL DEELOPMET PEARL ert is ene  the aearance of a alale reast  in females an enlargement of testes 4 mL (2.5 cm long ais in males.

1 Denition: see Tales 17 and 1



oral Pural Dlon

ale 4.7

Mal

erage

0.5

.5

ange

8–

9–4

tage



helarche eeloment of reast tisse

ncrease testiclar olme .4 mL

2

arche aearance of ic hair

arche aearance of ic hair



Groth srt 8–4 cm/ear

longation of enis

4

enarche rst menstral lee, aroimatel 2 ears after thelarche

Groth srt 8–4 cm/ ear

Endocrinology

ge of onset (ears

ale 4.8

Tannr Sain

Male nial Dlon

Female Bras Dlon

Both Sexes Pui Hair



reertal testes, scrotm, an enis as in earl chilhoo

reertal aillar eleation onl

reertal no ic hair



nlargement of scrotm an testes

Breast  stage eleation of reast an ailla

arse groth of on hair at ase of enis/ along laia



enile lengthening, frther groth of testes an scrotm

Frther enlargement an eleation of reast an areola

arker, coarser, more crle sreas oer nction of es



ncrease reath of enis, eeloment of glans scrotal skin arkene

roection of areola an ailla to form a seconar mon

ltte hair, no sreaing to meial srface of thighs



lt genitalia

lt reast roection of ailla onl, areola ithin general contor of reast

lt antit an te sreaing to meial srface of thighs

Sa

14

al

PRECOCIOUS PUBERTY 1 Denition: onset o puerty  to  standard deiations eore population aerage ie thelarche eore age  years in emales testicular enlargement eore age  years in males a Etnic variation common: earlier onset o puerty may e typical  istory a econdary seal caracteristics: reast deelopment puic hair acial hair testicular enlargement penile enlargement  rot velocity: measured as cmyear 314 c amily istory: puertal timing o parents and silings

ale 4.9

Dirnial Dianosis o Proious Pur

oral arians rematre thelarche rematre arenarche

Cnral (onadoroin Dndn

Priral (onadoroin Indndn

ioathic (most common in females  malformation, trama or tmor  infection  raiation Genetic/familial reios se steroi eosre

 ogenos sesteroi eosre arian cst/tmor estosteronerocing tmor (e.g., Leig cell tmor, arenal aenoma or carcinoma hGrocing tmor (e.g., germi noma, teratoma, heatolastoma Familial malelimite recocios ert eere hothroiism cnelright snrome

Endocrinology

d ed ags: changes in ehaior or ision headaches seiures history o C diseaseradiation head trauma adominal pain exposure to exogenous sex steroids eg oral contracepties testosterone gel  Pysical: height eight Tanner stage acne acialaxillary hair ody odor  Dierential diagnosis: see Tale 1 a Precocious puerty less common in males more liely to e associated ith underlying pathology  Isosexual puertal changes occurring in seuence most liely central in origin c Out o seuence isosexual or contrasexual puertal changes more liely peripheral

14

CAH, ongenital adrenal hyperplasia; CNS, central nerous system; hCG, human chorionic gonadotropin. Modied from Sperling M. Pediatric Endocrinology. 4th ed. Philadelphia, PA: B Saunders; 4.

 Investigations a :  I predicts progression o central puerty ithin  months , I suggests peripheral etiology or enign ariant  : may e helpul ut eleated leels not specic or central puerty c estosterone or estradiol d 1OP androstenedione DEA: i male patient or emale ith eidence o androgeniation e   Bone age g Pelvic and abdominal U in emales: i lo H H or estro5 gen eleated or age

Endocrinology

h MI sella trcica: i diagnosis o central precocious puerty suspected or estalished particularly i , years at puertal onset or here puertal timing is out o eeping ith ethnicity and amily history  Management a Treat underlying cause i identied  entral recocios berty: consider nH analogues eg leuprolide c Perieral recocios berty: treatment aries ith cause consider aromatase inhiitors 7 oals o treatment a Optimie nal adult height limitedno enet i treatment started a£er age  years  Psychosocial ensure age-appropriate sexualehaioural maturation

DELAYED PUBERTY 1 emales: lac o reast deelopment y age 1 years primary amenorrhea y age 1 years or  years post-thelarche  Males: lac o testicular enlargement y age 1 years  linical evalation: as or precocious puerty plus a Diet exercise intensity eight changes chronic medical illness  idline deects cryptorchidism seletal anormalities anosmia 14 c History o alylating chemotherapy or cranialcraniospinal radiation d eatures o Turner or lineelter syndromes see elected enetic Conditions in Chapter 1 enetics and Teratology  Dierential diagnosis a Constitutional delay o groth and puerty more common in males  unctional hypogonadotropic hypogonadism may e related to chronic illness nutritional state hypothyroidism igorous exercise c yndromic hypogonadotropic hypogonadism eg allmann syndrome anosmia Prader-illi CHA oonan syndrome d Damage to hypothalamic-pituitary-gonadal axis C tumor or radiation e Hyperprolactinemia  Idiopathic hypogonadotropic hypogonadism g Hypergonadotropic hypogonadism primary gonadal ailure lineelter syndrome Turner syndrome autoimmune oarian ailure gonadal dysgenesis alylating chemotherapy gonadal radiation h Delayed puerty less common in emales more liely to e pathological  Investigations a  : hin primary gonadal ailure gin central hypogonadism  estosterone or estradiol c Bone age 316 d Prolactin 

DISORDERS OF SEX DEVELOPMENT

Endocrinology

e aryotye  Pelvic U in emales: i hH H to determine presenceasence o internalullerian structures g Brain MI: i gH H to assess or hypothalamic or pituitary disease  Management a Treat underlying cause i identied  atcl aiting: or constitutional delay c Indction o berty: sex steroid replacement may e reuired transiently or lie-long depending on underlying etiology i ales I testosterone  or  eely ii emales PO or transdermal estradiol ith addition o progesterone as Proera a£er 1 to  years or i menstruation egins

1 Denition: congenital conditions in hich the deelopment o chromosomal gonadal or anatomic sex is atypical  lassication: see Tale 11

ale 4.0

Classiaion o Disordrs o Sx Dlon

Sx Crooso DSD

4 Y DSD

4  DSD

ases

45  rner snrome 47  linefelter sn rome 45 /4  mie go naal sgenesis, ootesticlar  4 /4  chimeric, ootesticlar 

isorers of gonaal (testiclar eeloment isorers of anrogen snthesis/action  nme efect in arenal steroi snthesis  Leig cell sgenesis/ agenesis 5arectase ecienc omlete/artial anrogen insensitiit

isorers of gonaal (oarian eeloment nrogen ecess  ongenital arenal herlasia  aternal anrogen eosre  aternal iriliing tmors

etails

ften hae mi of streak gona, oarian or testiclar tisse

estes resent, location ma ar

ccont for  to 70 

14

DSD, isorders of se deelopment. Adapted from ughes A et al. onsensus statement on management of interse disorders. Arch Di Child ;:4–.

 linical evalation a Antenatal: prenatal androgen exposure maternal iriliation and medication use antenatal  including details aout inant sex  eonatal: eeding diculty poor eight gain omiting lethargy

7

Endocrinology 14

c amily: unexplained inant death spontaneous aortions consanguinity amily history o atypical genitalia parental ethnicity d Pysical: appearance o genitalia presence o palpale gonads including inguinal canal other anomalies or dysmorphic eatures e eatres sggestive o disorders o se develoment DD i Apparent emale genitalia ith clitoral enlargement .1 cm in term neorn posterior laial usion inguinallaial mass ii Apparent male genitalia ith ilateral undescended testes micropenis isolated perineal hypospadias or mild hypospadias ith undescended testis  Investigations a eborn screening reslts reie  aryotye and orescent in sit ybridiation I or SRY: expedite c Abdominal and elvic U presence o uterus agina gonads anomalies o urinary tract adrenal glands pathognomonic “cereriorm” appearance in CAH d itin rst  days o lie: 17-OHP androstenedione testosterone dihydrotestosterone estradiol H H serum electrolytes normal electrolytes do not exclude salt-asting CAH  Management rinciles a Psycosocial emergency: expedite inestigations and consult tertiary care center ith multidisciplinary team pediatricianpediatric endocrinologist urologist gynecologist geneticist social orer  Delay gender assignment: including name selection until inestigations and expert ealuation completed encourage terms such as “the ay” rather than “heshe”

COEITAL ADREAL HYPERPLASIA 1 ost common DD in emales  Etiology: autosomal recessie deects in enymes inoled in adrenal steroidogenesis a All orms associated ith cortisol deciency  Depending on enyme inoled may hae mineralocorticoid aldosterone andor adrenal androgen deciencyexcess c h ACTH secretion may cause hyperpigmentation and adrenal hyperplasia  1ydroylase deciency: most common deect a Impaired glucocorticoid and mineralocorticoid synthesis ith accumulation o iosynthetic precursors 17-OHP hich are instead shunted into androgen synthesis pathays leading to iriliation  ³ree sutypes i evere saltasting congenital adrenal yerlasia A: impaired cortisol and mineralocorticoid production hyponatremia 318 hyperalemia hypoglycemia dehydration acidosis ascular collapse

ADRENAL DISORDERS

Endocrinology

ii imle viriliing A: inadeuate cortisol production adeuate mineralocorticoid actiity ithout salt-asting iii onclassic A: later onset milder orm present ith iriliation only oerlap ith PCO in adolescents or inertility  Investigations a 1OP androstenedione testosterone: all increased in 1-hydroxylase deciency i Dierent precursors eleated in other orms o CAH  erm 1OP: unreliale , hours o age i alse ositive: preterm lo irtheight inants perinatal stress ii alse negative: possile i mother receied antenatal steroids c erm electrolytes: typically normal eore day  to 7 o lie in salt-asting CAH d aryotye: consider or phenotypic males i suspicion or seere CAH may e irilied emale  Management a lucocorticoid 6 mineralocorticoid replacement  alt aCl supplementation o£en reuired in neonatal period c anagement o intercurrent illness see next section on Adrenal Insuciency

14

ADREAL ISUICIECY 1 Etiology: see ox 1 Box 14.

Etiology of drenal Insufciency

Priar Adrnal Insuin ongenital congenital arenal herlasia, arenal holasia congenita toimmne ison isease, atoimmne olglanlar snromes  resistance snromes trile  snrome, familial glcocorticoi eciencies nfection sesis, terclosis, iral, fngal linke arenolekostroh isorers of cholesterol metaolism mithLemliit snrome itochonrial earnsare snrome mloiosis renal hemorrhage aterhoseFrierichsen snrome rgs inhiiting steroi iosnthesis, e.g., mitotane, ketoconaole Sondar Adrnal Insuin rolonge glcocorticoi thera othalamic/ititar tmors, srger, raiation ongenital hoititarism solate  ecienc ACTH Adrenocorticotropic hormone. Modied from Sperling M. Pediatric Endocrinology. 4th ed. Philadelphia, PA: B Saunders; 4.

9

Endocrinology

 Presentation: insidious onset may hae symptoms or months eore diagnosis a Anorexia eight loss  ausea omiting adominal pain c Hypoglycemia d loing o linear groth e uscle eaness atigue  Hypotension orthostatic diiness g in and mucous memrane hyperpigmentation h Adrenal crisis: acute onset or exaceration o insuciency ith electrolyte anormalities metaolic acidosis and hypotensie shoc  Investigations: see Tale 111 a lectrolytes urea creatinine  orning cortisol  am ACTH c Conrmation ACTH stimulation test

ale 4.

Laoraor indins in Adrnal Insuin Priar AI

Sondar AI

tiolog

renal failre

ecrease  release ecase of imaire snthesis or eogenos sression

ortisol

g

g



h

g

ineralocorticoi ecienc

a e resent

eer resent

14

ACTH, Adrenocorticotropic hormone; AI adrenal insufciency.

 ongterm management a ydrocortisone: diided ID or TID  ldrocortisone: i mineralocorticoid-decient c odim slements: or salt-asting phenotypes in inancy  Management o adrenal crisis a lid resscitation  I ydrocortisone: 1 mgm max dose 1 mg olloed y  mgm max  mg h 3  hours or until patient is stale c Monitor B: treat as needed d ludrocortisone “stress-dose” not reuired e Treat underlying illness 320

CUSHI SYDROME 1 Denition: clinical syndrome associated ith glucocorticoid excess  Etiology a Iatrogenic most common ecause o prolonged supraphysiological glucocorticoid exposure including topicalinhaled steroids  ACTH-producing pituitary tumor Cushing disease c Tumors producing ectopic ACTH or corticotropin-releasing hormone d Adrenocortical adenomas carcinomas ery rare  Presentation a Hypertension  Hyperglycemia glucose intolerance eight gain c loing o linear groth delayed sexual deelopment d Increased acial and adominal at stores supraclaicular at pad e Osteoporosis  asting o extremities proximal muscle eaness g enstrual irregularities h igns o adrenal androgen excess hirsutism oily sin acne to ace nec shoulders 6 hyperpigmentation i in iolaceous striae ruising sin thinning acanthosis nigricans acial plethora

Endocrinology

 Management o intercrrent illnesses a Additional “stress-dose” hydrocortisone reuired or i erile illness ii omiting or diarrhea iii Drosiness or decreased energy  ydrocortisone stressdosing:  mgmday diided h until symptoms resole c o change to ¡udrocortisone dose d ignicant omiting diarrhea or inaility to tolerate oral meds reuires admission or I hydrocortisone e IM ydrocortisone: may e reuired in emergency situations home supply can e gien y amily or emergency serices 7 Management dring anestesia a Minor rocedres:  mgm I hydrocortisone once at induction o anesthesia  Maor rocedres: 1 mgm max 1 mg I hydrocortisone at induction then  mgm h 3  to  hours

14

2

Endocrinology

 creening investigations a -hour urinary ree cortisol  idnight saliary cortisol c Perorm at least tice as hypercortisolism can e ariale  Diagnosis: dexamethasone suppression test a ie 1 mg dexamethasone eteen 1 pm and midnight measure next day early-morning cortisol  ormal response serum cortisol , nmol c I anormal reer to endocrinologist to conrm diagnosis and etiology  Management a Treat underlying cause  I caused y exogenous steroids ean gradually stress-dosing may e reuired during intercurrent illness

SHORT STATURE 1 Denition: height  standard deiations elo mean , percentile a ormal groth parameters see Assessment o roth in Chapter 1 roth and utrition  Dierential diagnosis: may e normal ariant or pathological Tale 11

14

ale 4.2

Dirnial Dianosis o Sor Saur

Ons

Dirnial

renatal

G lacental isease, congenital infection, teratogens, e.g., ethanol, meications hromosomal isorers, e.g., rner snrome, trisom 2 nromic, e.g., sselliler snrome

ostnatal

isroortionate groth

keletal slasia, e.g., achonrolasia

roortionate groth

ormal groth elocit

ickets onstittional groth ela (one age , chronological age Familial short statre (one age 5 chronological age ioathic short statre (iagnosis of eclsion normal groth elocit

hronic isease, e.g., G, renal, cariac, malignanc alntrition/malasortion nocrinoathies hothroiism, G ecienc, glcocorticoi ecess schosocial short statre rgs, e.g., sterois Genetic, e.g., raerilli, oonan snrome

322

GH, roth hormone; GI, gastrointestinal; IGR, intrauterine groth restriction.

ale 4.

Insiaion o Sor Saur

Insiaion

Possil Eiolo

B, 

hronic isease, e.g., B

GF

G ecienc



othroiism

reatinine

enal isease

ntiG, g

eliac isease

arote in females

rner snrome

L/F if signs of seal recocit

recocios ert

Bone age ra

a ifferentiate eteen constittional ela, familial short state, recocios ert

Endocrinology

 linical evalation a istory: prenatal insults irth eight eatures o underlying systemic genetic or endocrine disease nutritional intae symptoms o malasorption or I in¡ammation puertal changes  rot velocity: reie longitudinal groth charts c amily istory: parental heights puertal timing d Midarental eigt cm: 1⁄ mother’s height 1 ather’s height 1 1 males O 2 1 emales e Pysical eamination: height eight puertal stage dysmorphic eatures  Psychosocial urden o short stature  Investigations: ased on history and physical see Tale 11

14

CBC, omplete lood count; ESR, erythrocyte sedimentation rate; SH, follicle stimulating hormone; GH, groth hormone; IBD, inammatory oel disease; IgA, immunogloulin A; IG, insulin-lie groth factor ; LH, luteiniing hormone; TSH, thyroid stimulating hormone; TTG, tissue transglutaminase.

ROTH HORMOE DEICIECY 1 ongenital grot ormone  deciency a irth length normal or slightly reduced insulin not H is the primary antenatal groth actor  roth ailure may not e oious until  years or later c ay hae history o hypoglycemia micropenis prolonged neonatal aundice d Assess or midline deects hypopituitarism hypoglycemia more pronounced i concomitant ACTH deciency 2

Endocrinology 14

 Acired  deciency a roth ailure  Delayed one age c lo groth elocity or age d Increased eightheight ratio e Immature “cheruic” ace inantile oice  ost common pituitary deciency olloing cranial radiation  Investigations: as in Tale 11 a xclude other causes o groth ailure see Tale 11  aluate or other pituitary hormone deciencies TH ree T cortisol prolactin sodium urine specic graity i H deciency suspecteddemonstrated c ay reuire reerral or dynamic H testing  Indications or  treatment a H deciency  mall or gestational age ith ailure to catch-up y age  years c Chronic renal ailure d Turner oonan Prader-illi syndromes e Idiopathic short stature controersial  o enet in trisomy 1 most seletal dysplasias  iss o  treatment a enign intracranial hypertension  lipped capital emoral epiphyses c Progression o scoliosis d Transaminitis e lucose intolerance may progress to medication-induced diaetes  Increase in groth and pigmentation o nei g o eidence or increasing ris o malignancy aoid during actie treatment or malignancy and or 1 year olloing treatment

DISORDERS OF WATER BALANCE DIABETES ISIPIDUS 1 entral: deect in asopressin synthesis andor secretion  o pediatric DI  erogenic: see Tuular Disorders in Chapter  ephrology and rology  Etiology: see Tale 11

324

ale 4.4

Eiolo o Dias Insiidus

Cnral

roni

Genetic nltratie isease, e.g., L eolasm, e.g., germinoma  trama/srger  infection iline rain efects

Genetic rgince, e.g., lithim, amhotericin enal failre strctie roath Fanconi snrome ickle cell isease/trait arcoiosis

 Presentation a Polyria: . mday dilute urine  Deydration yernatremia: i no access to ater or impaired thirst eg hypothalamic inury c 6 ailure to thrie  Investigations a erm: osmolality glucose a  Ca  Urine: osmolality 6 specic graity glucose  onsider dierential diagnosis or olyria a Primary polydipsia eep ¡uid intae diary  Hyperglycemia hypercalcemia c Diuresis post-renal inury or urinary tract ostruction d Increased  eg hyperthyroidism erile illness e edications eg diuretics  anconi syndrome 7 Diagnosis a erum osmolality . ith urine , mOsmg 5 highly suspicious or DI  erum osmolality ,7 or urine . mOsmg 5 DI highly unliely c Conrm ith ater depriation test only in experienced centers d rain I renal  or genetic testing as indicated  Management o central DI a Desmopressin DDAP ia PO  C or intranasal route  ³iaide diuretics lo-solute ormula o£en used in neonates c Treat underlying disorder  Management o nerogenic DI a Access to ree ater lo-solute diet  ³iaide diuretics c Indomethacin

Endocrinology

CNS, entral nerous system; LCH, angerhans cell histiocytosis.

14

25

SYDROME O IAPPROPRIATE ATIDIURETIC HORMOE SECRETIO 1 ee uolemic Hyponatremia in Chapter 1 luids lectrolytes and Acid-ase

FURTHER READING Daneman D arret  Harrington  en a Cild Has Diaetes Toronto O oert ose 1 Endocrinology 14

326

USEFUL WEBSITES Diaetes Canada diaetesca International ociety or Pediatric and Adolescent Diaetes ispadorg

CHAPTER

15

Fluids, Electrolytes, and Acid–Base Laura Betcherman • Emma Ulrich • Katie Sullivan • Damien Noone

Common abbreviations Components of uid therapy Approach to uid management Dehydration Salt and water Potassium Magnesium Phosphate Calcium Acid–base disturbances

327

COMMON ABBREVIATIONS Also see pae iii for a list of other abbreiations used throuhout this book

Fluids, Electrolytes, and Acid–Base 15

ADH ATN BSA CK DI DKA EC ECsm E  HC IC ICsm IS N T C  K Na sm TH DI TA SA SIADH TB T TTK Cl K Na A NC sm 1

1

1

328

antidiuretic hormone acute tubular necrosis body surface area creatine kinase diabetes insipidus diabetic ketoacidosis etracellular uid EC osmolality fractional ecretion lomerular ltration rate bicarbonate ion intracellular uid IC osmolality interstitial uid nasoastric oral rehydration therapy partial pressure of carbon dioide phosphate plasma potassium plasma sodium plasma osmolality parathyroid hormone recommended daily intake renal tubular acidosis serum anion ap syndrome of inappropriate antidiuretic hormone total body ater tubular reabsorption of phosphate transtubular potassium radient urinary chloride urinary potassium urinary sodium urinary anion ap urinary net chare urinary osmolality

 Water: homeostasis depends on ADH renal function and intake a osses come from insensible losses diarrhea emesis astric aspira tion surical drains  Electrolytes: Na1 K1 Cl a osses mainly throuh urinary and astrointestinal I tract ery little throuh insensible losses  Dextrose: added in intraenous I solutions may not be necessary in patients ith hyperlycemia older teenaers or on ketoenic diet  luids can be administered parenterally Table  or enterally al 15.1 Fluid

Coonly sed ntraenous Fluid olutions [Na ] 1

[K ]

[Cl ]

Other

Glucose

1

Tonicity ersus Plasa

D5W

0

0

0

0

5 g/100 mL

Hypotonic

D10W

0

0

0

0

10 g/100 mL

Hypotonic

0.9% NaCl

154

0

154

0

0

Isotonic

0.45% NaCl

77

0

77

0

0

Hypotonic

0.2% NaCl

33

0

33

0

0

Hypotonic

130

4

109

Lactat 2 Ca 3

0

Isotonic

ing’s

Fluids, Electrolytes, and Acid–Base

COMPONENTS OF FLUID THERAPY

15

Note: Electrolyte concentration and relative tonicity of available IV uids. All concentrations in mmol/L; any dextrose solution may be mixed wit any of te Nal or iner’s solutions; l may be added at   or  mmol/L.

APPROACH TO FLUID MANAGEMENT  aintenance uids uid balance at homeostasis  eplacin uid losses a noin losses urine I—diarrhea nasoastric Nenterostomy tube output surical drains other body caities b Insensible physioloical losses from seat eaporation breathin  Decit replacement A. Maintenance requirements  Denition: uid and electrolyte reuirements ith normal hydration status olume of “maintenance uid” is an estimate of normal insensible losses and urine output in a healthy euolemic child  Based on estimated daily uid and electrolyte reuirements Table   Insensible uid losses are h in feer or oerhead armer g ith humidied 329 entilation

al 15.2

Recoended aily ntae a o Na1 K1 and ater

Fluids, Electrolytes, and Acid–Base

Paraeter

eiht Rane

R

Eale or a  Child

Na1

ny igt

2–3 mmol/g/ay

94–141 mmol/ay

1

ny igt

1–3 mmol/g/ay

47–141 mmol/ay

H2

1–10 g 100 mL/g/ay 11–20 g 50 mL/g/ay ac g .20 g 20 mL/g/ay  als ao a aiti

1000 mL/ay 500 mL/ay 540 mL/ay otal 5 2040 mL/ay

a

ecommended daily intae for ealty cildren beyond te neonatal eriod.

RDI ecommended daily intae.

PEAR se the “1” rule or calculation o hourly aintenance rate h 4 mL/g/ o t st 10 g 2 mL/g/ o t nt 10 g 1 mL/g/ ac aitional g o igt .20 g oly maintnanc at is igt g 1 40

15

B. rinciles o intraenous maintenance uids  No sinle solution ill precisely ie the reuired ater and electrolytes in all situations  eealuate freuently based on clinical picture eiht plasma and urine electrolytes  Include oral uids in uid balance  Infants and youn children hae limited lycoen stores so saline solutions ith added detrose are reuired to preent hypolycemia and ketosis detrose may be contraindicated in specic situations for eample patients on a ketoenic diet  Hospitalied children are at hih risk of SIADH Althouh hypotonic uids hae enouh sodium to meet daily reuirements administration can lead to hyponatremia a roups particularly at risk include children underoin surery in intensie care units IC and those ith acute illnesses includin meninitis encephalitis bronchiolitis and pneumonia  See iure  for prescribin recommendations 330

Fiure 151 Prescriin Recoendations or ntraenous Fluid aintenance in Children Maintenance fluid therapy Determine indication for IV fluids

Bolus fluid therapy Therapy to replace abnormal losses, i.e., from GI tract Order lab tests

to fluid administration

Complete assessment

Serum electrolytes (Na, K, lucose, creatinine Serum electrolytes (Na, K, lucose, creatinine

assessment for  patients receiin maintenance or replacement I fluids

aily intae and output aily eiht measurements IV Solution Na mmol/L

I bolus for seere  contractionimpendin shoc erioperatie

nnon serum Na Determine fluid selection and prescription

Serum

Na

 mmol

Serum Na – mmol

Serum Na – mmol otassium (KI may need to be added to I fluid prescription maintenance reuirement is approimately  m, but eact amount ill depend on indiidual factors includin serum K, serum r, and urine output.

. Nal



 . Nal



iner’s actate ith or ithout detrose



 . Nal



 . Nal



 . Nal or  . Nal



or maintenance I fluid Na should appro. ½ normal saline  . Nal If dehydration  . Nal

ECF, Etracellular uid Adapted from ¢e Hospital for Sick Children luid and Electrolyte Administration in Children Clinical ractice uideline 

15

331

Fluids, Electrolytes, and Acid–Base





. Fluid losses  Identify type of physioloical uid lost Table  al 15.3

Fluids, Electrolytes, and Acid–Base 15

Aroiate Electrolyte Coosition o Gastrointestinal Fluidsa

Fluid

[Na1]

[K1]

[Cl ]

[HCO ]

Nomal stool

20–30

55–75

15–25

0

omits/stomac ainag

20–100

10–15

120–10

0

Inammatoy iaa

50–100

15–20

50–100

10

ctoy iaa

40–140

15–40

25–105

20–75

115–140

5–15

95–125

30

40–90

5

20

15–30

Ilostomy ainag N isting a

in mmol/L ata from ennari  and iese . Acidbase disturbances in astrointestinal disease. Clin J Am Soc Nephrol. ;:–.

 Choose I uid see Table  that approimates epected losses  eplace onoin losses I diarrhea other body caities a If olume unknon can estimate  mk for each diarrhea and  mk for each emesis PEAR s 0.45% salin it potassim to plac gastic o iaal losss. It sol not  s to plac ilostomy losss as it ill  ypotonic to t ostomy otpt ptting t patint at is o yponatmia. Insta aim to plac ilostomy losss it 0.9% nomal salin 1 20 m/L Cl.

D. nsensile losses  Includes seat eaporation breathin  Approimated by BSA m  weight  height 3600 weight (kg); height (cm)

BSA 

 Estimates mday a Neonates 5 – mm 3 BSA h ith oerhead armer b entilated nonneonates 5  mm 3 BSA c Nonentilated nonneonates 5  mm 3 BSA E. Decit relacement  Determine decit deree of dehydration based on a eiht loss i ater decit  5 preillness eiht k – illness eiht k 332 ii  dehydration 5  3 ater decit   preillness eiht k

b Clinical assessment see Table  Each  dehydration corresponds to  mk of uid decit  A©er initial stabiliation replace remainin decit sloly oer net  to  hours Clinical Assessent o eree o ehydration a

ild ,5

oderate 5–1

eere .1

 ligtly cas in otpt  ligtly incas tist  ligtly y mcos mmans  ligtly lat at at

 Dcas in otpt  oatly incas tist  Dy mcos mmans  lat at at  Dcas sin tgo  nn ys  nn antio ontanll

          

aly cas o asnt in otpt atly incas tist y y mcos mmans atly lat at at Dcas sin tgo y snn ys y snn antio ontanll Ltagy Col tmitis Hypotnsion Coma

a

ese ndins are for atients wit a serum sodium in te normal rane. linical manifestations may differ wit yernatremia or yonatremia. Not all sins may be resent.

Fluids, Electrolytes, and Acid–Base

al 15.4

ata from Leun A rince . ral reydration teray and early refeedin in te manaement of cildood astroenteritis. Paediatr Child Health. ;:–.

DEHYDRATION

15

 ral rehydration therapy is as e§ectie as I therapy for mild to moderate dehydration from acute astroenteritis and is rst line  ral rehydration solutions S contains lucose hich enhances Na 1 and H transport across intestinal mucosa  See Table  for components of di§erent S and commonly consumed uids al 15.5

Coosition o Oral Rehydration olutions and Clear iuids Carohydrate ol

Na1 ol

K1 ol

ase ol

Osolality Os

ialyt 

140

45

20

30

250

WH/NIC 

111

90

20

30

310

Cola

700

2

0

13

750

ppl ic

90

3

32

0

730

0

250



0

500

pots ag

255

20

3

3

330

ing al

500

3

1

4

540

olution

Cicn ot

ORS ral reydration solution UNICEF nited Nations International ildren’s und; WHO orld ealt raniation.

333

Fluids, Electrolytes, and Acid–Base

 See iure  for alorithm for manain acute dehydration based on deree of dehydration  Considerations a Early refeedin i Commence  hours into therapy breastfeedin should continue despite diarrhea ii Etra S may be ien  to  mk for each diarrhea and  mk for each emesis iii Appropriate foods comple carbohydrates lean meat yourt fruits eetables i Aoid foods hih in simple suars b omitin i Administer small olume of S freuently  m eery – minutes ii Consider oral ondansetron therapy if omitin persists iii If omitin continues I hydration is indicated c efusal to take S i ie solution in small amounts to et used to salty taste ii Add  m of uice to  m of S to improe compliance iii N tube may be used to administer S Fiure 15 Alorith or anain Acute ehydration

15

Assessment of degree of dehydration

No dehydration 1. Age-appropriate diet 2. Replace ongoing losses with ORS

Mild dehydration 1. Rehydrate with ORS (5 mg oer h 2. Replace ongoing losses with ORS . Age-appropriate diet after rehydration

Moderate dehydration

Seere dehydration

1. Rehydrate with ORS (1 mg oer h 2. Replace ongoing losses with ORS . Age-appropriate diet after rehydration

1. ntraenos resscitation with normal saline (2 mg 2. Reassess and repeat if necessary . egin administering ORS when patient is stale . Replace ongoing losses with ORS 5. Age-appropriate diet after rehydration

ORS, ral rehydration solution Adapted from eun A rince T ral rehydration therapy and early refeedin in the manaement of childhood astroenteritis Paediatr Child Health.  –

SALT AND WATER  smolality a easure of the concentration of a solute in a ien eiht of ater 334 b nits msmk

c ain constituents Na1 accompanyin anions urea blood urea nitroen BN lucose d ost important plasma osmole is  Na e sm either measured in the laboratory or calculated (“Two salts and a sticky BUN”) i sm 5  3 Na  1 lucose 1 BN all in mmol  Water a As percentae of body eiht total body ater TB aries inersely ith ae Table  b Composition of TB see iure  1

al 15.

Total ody ater y Ae

Ae

 o ody eiht



In to

5%

0.5

m inants

70%

0.7

ols/ciln

5%

0.5

olscnts/alts

0%

0.

Fluids, Electrolytes, and Acid–Base

1

Fiure 15 Coosition o Total ody ater 15

TBW

ECF (1/3)

Plasma (1/4)

ICF (2/3)

ISF (3/4)

ECF, Etracellular uid ICF, intracellular uid ISF, interstitial uid W, total body ater

 Dynamics of uid shi©s beteen compartments a At euilibrium ECsm 5 ICsm b Chane in sm in either compartment causes rapid H  shi©s until ECsm 5 ICsm c Acute chanes minutes in sm occur only in the EC ICsm chanes occur sloly hours to days and are enerally not the primary problem d Chanes in Na alays result in proportional alterations in IC olume because Na remains larely outside the cell membrane and is there fore a ery e§ectie osmolyte i Hypernatremia results in IC olume contraction ater shi©s from IC nECF) ii Hyponatremia results in IC olume epansion ater shi©s from 335 EC nICF) 1

HPONATREA  Denition: Na , mE a Acute , h or chronic . h b ost common electrolyte abnormality in hospitalied patients  atoysioloy: typically a result of free ater retention arely because of ecess loss of urinary sodium  linical maniestations a See iure  b ostly asymptomatic ith mild . mE or chronic hyponatremia c Symptoms imply acute onset andor cerebral edema and occur because a rapid gECsm has caused H to shi© to the IC d Hyponatremia may eaerate sins of hypoolemia because uid shi©s from EC nICF (circulatory compromise occurs earlier than epecte) 1

Fluids, Electrolytes, and Acid–Base

Fiure 15 Clinical aniestations o Hyonatreia Sodium level

Symptoms

>125 mEq/L

----Headache

15

Nausea or vomiti Lethary/altered L taia sychosis 20 mmol/L

20 mmol/L

20 mmoI/L, variable

Hovolemia

Evolemia

Hervolemia

UNa+ UOsm

UNa+ UOsm

UNa+ UOsm

>Na + losses

Extrarenal H2O losses

Osmotic diuresis

Diarrhea with vomiting Increased insensibles Poor water intake

H2O shift into ells

Seizure Strong exercise

ariable, ver lo

ariable, not ver lo

Renal H2O losses >>>> intae

Renal H2O losses >> intae

Diabetes insiidus DI centralnehrogenic

Partial DI

>20 mmoI/L, variable

> H2O

ertonic IV luid igh Na+ intake

eraldosteronism

al 15.9

anaeent o Hyo or Euoleic Hyernatreia

 1

o patints it moynamic compomis sto intaascla olm it i sscitation

 2

Calclat  at cit pois t amont o lctolyt i n to sto nomal Na1  at cit L 5 W L 3 mas Na – ial Na  4 ial Na  1

1

1

 3

plac t cit sloly aim to coct o 4  Not Do not g Na y .0.5 mmol/ 12 mmol/L/ay cas o is o cal ma. is is spcially t in conic ypnatmia  osmotic iliation tn C an IC as alay occ. api coction cass clls to sll aising intacanial pss an incasing t is o ain niation.

Fluids, Electrolytes, and Acid–Base

 Manaement a Based on etioloy seerity of symptoms and timin acute or chronic i Hyperolemic hypernatremia reduce Na 1 intake ii See Table  for manaement steps of hypo or euolemic hypernatremia iii No eidencebased uidelines for therapy for seere . mmol or etreme . mmol hypernatremia Consider nephroloy consultation

1

 4

15

Coos an intanos soltion s al 15.1 an tmin t olm n to coct t  at cit 1 L D5W 5 100%  at 1 L 0.2%N 5 0%  at 1 L 0.45%N 5 50%  at 1 L 0.9%N 5 5%  at ampl I t  at cit is 250 mL an yo ant to s 0.45%N  at cit 4 %  at in 1 L o t cosn soltion 4 100 250 mL 4 50 4 100 5 500 mL 500 mL o 0.45%N is n to coct t cit

 5

1. plac t olm to coct  at cit o 4  2. Coos an I i o maintnanc an insnsil losss.  tos an potassim as p ns. 3.  in placmnt o ongoing losss msis iaa it 11 atio coos a i simila to t composition o t i lost s al 15.3

 

cc lctolyts 2–4

t tatmnt options

1. DD o cntal iats insipis 2. Dialysis o actoy cass

DDAP esmoressin; ECF extracellular uid; ICF intracellular uid; W total body water.

341

POTASSIUM

Fluids, Electrolytes, and Acid–Base

 aor intracellular cation crucial for many cellular functions a aintains cellular membrane potential and reulatin electrical ecitability muscles heart neurons  Input mainly from diet – mmolday  absorbed output mainly ia kidneys  otassium is in part reulated by aldosterone hih aldosterone 5 more potassium ecretion  se transtubular potassium radient TTK Bo  to estimate the deree of aldosterone actiity renal ecretory mechanisms o 15

Calculation of Transtubular Potassium Gradient TTKG 

[Urine K  ]  POsm [Plasma K  ]  UOsm

imnts o accacy sm/sm .1 Na .25 mmol/L Intptation  5  is nomal  .  sggsts psnc o aloston  , 5 sggsts lo aloston lls 1

15

342

HPOKAEA  Denition: K1 , mmol  Etioloy a K1 loss urine or I b h shi©s of K1 to the intracellular compartment  linical maniestations a Symptoms usually occur hen  K1 , mmol b hysical symptoms eakness muscle crampin constipation respiratory failure cardiac arrhythmias c Electrocardioram EC gST sements gT aes  ae eleation hT interals   Aroac to dianosis a See iure  b Characteristic EC chanes mandate rapid treatment c Inestiations i lasma Na1 K1 Cl2 Ca1 2 1 creatinine urea osmolal ity pH HC2 ii rinary K1 osmolality pH iii EC

Fiure 15 Hyoaleia ianostic Alorith ecent –  insulin or catecholamine treatment? es

o

Intracellular shifts

UK+ >20 mmol/L?

es

o

Medication-induced?

Antimicrobials?

Aminoglcosides enicillin amhotericin

Alalosis?

cessie omiting or I losses erentilation

o An of the folloing abnormalities?

Magnesium 0?

anconi itelman or artter sndrome

cessie seating?

iuretic use?

iuretic-induced K+ loss

o

idence of heraldosteronism?

es

o

cessie cutaneous K+ losses

iarrhea? omiting?

es

igh l K+ losses ith hotension

o

Lo dietar K+ or cla ingestion

I astrointestinal

15

343

Fluids, Electrolytes, and Acid–Base

 Manaement: see Table  for manaement steps al 15.10

Fluids, Electrolytes, and Acid–Base 15

344

anaeent o Hyoaleia

 1

Caiac monitoing

 2

otassim placmnt 1. I symptomatic o 1 ,2.9 mmol/L consi pantal tatmnt incling aing 20–0 m/L o 1 to intanos I i. oi I olss o potas sim to potct t at. 2. I asymptomatic an 1 .3.0 mmol/L ntal o slo I 1 spplmntation 3. I conic an/o stal may consi ntal tatmnt

 3

ntly mas sm 1

 4

at nlying cas

 5

Cc magnsim calcim pospat an tat accoingly ntat ypomagnsmia may aggaat t as cts o ypoalmia atmnt o concomitant aciosis it icaonat ill osn ypoalmia sconay to tanscllla sits

HPERKAEA  Denition: K1 . mmol in neonates .  mmol  Etioloy a g rinary K1 ecretion e renal failure b h I absorption of K1 or h intake c Cellular shi© hyperlycemia acidosis d seudohyperkalemia hemolysis seere leucocytosis thrombocytosis  linical maniestations a Symptoms usually occur in the contet of seere hyperkalemia b hysical symptoms muscle eakness paralysis palpitations syncope c EC chanes include i K1 .  mE peaked T aes ii K1 .  mE  ae idens and attens  lenthens  aes eentually disappear iii K1 .  mE S prolonation any conduction abnor mality sinus bradycardia entricular brillation entricular tachycardia  Aroac to dianosis a See iure  dianostic alorithm b Characteristic EC chanes mandate rapid treatment

Fiure 15 Hyeraleia ianostic Alorith Abnormal ECG changes? Yes

o Hemolysis Extreme thrombocytosis Extreme leukocytosis

Abnormal blood sample? Prescription of potassium?

Start treatment

Yes

o Plasma creatinine?

Pseudohyperkalemia latrogenic hyperK

Low/normal

Fluids, Electrolytes, and Acid–Base

c Inestiations i lasma Na1 K1 Cl2 Ca1 2 1 lucose creatinine urea osmolality pH HC2 CK CBC ii rinary K1 osmolality pH iii EC  Manaement: see iure  for manaement alorithm

High

15 enal failure

TTKG ? Yes

o

rine net charge

Positie

Type  TA

igh Psm or p ?

egatie

ypoaldosteronism Adrenal insufficiency Pseudohypoaldosteronism bstructie uropathy

Yes

K moes from cells to plasma

o

latrogenic hyperK yperK periodic paralysis habdomyolysis

EC Electrocardioram; RA renal tubular acidosis;  transtubular otassium radient. ee ox . for urine net care calculation.

345

Fiure 15 anaeent o Hyeraleia Potassium [K+] >6 mmol/L

TRUE

SPURIOUS

- Assess CAB and LOC - Attach monitors - Stop K+ infusions, TPN, and supplements - Estalish  access

Fluids, Electrolytes, and Acid–Base

rent EC ost important test to assess seerit of hperalemia

EC chanes present - Peaed T aes - Proloned P interal - lattened or asent P aes - idened S interal - Shortened Tc interal - Arrhthmias

Calcium gluconate 1 Neonates – mLdose  – mdose calcium luconate, – mmoldose elemental calcium Anormal EC

nfants and older children – mLdose  – mdose calcium luconate, – mmoldose elemental calcium sual adult dose – mLdose  – dose calcium luconate, – mmoldose elemental calcium

o EC canges Sit otassium into cells

15

- Specimen hemoled - Consider repeat K+ STAT

Salutamol 1 mcg/u    puffs min ×     puffs min ×  eentilate f difficult access and aaitin meds

Eliminate otassium om o Soium icaonate  ears old Sodium icaronate   mmolmL – mmoldose  oer – min

Soium olstene sulonate aealate  dose PO h  dose P h a  dose sual adult oral  dose

≥ ears old Sodium icaronate   mmolmL – mmoldose  oer – min

Contraindications ileus, reduced ut motilit, recent adominal surer,  perforation, hpernatremia

Ensure patient is effectiel entilated

or neonates, P is preferred oer PO

f ph , consider

uosemie –mdose O a  mdose

Chec K+  min after initiatin an treatment

- Consult nephrolo - Chec K+ min Insulin Regula Bolus  units diluted umulin-® insulin  unitsmL  ith  mL   oer  min Consider insulin infusion after olus

ealemia eacto to tea ialsis

CA Circulation airay and breathin EC electrocardioram I astrointestinal OC leel of consciousness PN total parenteral nutrition Adapted from ¢e Hospital for Sick Children uide to anaement of aediatric edical Emerencies 

346

 Input mainly from diet – mmolday of hich  is absorbed output mainly ia kidneys  anesium depletion is o©en because of astric losses  Important for calcium and potassium homeostasis a Hypomanesemia can cause hypocalcemia because of impaired TH release  To chane concentration of  1 from mmol to md multiply by   See Bo  for calculatin the fractional ecretion of manesium o 15

Calculation of Fractional Excretion of Magnesium 2

FE- Mg g21 Lo ,5% Hig .5% FE, ractional excretion.



Urine Mg2  PCr Plasma Mg2  UCr

 100

Fluids, Electrolytes, and Acid–Base

MAGNESIUM

HPOAGNEEA  Denition:  , mmol , md  Etioloy 15 a I causes g I absorption of 1 g intake staration acute pancreatitis ecess I losses omitindiarrhea b enal losses h urinary 1 loss diuretics nephrotoic medications itelmanBartter syndrome metabolic acidosis hypercalcemia familial hypomanesemia ith hypercalciuria  linical maniestations a sually asymptomatic b If associated ith hypokalemia or hypocalcemia presents ith their associated symptoms c EC proloned Tc interal d Seere risks include seiures cardiac arrhythmias torsades de pointes  Aroac to dianosis a Inestiations i lasma 1 Na1 K1 Ca1 2 creatinine urea ii rinary 1 usually timed collection Ca 1 spot or timed creatinine iii enal ultrasound for nephrocalcinosis if there is also hypercalcemia b Calculate the E1 see Bo  to determine if I or renal loss i If E1 . in a patient ith normal renal function renal manesium astin most likely 347 c See iure  for dianostic alorithm

15

Fluids, Electrolytes, and Acid–Base

348

Fiure 151 Hyoaneseia ianostic Alorith ased on Fractional Ecretion o anesiu -Mg2+? ig 

o 

Intake problem? Mg2+-deficient diet Protein-calorie malnutrition

Inappropriate renal losses

GI losses? IBD, Wipple, celiac, sort boel, familial Mg2+ malabsorption

Medication-induced Mg2+ losses

Genetic/familial Mg2+ wasting

o urine a2+r? a+-+-Pase mutation peractie a2+ sensor Primar perP

ig urine a2+r?

ntimicrobials?

ter medications?

Paracellin- mutation Gitelman

mpotericin B minoglcosides oscarnet

tanol isplatin closporine oop diuretics iaides

FE ractional excretion; I astrointestinal; ID inammatory bowel disease; PH aratyroid ormone.

HPERAGNEEA  Denition: 1 . mmol . md  Etioloy a g urinary 1 loss b h intake includin I administration of S  1 containin antacids  linical maniestations a ild hypermanesemia asymptomatic cutaneous ushin mild hypotension b Seere hypermanesemia . mmol muscle eakness hypore eia g B nausea omitin paralytic ileus hypocalcemia c Etreme hypermanesemia . mmol associated ith complete heart block and respiratory muscle paralysis d EC abnormalities bradycardia proloned  and Tc increased S duration typically seen at leels . mmol  Aroac to dianosis a Inestiations i lasma 1 Na1 K1 Ca1 2 creatinine urea ii rinary 1 usually timed collection Ca 1 spot or timed creatinine iii enal ultrasound for nephrocalcinosis b See iure  for dianostic alorithm c E1 see Bo  , implies suboptimal renal  1 ecretion  Manaement a Stop 1 infusions or supplementation b or rapid correction or if seere consider dialysis

Fluids, Electrolytes, and Acid–Base

 Manaement a Asymptomatic enteral  1 supplements b Symptomatic parenteral treatment measure  1 eery  to  hours a©er each dose of I 1 c Treat underlyin cause discontinue diuretics d Correct coeistin electrolyte abnormalities e onitor for hypermanesemia especially in patients ith abnormal renal function f Chronic hypomanesemia implies a decrease in both intracellular and etracellular 1 and reuires lonterm supplementation

15

349

Fiure 1511 Hyeraneseia ianostic Alorith Evidence of renal failure Yes

No FE-g

Renal failure

Fluids, Electrolytes, and Acid–Base

Low (5%)

eneic defec Herreasorion

g hera

Yes

 g infusion (reeclasia)

No



R

g-conaining anacids aharics

g enea

FE ractional excretion; I intravenous; PO er os by mout; PR er rectum.

15

PHOSPHATE  Central role in adenosine triphosphate AT deoyribonucleic acid DNA and ribonucleic acid NA bioloy maor constituent of bone  itamin D and TH are main hormones inoled in   homeostasis Table  al 15.11 Horones

Oriin Trier

Eect

Tarets

End Result

h H

aatyoi glan g Ca21 an to a lss tnt h 4  g 1 25itamin D an g g21

h Ca21

iny on itamin D

h asoption h oiliation h oction

g 4

iny

h ction

Li/iny h H g 4 

h Ca21

I tact

h asoption

h 4

I tact iny

h soption h asoption

yoi C clls h Ca21

g Ca21

I tact iny on

g asoption g asoption g stoclasts

h 4

iny

h asoption

h itamin D

Calcitonin

350

Ca1 and PO2 Reulation

I astrointestinal; PH aratyroid ormone.

 To chane concentration of   from mmol to md multiply by   See Bo  for calculatin the tubular reabsorption of phosphate T Calculation of Tubular Reabsorption of Phosphate FE-PO4 

UPO  PCr 4

PPO  UCr 4

TRP %  100  (1 – FE-PO4 )  Hig .95% Lo ,95% FE ractional excretion; RP tubular reabsortion of osate.

Fluids, Electrolytes, and Acid–Base

o 15

HPOPHOPHATEA  Denition: 2 , mmol , md  ost commonly seen in patients ith diabetes DKA or DKA recoery or anoreia refeedin syndrome  Etioloy a Inadeuate intake staration g I absorption of 2  of 15 dietary 2 is absorbed antacid use b enal losses h urinary 2 loss hyperparathyroidism diuretics anconi syndrome c Shi© into IC recoery from metabolic acidosis respiratory alkalosis refeedin syndrome insulin stimulated  linical maniestations a ost asymptomatic b Chronic seere hypophosphatemia  2 ,  mmol muscle eakness myalia Can lead to rhabdomyolysis skeletal deformities ith rickets  Aroac to dianosis a aboratory i lasma Na1 K1 Ca1 2 1 alkaline phosphatase TH itamin D albumin creatinine urea blood as CK ii rinary Ca1 2 lucose creatinine pH b See iure  for dianostic alorithm  Manaement a Based on etioloy and seerity of symptoms i ild asymptomatic enteral preferred ii Seere symptomatic parenteral  2 b Stop 2 binders calcium carbonate seelamer hydrochloride 351 c Correction of coeistin electrolyte abnormalities

15

Fluids, Electrolytes, and Acid–Base

352 Fiure 151 Hyohoshateia ianostic Alorith

ubular PO4– reabsorption?

High (>95%)

Low (2 weeks • History • Physical exam

Are there any red flags? fever vomiting loody diarrhea  anal stenosis adominal distensiontenderness palpale fecal mass sacral dimplingtuft flat uttocks anormal neurologic examination findings Yes Evaluate further Refer to gastroenterology • hild fails therapy • oncern for organic disease • hen management is complex Refer to general surgery • nfants with significant constipation or enterocolitis • lder children only if present with failure to thrive very atypical intractale constipation  have een referred y a pediatric gastroenterologist

Gastroenterology and Hepatology

Constipation: delayed or difficult defecation n the asence of organic pathology at least two of the following must occur

No

16

Functional constipation

Disimpact with • P  – gkgday for – days dose limit  gday R • Picoalax – sachetday for 2 days with appropriate fluid intake as per instructions on the ox

Reassess with primary care provider

Treatment Education • xplain pathogenesis of constipation • ot willful or defiant ehavior • uccess reuired ongoing treatment and followup • oiling may worsen initially can persist for 2–2 months • o not force new ehaviors on child

Diet • alanced diet of whole grains fruit and vegetales • ood fluid intake

Behavior modification • oilet time  timesday for  minutes • ood foot support while sitting on toilet • iary of stool freuency • Reward system reinforce good ehavior ignore soiling • nhurried daily emptying of stool crucial for success

Medication • P  – gkg after disimpaction completed • o not decrease dose until doing well for at least  months– year • ecrease dose slowly over several months ie y ⁄ dose

Close follow-up • Primary care provider • linic visits • elephone followup

FTT Failre to thrie PEG polyethylene lycol Adapted from ±e ospital for Sick hildren anaement of Fnctional onstipation linical Practice ideline 15 ith permission

31

PANCREATITIS

Gastroenterology and Hepatology 16

1 linial presentation: abdominal pain that may radiate to back or sholder omitin  tiology: see ox 1  Inestigations a Amylaselipase  times pper limits of normal i leated amylase also fond in pancreatic psedocyst parotitis biliary tract disease dodenal lcer peritonitis renal failre brns stress b Trypsinoen lier enymes conatednconated bilirbin amma-ltamyl transferase T lcose electrolytesextended electrolytes creatinine trilycerides blood as  blood cltre c S for detectin obstrction stones or bile dct dilatation  anagement a Analesia  ids antiemetics b ontrol of metabolic complications hyperlycemia hypocalcemia c asoastric sction consider early reintrodction of clear ids to solids as tolerated

Bo 16

Etiology of Acute Pancreatitis

Traa Infection - irus (eg mumps cosacievirus   epatitis A inuena A) - acteria - Parasites (eg malaria) Drgs - urosemie sterois aatioprine Ostrction - allstones - Coleocal cst - Pancreas ivisum - Sclerosing colangitis Ssteic diseases - C (pancreatic sufcient) - I - asculitis (SP S) - perparatroiism percalcemia - perlipoproteinemia I I Idioathic CF, Cystic brosis; EB, pstein-arr irus; HSP, Henoch-Schönlein purpura; , inammatory bowel disease; SLE, systemic lupus erythematosus

392

1 Laboratory ealation see Table 11 a Lier cell inry aspartate aminotransferase AST alanine aminotransferase ALT lactate dehydroenase L b holestasis bilirbin total conateddirect T alkaline phosphatase ALP c Synthetic fnction i g albmin clottin factors     X X cholesterol lcose ii h R partial thromboplastin time PTT ammonia

able 161

aorator Ealation of the ier

a

Sorce

Increased in

Coents

A AS

Preominantl liver but also eart seletal muscle ine pancreas lung brain leuoctes ertroctes

1 epatocellular inammation  Muscle isease 3 abomolsis  emolsis  Anoreia nervosa 6 Celiac isease (mil)

1 A more sensitive tan AS for etecting liver amage  Concurrent increase in C or  suggests primar muscle source 3 AS . A in emolsis muscle isorers

AP

iver bone intestine ine leuoctes

1  3  

Colestasis Inltrative liver isease one growt or isease rauma Pregnanc

1 Must ifferentiate from bone source (ie istor eamination suggestive of bone isease no elevation in )  Subnormal levels in ilson isease



iver ine pancreas seminal vesicles spleen eart brain

1 Colestasis  ewborn perio 3 rug inuce (eg pentoin penobarbital ertromcin nitrofurantoin alcool)

1 Most sensitive inicator of biliar tract isease  ot present in bone

1 iver isease  itamin  ecienc 3 actor II  II  or brinogen ecienc  sbrinogenemia

1 actor II earliest to be eplete in liver isease I prolonge before P

I (P)

Gastroenterology and Hepatology

LIVER DISEASE

16

ALP, lkaline phosphatase; ALT, alanine aminotranserase; AST, asparate aminotranserase; GGT gamma-glutamyl transerase; C, creatine kinase; INR, international normalized ratio; LDH, lactate dehydrogenase; PT, prothrombin time; PTT, partial thromboplastin time

33

IRA HEPATITIS See Table 11 for a comparison of hepatotropic irses able 1613

Gastroenterology and Hepatology 16

394

Coarison of aor Heatotroic irses Heatitis A

Heatitis B

Heatitis C

ransmission

ecal-oral

Parenteral vertical seual

Parenteral vertical seual

Incubation

1– as

6–1 as

1–1 as

is factors

1 Contaminate water or foo  ravel to enemic countries

1 Infant born to infecte moter  Cilparents from countr were  enemic 3 Se partners of infecte persons  Inection rug use  attoos

1 Inection rug use  Infant born to infecte moter 3 eele-stic inur in ealt care settings

iagnostic tests

See igure 161

See igure 1613

See igure 161

Presentation

1 Acute self-limite illness  ulminant epatic failure (,1)

1 Mostl asmptomatic  ulminant epatitis rare

1 Asmptomatic to clinical epatitis  ulminant epatic failure rare

Signs an smptoms of acute infection

ever malaise aunice anoreia nausea abominal iscomfort

atigue aunice artralgia artritis rases

onspecic onset usuall mil , aunice

Caracteristics of infection b age

1 Cilren ,6 ears 3 smptomatic (–3 wees) few aunice  ler cilren aults most smptomatic (lasting 1 wee to 6 monts) . aunice

1 iger rates of cronic infection in ounger iniviuals

1 Cronic infection in –6 cilren 6– aults (usuall asmptomatic)   ris of vertical transmission

Cronic infection

o

sAgeAg A 1ve for .6 monts or sAgeAg A 1ve an antic IgM negative

C A positive for .6 monts

Coarison of aor Heatotroic irses—cont’d Heatitis A

Monitoring

Heatitis B

Heatitis C

1 ASA ever 6–1 monts  eAg anti-e  A ever 6–1 monts 3 AP ever 6–1 monts (at ris for CC)  iver S perioicall  Increase freuenc of S surveillance for CC if cirrosis 6 Screen before starting immunosuppressive meications  ransient elastograp ever 6–1 monts

1 ASA ever 6–1 monts  uantitative C A assa perioicall 3 AP earl (at ris for CC)  iver S ever  ears  Increase freuenc of S surveillance for CC if cirrosis 6 ransient elastograp ever 6–1 monts

accine prean posteposure proplais

See Capter 3 Immunoproplais

1 See Capter 3 Immunoproplais  accinate against A

1 o C vaccine available  accinate against A an 

Management

Supportive

1 Counseling  Surveillance for isease progression an complications 3 reatment epening on age availabilit

1 Counseling  Surveillance for isease progression an complications 3 reatment epening on age availabilit

1 Cirrosis CC  are sin manifestations (ianottiCrosti) ine isease

1 Cirrosis CC  are etraepatic manifestations (eg troi sfunction ine isease)

ong-term complications

Gastroenterology and Hepatology

able 1613

16

AFP, lpha etoprotein; ALT, alanine aminotranserase; ntHB IM, hepatitis  core antibody immunoglobulin ; ntHBe, hepatitis  e-antibody; AST, aspartate aminotranserase; DNA, deoyribonucleic acid; HA, hepatitis  irus; HBeA, hepatitis  e-antigen; HBsA, hepatitis  surace antigen; HB, hepatitis  irus; HCC, hepatocellular carcinoma; HC, hepatitis C irus; RNA, ribonucleic acid; US, ultrasound

3

igre 161 Heatitis A Serological arers aunice A eatie concentration

ptos IgM Anti-HAV

Feca HAV

IgG Anti-HAV

Gastroenterology and Hepatology

0

4

8

12

16

20

Weeks after exposure

ALT Alanine aminotransferase HA hepatatis A irs I immnoloblin From deptsashinton edlabebiisionsiroimaespaeap

igre 161 Heatitis B irs Serological arers Symptoms HBeAg

Anti-HBe

Titer

Total Anti-HBc

HBsAg

16

0

A

4

Anti-HBs

Anti-HBc IgM

8

12

16

20

24

28

2

6

2

100

Weeks ater eposre Acute (6 months)

Chronic (ears) Anti-HBe

HBeAg HBsAg

Titer

Total Anti-HBc

Anti-HBc IgM (may reappear during flares of activity)

B





  6     6



ears

ees after eposure

A Acte hepatitis  irs  ith recoery  hronic  ntHB epatitis  core antibody ntHBe hepatitis  e-antibody ntHBs hepatitis  srface antibody HBeA hepatitis  e-antien HBsA hepatitis  srface antien HB hepatitis  irs IM immnoloblin  From Andono A tler  Lin R et al P Ce Mneent of Hetts B—k

396 Refeene HBR oernment of anada Febrary 1

igre 161 Heatitis C irs aorator arers HC 

Window period 0

10

20

30

40

50

60

70

80

90

100

days

Days after exposure

HC epatitis  irs From iral epatitis Sbcommittee Inteetton of Hetts C s Test Reslts Gdne fo Lbotoes Association of Pblic ealth Laboratories anary 1

OACOHOIC ATT IER DISEASE 1 esription: characteried by fat accmlation steatosis absence of alcohol consmption occrrin ith or ithot inammation and brosis  Sreening a Screen ith ALT if normal rescreen eery  to  years b ein screenin at ae  years for all obese children  .5th percentile and for oereiht children  5th–th percentile ith additional risk factors central adiposity inslin resistance prediabetes or diabetes dyslipidemia sleep apnea or family history of AFLnonalcoholic steatohepatitis AS c onsider earlier screenin in yoner patients ith risk factors seere obesity family history of AFLAS or hypopititarism  Inestigations see Fire 115 for screenin alorithm a loodork ALT AST total bilirbin lipid prole  T ALP R albmin total protein hemolobin bA1c b main abdominal S c Lier biopsy di¦erentiates beteen simple steatosis and AS determines seerity of hepatic brosis and proides pronostic information for disease proression  anagement: dietary modication exercise eiht loss diabetes manaement

Gastroenterology and Hepatology

Concentration

ntiHC

16

3

igre 161 onalcoholic att ier Disease Screening Algorith Child ≥8 years with chronically elevated ALT and overweight or obese

ALT elevation detected due to symptoms?

Gastroenterology and Hepatology

No

Are there red flags? No

Yes (evaluate in context of symptoms)

agnitude of ALT elevation  Counsel on diet and eercise and 2 epeat liver chemistry in the short term – months

No

Yes ALT ≥80?

ALT ≥ 2×L boys ≥2 girls ≥?

16

Yes

No

escreen every 2– years

No

ALT  L boys 2– girls 22–?

Yes

igns or symptoms of liver disease?

Yes urther testing or referral

Yes

urther testing and or referral to pediatric  or hepatology

Exclude infections eg hepatitis A g hepatitis  surface antigen hepatitis C antibody other chronic viral infections Exclude endocrine disorders thyroidstimulating hormone T free thyroine T Exclude autoimmune causes of ALT elevation total gA total g and tissue transglutaminase antibody antinuclear antibody antismooth muscle antibody antiliveridney microsomal antibody Exclude genetic causes of ALT ceruloplasmin andor 2hour urine copper lysosomal acid lipase alpha antitrypsin phenotype Imaging: abdominal ultrasound to rule out anatomical abnormalities or assess features of portal hypertension magnetic resonance imaging or spectroscopy to measure hepatic fat

No ollow per clinical udgement

Red flags for advanced liver disease—chronic fatigue  bleeding aundice splenomegaly firm liver on eamination enlarged left lobe of the liver low platelets low white blood cell count elevated direct bilirubin elevated  long history of elevated liver enymes 2 years

ALT Alanine aminotransferase GI astrointestinal I immnoloblin  ULN pper limit of normal From os  Abrams S arlo S et al ASPA clinical practice ideline for the dianosis and treatment of nonalcoholic fatty lier disease in children recommendations from the expert committee on AFL  and the orth American Society of Pediatric astroenteroloy epatoloy and

398 trition ASPA J Pediatr Gastroenterol Nutr 11–

Bo 16 • • • • •

Suggestive Signs and Symptoms of Acute Liver Failure

Anoreia nausea vomiting atigue malaise letarg irritabilit ever ulie smptoms aunice (6ar urine pale stools pruritus) epatic encepalopat alterations in sleep anor beavior

• Asteriis • etor epaticus (sweetis sligtl fecal smell of eale breat) • leeing (varices gastrointestinal tract cutaneous mucous membranes) • Ascites epatomegal splenomegal

Gastroenterology and Hepatology

ACUTE IER AIURE 1 enition a hildren biochemical eidence of acte lier inry and hepatic-based coalopathy not responsie to itamin  R 15 ith clinical hepatic encephalopathy or R  reardless of clinical hepatic encephalopathy and no eidence of knon lier disease b eonates seere hepatic dysfnction ith coalopathy metabolic instability and sins of lier damae in the rst  days of life  linial presentation: see ox 1  Inestigations: see Fire 11  anagement: see Fire 11

igre 1616 Acte ier ailre Inestigations Investigations

16 Investigations for diagnosis

Investigations for etiology

Laboratory assessment of ALF • Coagulopathy ↑ ,  • ↓ actor  and  (important in prognosis • ↑ Biliruin, A, A, A,  • ↑ Ammonia • ↓ irinogen • ypoglycemia • CBC, urea, creatinine, lood gas, electrolytes, Ca, g, 

Abdominal US and Doppler • Diagnosis, liver size • Assessment of portal vein and ow (Budd-Chiari syndrome, veno-occlusive disease Liver biopsy* • irology, , antiody staining, histology, enzyme assay, Cu and e stains • enogram and venous pressure Other • gs (↑g in autoimmune hepatitis • Autoantiodies (autoimmune hepatitis anti- A, anti- A, AA

Serology epatitis A, B, C Adenovirus B, , , C aricella arvovirus B nteroviruses chovirus, cosacievirus ooplasmosis Toxicology screen (serum, urine Acetaminophen AA

etabolic screen • erum AAs, urine organic acids, urine succinylacetone, urine-reducing sustances, ammonia, lood gas, glucose, lactate, urine etones, alpha fetoprotein, acylcarnitine prole • Copper, ceruloplasmin (ilson disease • α- Antitrypsin • erritin (hemochromatosis • Cholesterol, triglycerides (olman disease

*Consider transjugular biopsy if percutaneous biopsy contraindicated because of coagulopathy.

AA Amino acid ALF acte lier failre ALP alkaline phosphatase ALT alanine aminotransferase ANA anti-nclear antibody ASA acetylsalicylic acid AST aspartate aminotransferase CBC complete blood cont CM cytomealoirs EB pstein-arr irs EM electron microscopy GGT amma-ltamyl transferase HH hman herpes irs  HS herpes simplex irs IG immnoloblin  INR international normalied ratio LM lier kidney microsome PTT partial thromboplastin time SM smooth mscle

3

igre 161 Acte ier ailre anageent Complications and supportive care

Gastroenterology and Hepatology 16

400

Encephalopathy • Close monitoring of neurologic status • No sedation, acetaminophen, NSAIDs • Lactulose for hyperammonemia (watch for hypovolemia

Sepsis, SBP • Antiiotics (roadspectrum with gramnegative coverage for S • Diagnostic paracentesis for S Hepatorenal syndrome, cardiovascular or respiratory failure, pancreatitis • Close monitoring (cardiac, oygen saturation • Intuation and ventilation as reuired

Bleeding • itamin  I • I I •  and C Fluid and electrolyte imbalance • lucose I (titrate to maintain euglycemia  Diuretics for ascites

FFP Fresh froen plasma I intraenos NSAID nonsteriodal antiinammatory dr PPI protonpmp inhibitor PRBC packed red blood cells SBP spontaneos bacterial peritonitis

CHROIC IER AIURE 1 linial maniestations: see Fire 11  anagement a Slo or reerse proression of lier disease preent additional lier inslts b mmniations schedled accines hepatitis A and  accines c Aoid hepatotoxic medications e acetaminophen nonsteroidal antiinammatory drs SAs methotrexate alproic acid d Preent and treat complications Table 11 e arly assessment ith transplant team

igre 161 Stigata of Chronic ier ailre PL PEES EES

LE DGE EES Growth failure developmental delay

Gastroenterology and Hepatology

Encephalopathy Scleral icterus

Jaundice

Esophageal varices

Proximal muscle wasting Gynecomastia Liver fibrosis/cirrhosis scites

Splenomegaly

Spider nevi

scites

Pruritus

aput medusae nemia

Palmar erythema Digital clubbing

Leuopenia

ruising and petechiae

hrombocytopenia

16

nemia

one marrow changes

Decreased bone mineral density nle edema

nle edema Peripheral neuropathy

odied from achos  ritch  ackson R astroenteroloy and hepatoloy n Laxer R ed Te Hostl fo Sk Clden Atls of Pedts Philadelphia PA rrent edicine LL 5–1

able 161

Colications and anageent of Chronic EndStage ier Disease

Prole

Aroach

Malnutrition an growt failure

1 Aeuate caloric intae (MC an branc cain AA supplementation)  Ma reuire supplemental  feeings or P to prevent catabolic state ricets coagulopat eroptalmia

at soluble vitamin ecienc

1 itamin A    supplementation Continued

1

able 161

Gastroenterology and Hepatology 16

Colications and anageent of Chronic EndStage ier ailre—cont’d

Prole

Aroach

Colestasis (aunice pruritus antomas antelasma)

1 Coleretic agents (ursoiol colestramine)  Antiistamines rifampin

leeing - romboctopenia (persplenism) - Prolonge I6P - itamin  ecienc - sopageal varices (portal pertension)

1  3   6

Ascites (6eemapleural effusions) - lui is transuative (h serumascitic albumin graient 11 g)

1 Soium restriction  iuretics 3 ater restriction (insensible losses 1 output) if ilutional ponatremia prominent  Albumin an furosemie in cases of aotemia an intravascular volume epletion  Paracentesis for respirator istress

Increase susceptibilit to infections

1 Prevention (inactivate vaccines an live vaccines before transplant)  Appropriate antibiotic treatment

SP (presentation 6fever abominal pain g bowel souns altere mental status)

1 arl recognition an treatment

enal insufcienc

1 Prerenal failure volume epansion  A 6ialsis 3 At ris for epatorenal snrome

Impaire rug clearance (epatic an renal failure portal–sstemic sunting poalbuminemia)

1 euce osage monitor toicit coose alternative agents

epatic encepalopat (precipitating factors I emorrage poalemia especiall wit alalosis sepsis ig-protein iet constipation seative-pnotic meications aotemia pancreatitis surger)

1  3  

Avoi antiplatelet rugs (ASA SAIs) Platelet transfusion P an activate factor IIa for active bleeing itamin  PPIsoctreotie (I bleeing) noscopic scleroterapbaning portosstemic sunting for varices

Ientif removecorrect an avoi precipitants igt ui an electrolte control ietar protein restriction actulose (PP) g ammonia resorption Antibiotics (neomcin metroniaole) g available bacterial urease

AA mino acid; ASA acetylsalicylic acid; ATN acute tubular necrosis; FFP resh rozen plasma; GI gastrointestinal; INR, international normalized ratio; MCT, medium chain triglycerides; NG, nasogastric; NSAID, nonsteroidal antiinammatory drug; PO, per os by mouth; PR, per rectum; PPI proton-pump inhibitor; PTT, partial thromboplastin time; TPN, total parenteral nutrition; SBP, spontaneous bacterial peritonitis; UGI, upper gastrointestinal

402

HPERBIIRUBIEIACHOESTASIS I IAC 1 easre total conateddirect bilirbin in all andiced infants a f breastfed at  eeks of life b f formla fed at  eeks of life c At any ae if pale stools or dark rine  onateddirect bilirbin abnormal if a .1 mmolL if the total bilirbin is ,5 mmolL b . total bilirbin if total bilirbin is .5 mmolL  See Fire 11 for approach  Treatment depends on etioloy For biliary atresia timely dianosis important for pronosis

Gastroenterology and Hepatology

GALLBLADDER AND BILIARY TRACT

16

3

igre 161 Aroach to Cholestasis in Infanc Step : Rule out acute illness

Gastroenterology and Hepatology 16

Consider • Blood culture • Urine culture • Lumbar puncture • Review NBS for metabolic disorders—galactosemia and hypothydroidism • Hypopituitarism • Hemolysis • ron storage disease • ommon bile duct obstruction

Step 2: Is this a direct/conjugated hyperbilirubinemia?

• onugated direct hyperbilirubinemia  mmolL is considered pathological and warrants further investigation • nsure history physical eamination dysmorphic features urine culture are complete • amine the stool—acholic or hypopigmented stools suggest cholestasis or biliary obstruction • onsider metabolic disorders as outlined in Step  • f persistent hyperbilirubinemia—N URN R  NSULN

Step 3: UR ediatric astroenterology consult

Labs • B platelet count • Bilirubin—total direct delta • L S  L • lucose • NR • lbumin • lphaantitrypsini typing • lasma amino acids • mmonia and lactate level • RB galactosephosphate uridyl transferase • ortisol • SH  if NBS results not available • Serum bile acids • holesterol triglycerides • erritin • RH screen

• Urine • Reducing substances • Urine organic acids • Succinylacetone • maging abdominal ultrasound • enetics • onsider gene panels or eome seuencing • Sweat chloride analysis • Serum RR genetic testing if appropriate

Step : urther management steps by ediatric astroenterology

• f concern for Biliary tresia—consider percutaneous liver biopsy and intraoperative cholangiogram • urther testing • R—cardiac or respiratory disease • Spine ray—butterfly vertebrae • cho—evaluate for cardiac features of lagille syndrome • Liver biopsy • urther consultations • etabolics • phthalmology—evaluate for signs of lagille syndrome • udiology • ardiology • ediatric surgery—choledochal cyst • Nutritional rehabilitation

LP Alkaline phosphatase ALT alanine aminotransferase AST aspartate aminotransferase CBC complete blood cont CR chest x-ray GGT amma-ltamyl transferase GI astroenteroloy INR international normalied ratio IRT immnoreactie trypsin NBS neborn screen RBC red blood cell TSH thyroid-stimlatin hormone TORCH screen Toxoplasma ondii other irses rbella cytome-

404 aloirs herpes simplex irs

able 161

Differential Diagnosis of Congated Herilirineia Otside of Infanc

Disease

aor Diagnostic Strateg

Ostrctie Cholestasis Coleocal cst or oter congenital bile uct anomal

S colangiogram

allstones or biliar sluge

S

umor

S or C abomen

Heatocelllar Cholestasis

Gastroenterology and Hepatology

HPERBIIRUBIEIA OUTSIDE O IAC 1 nestiate ne onset andice to delineate conated hyperbilirbinemia obstrctie or hepatocelllar cases erss nconated hyperbilirbinemia hemolysis or inadeate bilirbin conation a bstrutie: stones choledochal cyst primary sclerosin cholanitis tmors parasitic infections b Hepatoellular: iral medication indced ilson disease atoimmne hepatitis  See Table 115 for di¦erential dianoses and approach  Treatment depends on nderlyin etioloy

16

Atoine Autoimmune epatitis

Increase AA ASMA M-1 serum protein electroporesis uantitative immunoglobulins

Primar sclerosing colangitis (PSC)

Increase AP  immunoglobulins CPMCP

Primar biliar colangitis (PC)

Increase AMA AA liver enmes liver biops

Disorders of Intraheatic Bile Dcts Alagille snrome (paucit of intraepatic bile ucts)

cocariogram (periperal pulmonic stenosis) ee eamination (posterior embrotoin) C (butter vertebrae) liver biops (paucit of small ucts) tpical facial appearance (broa foreea pointe cin elongate nose wit bulbous tip) genetic testing

onsnromic paucit of te intraepatic bile ucts

S colangiogram

Congenital epatic brosisCaroli isease

S colangiogram liver biops renal S (associate abnormalities)

Genetic and etaolic Disorders ilson isease

CC liver enmes ecrease serum ceruloplasmin increase copper level ocular slit lamp eamination   urinar copper ecretion

ubin-onson snrome

rinar coproporprin ecretion C (liver is ensel blac sows iger attenuation) liver biops Continued 

able 161

Differential Diagnosis of Congated Herilirineia Otside of Infanc—cont’d

Gastroenterology and Hepatology

Disease

aor Diagnostic Strateg

otor snrome

rinar coproporprin ecretion oral colecstograp

Mitoconrial isorers

CC elevate C liver enmes albumin lactate pruvate serum amino acis serum aclcarnitine urine organic acis

a1-A ecienc

ga1-A level Pi genotping ( nul nul ecient)

emocromatosis

herritin g total iron bining

Cstic brosis

Sweat clorie test

Progressive familial intraepatic colestasis

iver biops  (gnormal in tpes 1 an  h in tpe 3) genetic testing

Endocrine potroiism

hS g 

Panpopituitarism

g cortisol gS g

ToicSecondar Parenteral nutrition–associate colestasis

16

rugs (eg acetaminopen anticonvulsants) an toins (eg alcool organopospates)

rine an serum toicolog testing

Infectios epatitis A  C   nontpeable epatitis

A-IgM sAg anti-s eAg Anti-e Antic -A anti-c-IgM anti-C -IgM -IgM -Ig

I

I A PC serolog C count

Sepsis

loo cultures

Other ascular anomalies

oppler S

u-Ciari snrome

oppler S protrombotic wor-up

emangioma

oppler S

Cariac insufcienc an poperfusion

cocariogram

aAT, lpha- antitrypsin; ALP, alkaline phosphatase; AMA, antimitochondrial antibody; ANA, antinuclear antibody; ASMA, antismooth muscle antibody; CBC, complete blood count; C, creatine kinase; CT, computed tomography; CR, chest -ray; DNA, deoyribonucleic acid; ERCP, endoscopic retrograde cholangiopancreatography; GGT, gamma-glutamyl transpeptidase; HI, human immunodeciency irus; LM lier kidney microsome type ; MRCP, magnetic resonance cholangiopancreatography; PCR, polymerase chain reaction; TSH, thyroid stimulating hormone; US, ultrasound

406

GASTOES CHOEITHIASIS 1 tiology: hemolytic disease TP ilson disease epilepsy medications malabsorption  hepatobiliary diseases obesity abdominal srery acte lekemia cystic brosis F a Stone featres cholesterol risk factors hyperlipidemia obesity F prenancy octreotide se black piment risk factors hemolytic anemia TP ce§riaxone se or bron piment bacterial infections parasitic infections bile dct anomaly se of birth control  linial maniestations a hildren predominantly asymptomatic fond incidentally on S b f symptomatic likely secondary to cholecystitis cholanitis or cholestasis casin abdominal pain nasea omitin icters and positie rphy’s sin  Inestigations: abdominal S  Treatment: based on location of the stone seerity of symptoms case of stone formation comorbidities eidence of inammatory chane and ae See Table 11 for therapetic options able 1616

Gastroenterology and Hepatology

ACUTE ASCEDIG CHOAGITIS 1 esription: characteried by feer andice and riht pper adrant abdominal pain secondary to stasisobstrction and infection in the biliary tract  Assoiations: biliary atresia calcli benin strictre sclerosin cholanitis postlier transplant  iagnosis: h hite blood cell  cont cholestatic pattern of LFTs blood cltre S  Treatment: broad-spectrm antibiotics to coer ram-neatie bacteria and enterococci 6 anaerobes e ampicillin entamicin and metronidaole biliary drainae in select cases e cholanitis secondary to biliary calcli

16

Theraetic Aroaches for Cholelithiasis

Aroach

Descrition

Srgical Colecstectom

Meto of coice if smptomatic

Colecstostom

or acute gallblaer rainage (ie acalculous colecstitis)

ERCP aset removal

ile uct stone removal

Mecanical baset litotrips

Stone crusing witin te bile ucts

lectroraulic or laser litotrips

Stone estruction witin te bile ucts Continued 

able 1616

Theraetic Aroaches for Cholelithiasis—cont’d

Aroach

Descrition

Dissoltion

Gastroenterology and Hepatology 16

408

ral meication

rsoeocolic aci an cenoeocolic aci

Contact

Metl tert-butl-eter (colesterol stones) or bile aci-A solution (pigment stones)

Preentatie easres nteral feeing

ropic fees can ecrease stone formation wile on P

eigt loss

In obese patients

EDTA thylenediaminetetraacetic acid; ERCP, endoscopic retrograde cholangiopancreatography; TPN, total parenteral nutrition ata rom arami H, ianiar H, arami S Cholelithasis in children a diagnostic and therapeutic approach  Pedt Re ;e

LIVER TRANSPLANT 1 ost common indication biliary atresia  See hapter  Transplantation

CHAPTER

General Surgery

17

Jonathon Hagel • Justyna M. Wolinska • Georges Azzie

Common abbreviations Obstruction—neonatal Obstruction—early infancy Obstruction—childhood Acute abdomen Congenital diaphragmatic hernia Gastroschisis and omphalocele Hernias and the groin Preoperative fasting guidelines

409

COMMON ABBREVIATIONS Also see page xviii for a list of other abbreviations used throughout this book

General Surgery

CDH EA NG OR RLQ RQ A  E G ACERL

ongenital diaphragati hernia esophageal atresia nasogastri operating roo right loer uadrant right upper uadrant superior esenteri arter superior esenteri vein traheoesophageal stula upper gastrointestinal series vertebral defets anoreatal alforation cardia anoalies traheoesophageal stula ith esophageal atresia renal anoalies lib anoalies

OBSTRUCTION—NEONATAL TRACHEOESOPHAGEAL FISTULA (TEF) AND ESOPHAGEAL ATRESIA (EA)  Denition and etiology a Congenital anoalies aused b a defet in septation of the foregut into the esophagus and trahea 17 b Classied aording to the anatoi onguration igure   Assoiated anoalies in  of ases oen as part of vertebral defets anoreatal alforation ardia anoalies traheoesophageal stula ith esophageal atresia renal anoalies lib anoalies ACERL  Presentation a Cases of EA ,95%) present in the newborn perio i Histor of aternal polhdranios ii Excessive oral secretions and choking/cyanosis with feeding b H tye E ay e a delayed diagnosis rare subtpe that uniuel presents ith reurrent aspiration pneuonia  nvestigations a naility to ass nasogastric G tue into stoach exept H tpe oen diagnostic b  i pper esophageal pouh dilated ith air for to ost oon tpes ii Curled OGNG in upper esophagus   air in the G trat onrs the presene of a E versus pure EA d Contrast studies are rarel ever reuired if neessar use ater 410 soluble ontrast beause of risk of aspiration

General Surgery

Figure 17.1 Most Coo Fors o Esoge Atresi  Treoesoge Fistu

17

ro Liaouras CA endel D up C et al Gastroenterolog n olin RA Ditar  eds Pediatric Secrets th ed hiladelphia A Elsevier 

 anageent a NO on aintenane uids b Replogle tube set to lo sution  Consider antibiotis if onern for aspiration d E screen abdoinal  ardia ECHO spine xra and  exaination for lib anoalies e Operative repair

411

PITFALL Avoid intubation in cases of tracheoesophageal stula because there is increased risk of gastric perforation as a result of positive pressure. If intubated, move quickl to operative repair.

 olications a Anastooti leak esophageal striture reurrent E b Longter surveillane for feeding intolerane groth gastroesophageal reux disease GERD and traheoalaia General Surgery 17

INTESTINAL ATRESIA  Denition a Congenital defet resulting in obstrution in a hollo visous luen duodenal atresia ost oon eunoileal atresia oloni atresia b Duodenal atresia assoiated ith triso  and other ardia renal G anoalies eunoileal atresia assoiated ith sti brosis  Presentation a ilious voiting ost oon abdoinal distension failure to pass eoniu in rst  hours  nvestigations a Duodenal atresia AR shos “double bubble” sign distension of stoah and proxial duodenu oen visible on prenatal  pper gastrointestinal series an onr diagnosis b eunoilealoloni atresia AR shos di¨use air uid levels in ultiple loops of dilated intestine Contrast enea rules out both isolated and assoiated oloni atresias  anageent a Deopression of proxial G trat ith NG tube  uids b urgial repair

HIRSCHSPRUNG DISEASE  Denition and etiology a Absene of ganglion ells beginning at anoretal untion and extending proxiall for variable distane leading to funtional obstrution b Loation of aganglioni segent retosigoid  oloni extension – total olon   Assoiated ith triso  aardenburg sndroe ongenital entral hpoventilation sndroe ardiovasular anoalies  Presentation a aorit present in neonatal period ith signs of distal intestinal obstrution failure to ass econiu in rst  hours 412 adoinal distension voiting

General Surgery

b Hirschsrung enterocolitis potentiall lifethreatening opliation presenting ith diarrhea anorexia abdoinal distension and fever ith letharg or shok  Explosive release of stool and gas on retal exaination “blast sign” hih a teporaril relieve obstrution d Late presentation ith hroni refrator onstipation  nvestigations a  a sho nonspei ndings of distal boel obstrution di¨usel dilated boel ith air air uid levels b ontrast enea an suggest diagnosis and loation of transition one i allaliber retu retu should alas be ore dilated than sigoid ii Dilation of olon proxial to narroed aganglioni segent diaeter of olon . diaeter of retu ith transition one present in  of ases  Gold standard rectal iosy absene of ganglion ells d norectal anoetry absene of anoretal inhibitor reex a aid in diagnosis in older patients able to ooperate  anageent a Anal stiulation or retal irrigations for obstrutive sptos eneas should NO be used beause these infants annot sponta neousl pass stool b Denitive anageent is surgial ost oon surgial treatent is onestage ullthrough proedure  f enterocolitis present  uid resusitation broad spetru antibiotis retal irrigations to deopress  kg noral saline h a reuire urgent diverting osto if no response

17

MECONIUM ILEUS  Denition and etiology a Obstrution of the sall intestine at the level of the terinal ileu ith inspissated eoniu b ore than  of patients ith eoniu ileus have sti brosis C but it is the presenting feature in onl  to  of patients ith C PEARL All cases of meconium ileus should prompt a seat chloride test to rule out cstic brosis.

 Presentation a n general presents ithin rst  das of life failure to ass econiu in the setting of a patent anus adoinal distension 41 6 ilious eesis

General Surgery 17

 nvestigations a  i Dilated loops of sall boel without air–uid levels ii “Soaule” aearance of intestinal ontents proxial to the obstrution air ixed ith eoniu b ontrast enea i iroolon unused olon ii all pellets of eoniu outlined b ontrast aterial in terinal ileu  anageent a Nonoperative atersoluble eneas to break up eoniu and relieve obstrution b Operative if nonoperative anageent fails or “opliated” ases perforation ith eoniu peritonitis volvulus intestinal atresia ANORECTAL MALFORMATION (ARM)  Denition and etiology a Abnoralities in anoretal developent inluding iperforate anus perineal stula and persistent loaa a single orie in feales oen assoiated ith a hdroolpos b Anal atresia rare also assoiated ith ACERL  nvestigations a Look for eoniu exreted fro urethra vestibule of vagina or on perineal skin inluding the srotu via stula PEARL 0 to 90 of anorectal malformation cases can be determined b good clinical evaluation. All cases must, hoever, have thorough investigations for A associations. In males, eventual distal contrast stud via the mucus stula is required to dene the precise anatom.

 anageent a At presentation npo deopress the G trat urine assessent for stool and ACERL sreen see E setion for details Antibiotis if urethral stula suspeted b sually threestage surgical treatent involving olosto anoretoplast and olosto losure elet ases a be aenable to singlestage proedure

OBSTRUCTION—EARLY INFANCY HPERTROPHIC PLORIC STENOSIS  Denition and etiology a Hpertroph of plorus leading to gastri outlet obstrution 414 b Risk fators ales . feales  rstborn failial tenden

General Surgery

 Presentation a  eeks to  onths of life b Classi presentation postprandial nonilious roectile voiting folloed b desire to feed “hungr voiter”  Aute eight loss and dehdration an be seen ith prolonged sptos d Laborator evaluation hyochloreic hyokaleic etaolic alkalosis e Other ndings “olivelike” like ass in epigastriu and upper abdoinal peristalti aves late nding  Physical exaination a Epigastri “olive” ie the hpertrophied plorus a be palpable under the ostal argin in the idline hen infant is al and abdoinal usles relaxed  nvestigations a doinal ultrasound rst line i lorus easureents thikness .  length .  ii ailure to see gastri epting through the plori hannel b pper G ontrast stud onl if  not available looking for elongated plori hannel “string sign”  anageent a NO 6 NG tube for deopression b luid resusitation uid bolus as needed  3 aintenane uids a reuire – hours to orret eletrolte abnoralities and uid deit  urger should be delaed in the setting of dehdration andor eletrolte derangeents risk of hpotension at indution or apnea d Operative repair plorooto

17

OBSTRUCTION—CHILDHOOD INTUSSUSCEPTION  Denition and etiology a elesoping or invagination of proxial portion of intestine into ore distal portion b ost oonl ileocolic  leoileal or eunoileal intussus eptions usuall not signiant usuall inidental abdoinal  nding and generall no treatent neessar   of ases onsidered to be idiopathi G lphoid hperplasia fro viral infetion a serve as lead point d athologi lead points ekel divertiulu appendix polps dupliations subuosal heorrhage eg Henohhönlein 41 purpura lphoas tuors haartoa eut aeger

e f the intussuseption is seondar to a gastrostoeunosto G tube lead point interventional radiolog should be asked to anipulate the tube PEARL Atpical cases of intussusception atpical age, recurrent cases should prompt further investi gations to look for pathologic lead point.

General Surgery

 Presentation a piall presents  onths to  years of age b Cli eisodes of sudden cray severe adoinal ain –in oen aopanied b ring and draing legs up to abdoen atient a appear al pain free or lethargi beteen episodes  oiting a beoe bilious as obstrution progresses d loody stools grossl blood or oult blood positive “red urrant ell” stools are a late sign beause of intestinal uosal ongestion isheia or uosal sloughing e alpable right sided abdoinal ass  nvestigations a ltrasound is diagnosti “target sign”  sensitivit  17 speiit hen intussusepted  anageent a leocolic ost oon and colocolic ver rare intussusce tions should e treated all boelsall boel intussuseptions do not generall reuire treatent b NO uid resusitation as needed then proeed to air enea Consider broad spetru antibiotis onl if unstable or suspeted perforation  onoerative enea reduction i neuati air enea redution under ultrasound or uorosopi guidane is proedure of hoie ii Contrast bariu enea an be used slightl loer suess rates and opliations if intestinal perforation ours iii Delaed repeat enea for partl redued intussuseptions in stable patients oen suessful and avoids surger perfored at disretion of treating phsiians iv ostproedure advane to lear uids hen aake disharge hoe aer  to  hours if asptoati v Absolute ontraindiations to air enea heodnai instabilit pneuoperitoneu peritonitis d erative anageent indiated for patients ho are autel illunstable those ith signs of boel perforation or for failed 416 nonoperative anageent

OEL OSTRUCTION  Denition and etiology a echanical ostruction partial or oplete blokage of the boel resulting in failure of intestinal ontents to pass through luen b Paralytic ileus nonehanial boel obstrution funtional blokage of the boel beause of paralsis of intestinal usles  auses a ehanial obstrution i Extrinsi auses adhesions hernias inaator ass  lifetie risk of adhesive sall boel obstrution for postsurgial patients ost oon in rst  ears aer abdoinal surger ii ntrinsi auses intestinal atresia eoniu ileus Hirshsprung disease foreign bod ass dupliation b aralti ileus a our postoperativel as a side e¨et of drugs or reation to various inuries illnesses infetions  Presentation a Sall owel ostruction largevolue freuent eesis bilious eesis hen distal to apulla of ater rap abdoinal pain re lieved b voiting inial distension if proxial ore distended if distal undopliation patients a be unable to voit b arge owel ostruction abdoinal distension eesis that is rogressively feculent rap abdoinal pain  olete ostruction obstipation unable to pass gasstool versus incolete ostruction loose stools d Peritonitis surgial eergen should be suspeted if patient develops fever tahardia hpotension heateesis bleeding fro retu or linial peritoneal signs rebound guarding

General Surgery

e ecurrence risk  to  aer suessful nonoperative redution  to  aer surgial redution

17

PITFALL ailure to consider obstruction in a patient ith vomiting and abdominal distension but ho is still passing stools can lead to missed diagnosis.

 nvestigations a  at least to vies at and upright vies or at and le lateral deubitus i dilated loops of boel ii air–uid levels 41 iii pauit of gas in olon

General Surgery

b outed toograhy  adoen ay e reuired in ases of ehanial obstrution to full deterine the levelause of an obstrution and assess for oproised boel  anageent a P and  uids as needed bloodork CC eletroltes b G tue for deopression of the stoah  Antibiotis for an signs of boel perforation d Surgical consultation indiations for urgent surgial onsultation and possible operative intervention inlude peritonitis free air on AR radiologi evidene of nonevolving gas pattern sign of high grade obstrution obstrution in patients ith virgin abdoen ie no prior abdoinal surgeries

ACUTE ABDOMEN NEONATAL NECROTIING ENTEROCOLITIS ee nerotiing enteroolitis in Chapter  Neonatolog for further details

17

418

MALROTATION AND MIDGUT OLULUS  Denition and etiology a ntestinal alrotation ongenital anoal of rotation of the ebroni gut hih results in abnoral positioning of sall and large intestine ith a narrobased esenter igure  b idgut volvulus a true surgial eergen narro esenteri base perits tisting of the sall intestine hih obstruts esen teri blood vessels and auses gut isheia and infartion  Presentation a olvulus i ilious voiting sudden onset of abdoinal pain saphoid abdoen hih eventuall progresses to abdoinal distension as a late sign ii igns of progression fro isheia to infartion and nerosis fever peritonitis abdoinal distension dehdration heod nai instabilit etaboli aidosis septi appearane iii oe  of patients ith sptoati alrotation present in rst eek of life . in rst onth but a present at an age b alrotation older hildren ith unorreted alrotation an present ith hroni vague sptos suh as reurrent interittent voiting rap abdoinal pain failure to thrive or onstipation  alrotation an also be asptoati

Figure 17. Itesti Mrottio

1

Ascending colon

Descending colon

General Surgery

Ligament of Treitz

Cecum

17

2

A

B

C 1 Noral intestinal anato and esenteri xation  Abnoral esenteri xation in nonro tation A and inoplete rotation  leading to intestinal volvulus C ro Evans  Surgical Pitfalls aunders  and llie R Has  Pediatric Gastrointestinal and Liver Disease th ed hiladelphia A aunders 

419

General Surgery

 nvestigations a  should be used to rule out perforation if there is linial suspiion b er G series is the gold standard in stable patients i indings in alrotation duodenoeunal exure is to the right of the idline and loer than level of plorus ii indings in alrotation ith volvulus duodenu stas to right of spine and has a “orksre” appearane Contrast fails to ll eunu as a result of obstrution  aging aduncts i ltrasound inversion of the superior esenteri vein to superior esenteri arter relationship a suggest alrotation “hirlpool sign” suggests volvulus ii Contrast enea an be used to identif abnoral position of eu hoever high false positive and false negative rate iii AR a be noral or sho nonspei ndings intestinal dilatation gasless abdoen or rarel diagnosti ndings suh as “double bubble” sign signifing duodenal obstrution PITFALL ormal abdominal ra ndings do not rule out a malrotation or volvulus.

17

 anageent a NO ith uid resusitation as needed b NG tube  roadspetru  antibiotis d table patients should undergo iediate radiologi assessent e Critiall ill or unstable patients ith high suspiion for volvulus should be rapidl resusitated and taken diretl to the operating roo OR for exploration and repair f OR repair laparoto and Ladd’s proedure APPENDICITIS  Denition and etiology a naation of the appendix ost oonl aused b nonspei obstrution of the appendieal luen b eak inidene  to  ears of age  Presentation a eriubilial dull onstant pain folloed b anorexia and voiting b ain oves to the RLQ as inaation progresses hoever  have atpiall loated appendix 420  a be aopanied b ater diarrhea lograde fever

d RLQ tenderness ith loalied peritonitis is ost valuable nding on exaination e Generalied peritonitis a our ith nonontained perforation f erforation orrelates ith duration of sptos a our –hours aer onset of sptos infants and hildren , ears of age have highest rates of perforation

ommon mimickers of appendicitis include ovarian torsion, ruptured ovarian cst, ectopic pregnanc, referred testicular pain, mesenteric adenitis, urinar tract infections, ileitis, and inammator boel disease.

 nvestigations a aoratory tests i C and absolute neutrophil ount ANC freuentl elevated but not spei nor sensitive enough to rule diagnosis in nor out ii bhCG to rule out etopi pregnan in adolesent feales iii rinalsis an help rule out  hoever sterile puria is oonl seen in appendiitis b aging i ltrasound should be used as rstline diagnosti stud in stable patients nonopressible tubular struture diaeter . hpereia 6 alied appendiolith surrounding ehogeni fat free uid uid olletions if perforation ii  scan or  for ases ith inonlusive  ndings and high linial suspiion  anageent a P ith  uids b  antiiotics i Cefoxitin if not perforated ii road spetru antibiotis if perforated eg eriaxone and etronidaole  onerforated aendicitis or advaned appendiitis ithout appendieal absess urgent appendeto d Delayed resentation associated with erforation and hlegon or ascess i road spetru  antibiotis until afebrile and liniall iproved ii f no linial iproveent obtain additional iaging to rule out absess foration onsider drainage and additional antibiotis iii nterval appendeto an be onsidered aer iniu of  to  eeks

General Surgery

PEARL

17

41

CONGENITAL DIAPHRAGMATIC HERNIA

General Surgery 17

422

OCHDALE HERNIA  Denition and etiology a Herniation through posterolateral foraen of ohdalek ost oon b Lesided defets ost oon – oasionall bilateral ,  Assoiated anoalies – CN lesions esophageal atresia ophaloele ardia lesions triso  lethal sndroes  Presentation a Prenatal diagnosis ost oonl diagnosed on routine  b Postnatal resentation severe respirator distress in rst hours of life seondar to pulonar hpoplasia and resultant pulonar hpertension auses R→L shunt and persistent fetal irulation  Physical ndings i Absent breath sounds on a¨eted side ith barrelshaped hest ii aphoid abdoen seondar to abdoinal ontents in the hest iii hied heart sounds ediastinal shi  nvestigations a / i Loops of airlled intestine ithin thorai avit ii Absene of intestine in abdoinal avit iii hied ediastinu iv NG tube in hest if stoah above diaphrag b easureent of re and ostductal saturations  EH to assess pulonar hpertension assoiated ardia anoal inreases ortalit  anageent a Avoid bag and ask ventilation leads to gastri distension lung opression b Rapid endotraheal intubation if breathing oproised i entilate ith lo peak inspirator pressures ii a reuire high freuen osillation ventilation pulonar vasodilators eg nitri oxide and extraorporeal ebrane oxgenation ECO  NG tube to lo sution deopress stoah d Operative repair hen heodnaiall stable on inial ventilator support

e ostoperative anageent is opliated b pulonar hperten sion beause of pulonar hpoplasia neither an be orreted surgiall ortalit is diretl proportional to the degree of pulonar hpoplasia PITFALL

MORGAGNI HERNIA  Denition and etiology a Herniation of boel through anterior foraen of orgagni ost oonl rightsided b Aounts for , of all ongenital diaphragati hernias  nvestigations a CR inidental nding in hildren ith sptos of intestinal obstrutionrespirator illness  anageent a One identied repair to avoid sall risk of inareration

General Surgery

entilation ith high positive pressures in cases of congenital diaphragmatic hernia can cause barotrauma to hpoplastic lung.

17

GASTROSCHISIS AND OMPHALOCELE ee able  able 1.1

Crteristis o Gstrosisis  Ooee

Ftor

Gstrosisis

Ooee

enition

Abdominal contents free on anterior abdominal all defect commonl to right of umbilical cord

Abdominal contents herniated onto anterior abdominal all but are encased in sac unless ruptured

mbilical cord

eparate

Included in defect

ie of defect

mall – cm

ariable –1 cm

ontents

oel occasionall bladder ovaries, testes

oel frequentl liver

embrane

ever

Alas ma rupture

otational anomal

es

es

Intestinal function

rolonged ileusdsmotilit

suall normal

Associated defects

Intestinal atresia 10–1

ardiac 0–, chromosomal, e.g., eckith iedemann sndrome –1 Continued 4

able 1.1

Crteristis o Gstrosisis  Ooee—ot’

General Surgery

Ftor

Gstrosisis

Ooee

anagement

 osition in right lateral decubitus,  to suction  over viscera ith sterile saline soaked gaue and cellophane rap  enerous I uids goal urine output 1 mkgh and antibiotics   for reduction primar closure or staged closure ith silastic silo

  to suction  terile rapping of boel  enerous I uids goal urine output 1 mkgh and antibiotics  mall defects – cm primar closure in   arger defects stageddelaed closure ith use of silo, patch, escharinducing dressing

IV, Intravenous; NG, nasogastric; OR, operating room. Modied from Holcombe GW, Murphy J, stlie J. Ashcraft’s Pediatric Surgery. th ed. hiladelphia lsevier; .

HERNIAS AND THE GROIN UMILICAL HERNIA  Due to inoplete losure of ubilial ring aer ord separation  Alost never inarerate aorit lose b  ears of age  nlikel to lose spontaneousl aer  ears of age surgial repair reuired 17

INGUINAL HERNIA  Denition and etiology a rotrusion of a portion of the abdoinal ontents into the srotu or labia through an abnoral opening in the inguinal anal usuall seondar to a patent proessus vaginalis a ontain oentu boel and oen ovar 6 fallopian tube in infant feales  Presentation a ulging in groin or scrotal sac inreased ith ring or straining b ncarceration herniated ontents not reduible b anipulation but ontents are viable i Ours in  of inguinal hernias in the rst ear of life ii igns of inareration irritabilit distension voiting r tender ass  Strangulation vasular oproise of the ontents of an inarer ated hernia an our ithin  hours of inareration a lead to nerosis and perforation i igns of strangulation ertheatous hard xed painful ass fever PEARL

424

isk of incarceration is higher in infants ,4 months or histor of prematurit therefore these patients require earl referral.

HDROCELE  Denition and etiology a luidlled olletion around a testile or anhere along the path of testiular desent beause of a persistentl patent proessus vaginalis seen in approxiatel  of hildren a be assoiated ith an inguinal hernia  Presentation a o nontender transilluinating uidlled sa b f uid in hdroele ouniates ith peritoneal avit sie of hdroele a utuate ith hanges in position PITFALL

General Surgery

 nvestigations a hsial exaination ost iportant investigation b ltrasound an be helpful in diagnosis of inarerated hernia  AR a reveal boel loop in inguinal region  anageent a nareration and strangulation are surgial eergenies unsuess ful redution reuires urgent surgial orretion b f hernia is reduible refer to surgeon for eletive operative repair  hile aaiting surger ounsel parents regarding need for edial attention should sptos of inarerationstrangulation develop

17

speciall large hdroceles or hdroceles of the spermatic cord ma be difcult to distinguish from an incarcerated inguinal hernia. ltrasound ma help clarif the diagnosis.

 anageent a Nonouniating hdroeles and sall ouniating hdroeles oen involute in rst  onths of life b Larger counicating hdroeles or those that ersist eyond  onths of life reuire surgial intervention CRPTORCHIDISM  Denition and etiology a estile not present in noral intrasrotal position b ore oon in preature bos ost oen unilateral and rightsided  nvestigations a hsial exaination ept and soeties hpoplasti srotu heisrotu b o deterine loation of testile phsial exa  R laparosop  n ases of bilateral undesended testiles onsider disorders of 4 sexual developent

PEARL etractile testes can be mistaken for crptorchidism. ost testes can be palpated b eperienced eaminer under ideal conditions repeated eaminations ma be required. hen in doubt, refer.

General Surgery

 anageent a ost testiles that are undesended at birth oplete their desent b  onths of age b estiles that have not desended b  onths of age should be referred to surgeon to iniie risk of developing opliations testiular torsion or traua subfertilit Orhidopex an iprove fertilit and failitate onitoring for testiular alignan operative repair does not derease lifetie risk of alignan

PREOPERATIVE FASTING GUIDELINES ee able  able 1. 17

Preorertie Fstig Reoetios

Igeste Mteri

Miiu Fstig Perio

lear uids

h

reast milk

4h

ormula

h

olid food

h

rom merican ociety of nesthesiologists. ractice guidelines for preoperative fasting and the use of pharmacologic agents to reduce the ris of pulmonary aspiration application to healthy patients undergoing elective procedures. Anesthesiology. ;–.

426

CHAPTER

18

Genetics and Teratology Areej Mahjoub • Gregory Costain • Roberto Mendoza-Londono

Common abbreviations Denitions Modes of inheritance Genetic testing Child with multiple congenital anomalies Selected genetic conditions Teratology Perimortem workup in child with dysmorphisms

427

COMMON ABBREVIATIONS Also see page xviii for a list of other abbreviations used throughout this book

Genetics and Teratology 18

7-DHC AD ADHD AR CMA DNA FIH F HC MA NF N ND CR  C F D D

7-dehdroholesterol autosoal doinant attention-deithperativit disorder autosoal reessive hroosoal iroarra analsis deoxribonulei aid uoresene in situ hbridiation failure to thrive head iruferene ulti-plex ligation dependent probe apliation neurobroatosis next-generation seuening neural tube defets polerase hain reation phenlketonuria tuberous slerosis oplex tetralog of Fallot uniparental diso ventriular septal defet

DEFINITIONS  Anticipation: orsening of disease severit and dereasing age of onset in suessive generations feature of a fe AD onditions and harateristiall ours in triplet repeat disorders eg otoni dstroph aternal Huntington disease paternal  Association: nonrando group of developental anoalies seen ore freuentl than expeted b hane not aused b a seuene or sndroe eg vertebral defets anoretal alforation ardia anoalies traheoesophageal stula ith esophageal atresia renal anoalies lib anoalies ACR  Deformation: extrinsi nondisruptive ehanial fores on a noral struture ausing abnoralities  Disruption: extrinsi fators disrupt developent of noral tissue ausing destrution  Dysplasia: abnoral ellular organiation of funtion aeting a single tissue tpe  Genotype: geneti akeup of an organis 7 Malformation: intrinsi defets in orphogenesis aused b disturbane in developent or groth in ebrogenesis  Mosaicism: presene of to or ore genotpiall dierent ell popula428 tions ithin an individual

PEARL Variable expression of phenotype is the norm for the majority of genetic conditions, even within a family.

Genetics and Teratology

 Penetrance: proportion of individuals ith a partiular geneti hange ho exhibit an signs or sptos of the assoiated geneti disorder either oplete or inoplete  Phenotype: linial expression of the genotpe  Sequence: series of developental abnoralities aused b a single priar defet eg otter seuene  Syndrome: reogniable pattern of developental anoalies ith a single etiolog eg Don sndroe  Uniparental disomy: individual reeived to opies of a hroosoe or part of a hroosoe fro one parent and no op fro the other parent  ariale epressiity: range of signs and sptos in individuals ith a partiular genotpe

MODES OF INHERITANCE ee able  18

GENETIC TESTING A Approach to interpreting genetic tests  retest ounselling and infored onsent reuired before ordering geneti testing Carrier testing or preditive testing for later onset disorders is disouraged until a patient is able to provide their on infored onsent  otential outoes of geneti testing a ormal or negatie: no abnoralit identied In ost ases this does not rule out a geneti ontribution to the presenting phenotpe b iely pathogenic ariant or change: this nding is likel assoiated ith a spei pattern of health andor developental probles An additional blood saple fro the hild and parents a be reoended to investigate the origin of the variant  ariant of unnon signicance: this variant a or a not be assoiated ith health andor developental probles esting of parents a be reoended to investigate the origin of the variant d Unepected nding: this variant is unrelated to the reported healthdevelopental probles in the hild but ould be assoiated 429 ith risk for another disease

Inheritance

Genetics and Teratology

18

430

able .

Characteristics of ifferent odes of enetic Inheritance Affected Sex

Transmission Risk

Typical Characteristics

Examples

endelian A

5

  if parent is affected  f de novo variant parents are naf fected, low ris , in sbseent pregnancies

 henotype appears in every generation  aletomale transmission possible  otential for incomplete penetrance andor signicant variable expressivity

oonan syndrome, , arfan syndrome

A

5

naffected carrier parents, ris of having  Affected child 2  naffected not carrier child 2  naffected carrier child 

 arents are typically obligate carriers  f consanginity or from same ethnic poplation, more liely to be carriers for the same genetic condition

ystic brosis, sicle cell disease

lined

.

 ales transmit the variant to all their daghters and none of their sons  emales have a  chance of transmit ting the variant  emale carrier’s ris of disease depends on the nderlying condition and the random pattern of  inactivation

 n general, more severe phenotype inclding perinatal death in males, compared with females  eteroygos female carriers range from naffected to less severely affected, based on  inactivation  o maletomale transmission  Variant can be passed throgh mltiple carrier females, so may appear to “sip” generations

chenne msclar dystrophy, fragile  syndrome, hemophilia A

onendelian ltifactorial

isease specic

is of recrrence related to disease incidence, bt typically lower than a endelian condition

 ltiple genetic and environmental factors  ccrs more often in rst and seconddegree relatives than expected by chance

onsyndromic cleft lip andor cleft palate, , diabetes mellits, asthma

itochondrial A

5

All maternal offspring can be affected

   

A mitochondrial encephalomyopathy, lactic acidosis, and stroelie episodes, mitochondrial Aassociated eigh syndrome

aternal inheritance of mitochondrial A henotypic variability threshold effect ales cannot transmit disease to offspring Affects highenergy tisses cardiac and seletal mscle, brain, liver, optic tract

AD, Autosomal dominant; AR, autosomal recessive; DNA, deoxyribonucleic acid; NF1, neurobromatosis 1; NTD, neural tube defects.

18

4

Genetics and Teratology

Genetics and Teratology 18

432

 Types of genetic tests  luorescence in situ hyridiation S a argeted test that reuires seleting a probe for a spei hroosoe region b uanties the nuber of spei hroosoes or hroosoal regions through hbridiation attahent of uoresent-labeled DNA probes to denatured hroosoal DNA  sed for rapid detetion of irodeletionirodupliation sndroes eg  deletion sndroe hroosoal aneuploid eg triso  and sex hroosoes disorders eg in the setting of abiguous genitalia  hromosomal microarray analysis MA a Nontargeted higher resolution test to detet genoe-ide DNA op nuber abnoralities ie gains and losses of hroosoal aterial b First-line test for presentations suh as global developental dela intelletual disabilit autis spetru disorder ultiple ongenital anoalies  aryotype a Non-targeted lo-resolution test to deterine nuber and gross irosopi struture of all hroosoes b niuel inforative for balaned hroosoe rearrangeents and lo-level osaiis  oninasie prenatal testing PT a reening not diagnosti test using a blood saple fro a pregnant oan to dedue the presene or absene of triso  and liited nuber of additional hroosoal disorders in the fetus  Single gene testing a argeted tehniue used to detet variants in a gene of interest b sed in the setting of a phenotpe strongl suggestive of a speifi single-gene disorder eg frataxin gene in Friedreih ataxia  Gene panel testing a eitargeted tehniue of seuening ultiple preseleted genes in parallel via next-generation seuening sed in the setting of oon phenotpes ith a oderate degree of geneti heterogeneit eg nonsndroi epileps 7 holeeome sequencing S a Nontargeted test that attepts to seuene the exoe in a DNA saple b Does not reliabl detet all hroosoal disorders so does not negate the need for CMA

CHILD WITH MULTIPLE CONGENITAL ANOMALIES  istory and physical eamination a Coprehensive pregnanperinatal histor inluding infetions prenatal drugother exposures past edial histor developental histor b Detailed fail histor i At least three generations paing speial attention to siblings parents aternal ale relatives rst ousins ii Consanguinit ethniit siilarl aeted relatives aor ongenital anoalies global developental delaintelletual disabilitautis spetru disorder reurrent isarriages andor infertilit earl hildhood death  roth paraeters eight height head iruferene d hsial exaination Figure  tailored to phenotpe and suspeted geneti disease  Diagnostic orup a ailored to patient phenotpe and suspeted geneti disease b Consider i Midline orkup—head ultrasoundbrain agneti resonane iaging MRI ehoardiogra abdoinal ultrasound ii keletal surve iii Foral ee exaination iv Hearing test v eneral and etaboli laborator investigations based on presenting phenotpe eg seru holesterol and 7-DHC for suspeted ith-eli-pit sndroe  eneti testing should inlude pretest ounselling d First-tier test is CMA a in the future be aopanied b  or superseded b 

Genetics and Teratology

 sed in the setting of oplex nonspei phenotpes or a£er negative targeted testing in an individual strongl suspeted of having a geneti ondition  holegenome sequencing GS a Nontargeted test that attepts to seuene the genoe in a DNA saple b Can detet both seuene variation like  and hroosoal variation like CMA  ther disease-spei oleular tests that are not previousl listed eg fragile  sndroe DNA repeat expansion analsis via polerase hain reation CR iprinting disorder DNA ethlation ultiplex ligation-dependent probe apliation MA itohondrial DNA disorders itohondrialnulear DNA

18

4

ire 181 Physical Examination for enetic Syndrome Growth parameters: weight, length, head circumference Microcephaly: HC < 2 standard deviations below mean Macrocephaly: HC > 2 standard deviations above mean Skull: symmetry, shape, fontanelle (size), sutures (patency), scalp Hair: implantation (anterior and posterior hair line), teture pattern

Genetics and Teratology

yes: intercanthal distance, orientation, coloboma, eyelashes picanthal fold: sin fold at the inner corner of the eye where upper and lower eyelids meet Hypertelorism hypotelorism: increased (decreased) distance between pupils Palperal fissures:  pslantin: lateral corner of eyelid above medial corner  ownslantin: lateral corner of eyelid below medial corner owset ears: positioning of ear below a hypothetical line between occipit and outer canthus Mouth: cleft, shape of palate, lips, tongue (size, teture), teeth (eruption time, uality, number) Philtrum: shape (smooth philtrum flattening of the groove between nose and upper lip), length ose: shape of nasal root, bridge and tip, shape and position of nostrils ars: size, shape, placement, pitstags etronathia: posterior positioning of the mandible (micrognathia small mandible) eck: length, shape, webbing, pitsfistulas Thorax: shape, size, position of nipples, pectus deformities Spine: curvature, vertebral anomalies, sacral dimple

18

Viscera: size, shape, localization, etra masses Genitalia: hypoplasia, cryptorchidism, ambiguous genitalia, hypospadias Anus: patency, localization Hands and feet: creases, nails, digits (shape, number) Arachnodactyly: abnormally thin, long fingers and toes Clinodactyly: lateral or medial curvature of a digit Camptodactyly: fleure contracture of a digit Polydactyly: etra digit  Postaxial: additional digit is on the ulnar margin of the hand, or lateral to the fifth toe  Preaxial: additional digit is towards the first digit of the hand (radial side) or foot (medially) Syndactyly: webbingfusion of partwhole of two or more consecutive digits ims: proportions, reductionsamputations, missing parts

HC Head iruferene

SELECTED GENETIC CONDITIONS    

euenes and assoiations able  Chroosoal aneuploidies able  ingle gene disorders able  Mirodeletion sndroes and iprinting disorders able  PEARL

434

se conditionspecic standardied health spervision gidelines andor growth charts, if available.

able .2

Common Seences and Associations

Type

Etiopathoenesis

Sins and Symptoms

Clinical tcomes

Selected Inestiations

ierre obin seence

 andiblar hypoplasia → posterior displacement of the tonge → incomplete closre of the palate  solated , syndromic , e.g., ticler syndrome

 icrognathia  lossoptosis  Airway obstrction andor cleft palate

 espiratory distress  eeding difclties  avorable prognosis with expert srgical care

 ye examination after  year of age for signs of ticler syndrome  onsider A to rle ot chromosomal disorder e.g., 22.2 deletion syndrome

otter seence oligohydramnios

 enal anomaly→ obstrctive ropathy→ oligohydramnios→ deformation by constraint  ilateral renal dysgenesisagenesis 2

 otter facies ¤attened nose, wideset eyes, retrognathia, large lowset ears  ilateral plmonary hypoplasia  imb positioning defects  rowth deciency

 espiratory failre secondary to plmonary hypoplasia  Acte renal failre  ater chronic lng disease, chronic renal failre

 enal ltrasond, plmonary imaging, seletal xrays  onsider A to rle ot chromosomal disorder andor testing for specic genetic renal disorders

VA association ypical diagnostic criteria  anomalies some sggest at least tracheoesophageal stlaesophageal atresia or an anorectal anomaly mst be present

 poradic inheritance  iagnosis of exclsion

V 5vertebral defects A 5anorectal malformation C 5cardiac anomalies TE5tracheoesophageal stla esophageal atresia R 5renal anomalies L5limb defects particlarly radial ray anomalies

    

 chocardiogram, renal ltrasond  onsider xray of the spine and pper extremities  A to rle ot chromosomal disorder  onsider chromosome breaage stdies for anconi anemia particlarly if radial defect

espiratory distress eedinggrowth difclties enal failre ypical development avorable prognosis with expert srgical care  ater scoliosisbac pain, constipation, nephrolithiasis 

CMA, Chromosomal microarray; UTI, urinary tract infection.

18

4

Genetics and Teratology

Genetics and Teratology

18

436

able .

Common Chromosomal Aneploidies

Type

Inheritance

Early Clinical eatres

Later Clinical eatres

Selected Inestiations

Attention

risomy 2 own syndrome

 9 three copies of chromosome 2   nbalanced translocation between chromosome 2 and another chromosome often 4  2 mosaic  eneral recrrence ris  higher with maternal age and translocation carrier

 rachycephaly, mild microcephaly, small ears with overfolded helices, epicanthal folds, pslanting palpebral ssres, protrding tonge  earing loss  rsheld spots, myopia  ingle palmar creases, fth nger clinodactyly, sandal gap deformity  ypotonia  ardiac defects  e.g., A, V, AV   atresias 2 e.g., , dodenal atresiastenosis, irschsprng disease  ongenital hypothyroidism   ransient myeloproliferative disorder , polycythemia

 evelopmental and intellec tal disability  Asymptomatic atlantoaxial sblxation  bstrctive sleep apnea  ondctive hearing loss otitis media  Acte lymphoblastic leemia –2  besity  ip abnormalities dislocation, slipped capital femoral epiph ysis, avasclar necrosis  nfertility primary gonadal deciency  ataracts  arlyonset Alheimer disease

  with chromosome 2 probe for rapid diagnosis  aryotype if nbalanced translocation, then test parents  chocardiogram  ye examination  earing test  hyroid fnction, , differential  ervical xray

 arefl nerologic ex amination any change in bowelbladder fnc tion, change in gait, nec paintorticollis rle ot spinal cord compression  ncreased ris of celiac disease  ollow risomy 2 health spervision gidelines and growth chart

risomy  ata syndrome

 ajority have three copies of chromosome   arely mosaic or partial trisomy   eneral recrrence ris ,

 icrocephaly, depressed nasal bridge, lowset ears, ctis aplasia, micro anophthalmia, hearing defects, holoprosencephaly, cleft lippalate, omphalocele, polydactyly, clenched st  eart defects  A, V  enal anomalies , horseshoe idney

 ostnatal growth retardation  evere to profond develop mental and cognitive disability

  with chromosome  probe for rapid diagnosis  aryotype  chocardiogram  enal ltrasond  ead ltrasond

 edian srvival is –2 wees  rvival to  year 2 in some stdies  ongterm srvival possible

risomy  dwards syndrome

 ajority have three cop ies of chromosome   arely mosaic or partial trisomy   eneral recrrence ris , higher with maternal age

 rominent occipt, narrow bifrontal di ameter, small moth, micrognathia  hort sternm, overlapping ngers, rocerbottom feet  ntraterine growth restriction  eart defects 9 V 6 polyvalvlar dysplasia  enal anomalies , horseshoe idney  ryptorchidism

   

rowth retardation Apneic episodes eeding difclties evere to profond developmental and cognitive disability

onosomy  rner syndrome

  one  chromo some, no  chromosome  emainder are mosaic andor have strctral abnormalities involving the second  chromosome  eneral recrrence ris low

 ystic hygroma in tero, low posterior hairline, short webbed nec, pfness of hands and feet congenital lymphedema, broad chest, widely spaced nipples  eftsided heart defects – bicspid aortic valve, coarctation of aorta, mitral valve prolapse  enal anomalies horseshoe idney

 hort statre  trabisms, glacoma  ypertension 4, aortic root dilatation  rimary ovarian failre, no growth sprt, minimal breast development, ovarian dysgenesis 9, infertility  ypothyroidism, glcose intolerance, hyperlipidemia  ormal intelligence

  with chromosome  probe for rapid diagnosis  aryotype  chocardiogram  enal ltrasond

 edian srvival is –2 wees  rvival to  year is  in some stdies  ongterm srvival possible  ncreased ris for ilms tmor and hepatoblastoma

 aryotype – will have  chromosome in allsome cells   with  chromosome 6  chromosome probe  chocardiogram  enal ltrasond  earing test  hyroid fnction

 rowth hormone therapy at early age  ater estrogen therapy  ncreased ris for atoimmne diseases  ncreased ris for gonadoblastoma or dysgerminoma 7–  ollow rner yndrome health spervision gidelines and growth chart

ASD, Atrial septal defect; AVSD, atrioventricular septal defect; CBC, complete blood count; FISH, uorescence in situ hybridization; GI, gastrointestinal; PCKD, polycystic idney disease; TEF, tracheal esophageal stula; VSD, ventricular septal defect.

18

47

Genetics and Teratology

Genetics and Teratology

18

438

able .4

Selected Sinle ene isorders

Types

Inheritance

Clinical eatres

Later Clinical eatres

Selected Inestiations

Attention

Achondroplasia

 A  ost cases sporadic

 acrocephaly, disproportionate short statre, low nasal bridge, prominent forehead, mild midfacial hypoplasia  hort vertebral pedicles, lmbar lordosis, short bones, trident hand, samelength ngers, short femoral nec incomplete extension of elbow  ild hypotonia

 acrocephaly, hydro cephals, short statre, bowing of legs, otitis media short estachian tbes, childhood obesity  leep apnea  ervicomedllar jnction compression narrow fo ramen magnm  pinal stenosis

 argeted variant testing in the FGFR3 gene  ray of bones   assess foramen magnm sie in rst year of life  olysomnography in rst year of life repeat if apneas

 f large fontanelle, rapid h in  or symptoms of raised intracranial pressre brain imaging  iscorage sitting positions where trn crves anteriorly  ollow Achondroplasia health spervision gidelines and growth chart

A syndrome

 A  ost cases sporadic

C5coloboma, bilateral  H 5heart defects – conotrncal defects, A, V, coarctation A 5atresia choanae R5retarded growth andor development delay 7– G 5genital hypoplasia micropenis, cryptorchidism 7 E5ear anomalies andor deafness  abnormal semicirclar canals Cranial nerves:  hypoanosmia V facial palsy V hypoplasia of aditory nerve  swallowing difclties and aspiration  left lippalate –2  cell deciency

 hort statre, delayed absent pberty hypogonadotropic hypogonadism, consider hormonal replacement  ild to profond intellectal disability  coliosis

 oleclar testing of the CHD7 gene   temporal bones semicirclar canal hypoplasia  chocardiogram  enal ltrasond  ye examinations, adiologic evalations

 Aspiration ris  ostoperative airway problems  Venos malformations of temporal bone can lead to complications dring otologic srgery

ragile  syndrome

 lined  acrocephaly, prominent forehead, elongated face,   4– large ears, pale ble irides, epicanthal folds, high  arched palate, dental crowding, cataracts, strabisms,   – myopia   and ¤apping or biting , poor eye contact 9  Atism spectrm disorder   pilepsy

Infancy/childhood:  evelopmental disability, feeding difclties, otitis media ery:  ntellectal disability milder in females, anxiety  acroorchidism Adls:  itral valve prolapse 

 A moleclar analysis for trincleotide  repeat of the FR gene

 evelopmental followp  others obligate carriers for at least a “premtation”  remtation carriers are at ris of prematre ovarian failre and fragile associated tremorataxia syndrome  ollow ragile  health spervision gidelines

arfan syndrome

 A  2–  sporadic

 eonatal presentation rare  earning disability and attention decit disorder 42

 oleclar testing of the F gene  Annal echocardio gram, eye examination  onitor blood pressre, scoliosis

 Antibiotic prophylaxis shold be sed before dental procedres  Avoid isometric exercise, contact sports, scba diving  onsider ßblocer as pro phylaxis for aortic dilatation  ollow arfan yndrome health spervision gidelines

 ctopia lentis  Aortic root enlargement  ee able . for systemic score criteria a score of 7 is considered positive

Conined

18

49

Genetics and Teratology

Genetics and Teratology

18

440

able .4

Selected Sinle ene isorders—cont’d

Types

Inheritance

Clinical eatres

Later Clinical eatres

Selected Inestiations

Attention

oonan syndrome

 A  ost cases sporadic

 raniofacial woollie hair crly, epicanthal folds, downslanting palpebral ssres, ptosis of eyelids, hypertelorism, low nasal bridge, lowsetabnormal ears, dental malocclsion, protrding pper lip, retrognathia, low posterior hairline, webbed nec  ects excavatmcarinatm, scoliosisvertebral abnormalities  ardiac – plmonary valve stenosis, hypertrophic cardiomyopathy  ilateral cryptorchidism 

Infancy:  hort statre   poor feeding, vomiting, constipation Laer:  ildmoderate cognitive disability  trabisms, refractive errors  leeding diathesis –9 thrombocy topenia, platelet dysfnction

 oleclar genetic test ing with  panel for oonan syndrome and related disorders  chocardiogram  ye examination  , coaglation screen

 2 with cardiomyopathy die in the rst 2 years of life  yelomonocytic leemia in the rst 2 months of life  ormal fertility in females and males with descended testes  acial featres change with age  ollow oonan yndrome health spervision gidelines

mith–emli– pit syndrome

 A

 raniofacial microcephaly, ptosis of eyelids, epican thal folds, strabisms, broad nasal tip, micrognathia  2– toe syndactyly, postaxial polydactyly  ardiac defect  endocardial cshion defect, hypoplastic left heart, A  enital abnormalities 7 hypospadias, cryptorchidism  enal anomalies 7 obstrction, agenesis   seires, holoprosencephaly   ptic cataract, optic atrophy, nystagms   rectal atresia, pyloric stenosis, intestinal malrota tion, diaphragmatic hernia, irschsprng disease  ther cleft palate, bid tonge, hearing loss, thyms hypoplasia

 eeding difclties . reire feeding tbe in childhood  leep distrbances in infancy  oderate to severe intellectal disability  ehavioral atism, self injrios, aggressive, forcefl bacward arching

 linical and biochemical diagnosis h 7 in blood h atio of 7 cholesterol 6 gholesterol 9 cases  oleclar testing of the DHCR7 gene  chocardiogram  enal ltrasond

ife expectancy determined by severity of internal malforma tions and ality of general spportive care

7-DHC, ehydrocholesterol; AD, autosomal dominant; AR, autosomal recessive; ASD, atrial septal defect; CBC, complete blood count; CNS, central nervous system; CT, computed tomography; DNA, deoxyribonucleic acid; , failure to thrive; GERD, gastroesophageal reux disease; GI, gastrointestinal; HC, head circumference; MRI, magnetic resonance imaging; NGS, nextgeneration seuencing; VSD, ventricular septal defect.

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Genetics and Teratology

able . Systemic Score Criteria for arfan Syndrome a eatre

Score

rist A A thmb  sign

Genetics and Teratology 18

A

1

B

rist  thmb sign

1

ects carinatm deformity

12

ects excavatm or chest asymmetry

1

indfoot deformity

12

es plans

1

pontaneos pnemothorax

12

ral ectasia

12

rotrsio acetabli

12

coliosisthoracolmbar yphosis

1

edced elbow extension

1

acial featres   olichocephaly, enophthalmos, downslanting palpebral ssres, malar hypoplasia, retrognathia

1

in striae

1

evere myopia . diopters

1

itral valve prolapse

1

pperlower segment ratio ,. acasian or ,.7 lac A arm spanheight .. ower segment—distance from top of symphysis pbis to ¤oor when standing

1

a

core of ≥ is considered positive.

ata from .marfan.orgdxscore. mages from cride A, argan . arfan syndrome. Curr Orthop. ;1–.

442

able .

Selected icrodeletion Syndromes Incldin Imprintin isorders

Type

Inheritance

Early Clinical eatres

Later Clinical eatres

Selected Inestiations

Attention

22.2 deletion syndrome ieorge syndrome

.9 sporadic  A

 left palate  ongenital cardiac disease e.g., conotrncal anomalies, V, right aortic arch  eonatal hypocalcemia hypoparathyroidism  cell immnodeciency thyms hypoplasia  enal tract anomalies  trctral brain defects

Childhood:  elayed motor milestones waling –24 months, speech and langage  ypotonia 7– leading to feeding difclties, hypernasal speech  earning difclties .9, with or withot intellectal disability Adls:  chiophrenia, generalied anxiety disor der, other psychiatric conditions  arlyonset arinson disease

 A or  with probe for 22.2 region if high index of sspicion  chocardiogram, renal ltrasond, adiogram  onitor calcim,   mmnologic stdies immnophenotyping, specic serologies

 bstrctive sleep apnea following pharyngeal sr gery to improve speech  o live vaccines ntil im mne fnction checed  f transfsion necessary, V negative and irra diated if , months of age  ollow 22.2 health spervision gidelines and growth chart

illiams syndrome

.9 sporadic  A

 ild microcephaly, prematre gray hair, medial eyebrow ¤are, short palpebral ssres, depressed nasal bridge, epicanthal folds, ble eyes, stellate pattern in the iris, long philtrm, anodontia, microdontia  ardiac spravalvlar aortic stenosis, AV, peripheral plmonary artery stenosis  ephrocalcinosis, asymmetry in idney sie, bladder diverticla, rethral stenosis, vesicoreteral re¤x

Infancy:  eeding problems, freent vomiting, colic

 A or  with probe for 7.2 region if high index of sspicion  chocardiogram, renal ltrasond  ye examination, hearing test  rinalysis, calcim creatinine ratio, renal fnction  onitor serm calcim, thyroid fnction

 ncreased incidence of celiac disease  ation regarding vitamin  spplementation  dden death associ ated with anesthesia  ollow illiams yndrome health spervision gide lines and growth chart

Childhood:  A, anxiety, average intelligence, hoarse voice Adls:  ypertension, joint limitations, recrrent , obesity, constipation, diverticlosis, cholelithiasis, hypercalcemia, hypercalci ria , hypothyroidism 

18

44

Conined

Genetics and Teratology

Genetics and Teratology

18

444

able .

Selected icrodeletion Syndromes Incldin Imprintin isorders—cont’d

Type

Inheritance

Early Clinical eatres

Later Clinical eatres

Selected Inestiations

Attention

Angelman syndrome

 poradic dele tion of maternal – 7–7  aternal  –7  mprinting de fect 2–  Variants in the E3A gene in 

icrobrachycephaly, blond hair , pale ble eyes , maxillary hypoplasia, prognathia large moth with tonge protrsion and widely spaced teeth

 evere developmentalintellectal disability, speech impairment  ovement disorder ataxiatremors, seires , hypotonia, hyperre¤exia, left hand preference  reent laghingsmiling, hand ¤apping, drooling, excessive chewing  trabisms 42, refractive errors, nystagms, scoliosis, hypopigmentation 9

 irsttier test A methylation analysis of chromosome   region  f negative, moleclar testing of E3A gene  , brain imaging as indicated

 besity and constipation in adolescence and adlthood

rader illi syndrome

 poradic dele tion of paternal chromosome – 7  aternal  2–  mprinting defects ,

 evere central hypotonia at birth improves with age  Almondshaped eyes with pslanting palpebral ssre, strabisms, thin pper lip  mall hands and feet  enital hypoplasia, cryptorchidism

Infancy:  espiratory and feeding difclties tbe feeding Childhood:  apid weight gain 2 years, increase interest in food 4. years, hyperphagia, inappropriate food seeing, binge eating  years, mild intellectal disability Laer:  besity, diabetes, hypothyroidism, short statre, osteoporosis, scoliosis, tempera tre instability, high pain threshold, geni tal hypoplasia, amenorrhea

 A methylation analy sis of chromosome  region

 arly growth hormone therapy benecial for body composition, even if growth is normal  Adolescents may reire testosterone replacement therapy

ecwith– iede mann syn drome

 ostly sporadic  Aberrant meth ylation –   aternal  2  Variants in the CDC gene in  more in familial cases

 acrosomia, macroglossia, hemihyper plasia, prominent eyes with infraorbital hypoplasia, large fontanelles, nderde veloped maxilla  Visceromegaly cardiac, renal, omphalocele, diastasis recti, posterior diaphragmatic eventration, cryptorchidism, cardiovasclar defects  olycythemia, hypoglycemia –  eires

Childhood:  mbryogenic malignancy ilms tmor, hepatoblastoma, neroblastoma, rhabdomyosarcoma present by  years ris 4–2 Adls:  earing loss, anerysmal arterial dilatations, male infertility, nephrocalcinosis, medllary sponge idney disease

 A methylation analysis of chromosome p region  ray bone accelerated osseos matration  enal ltrasond nephrocalcinosis  alignancy srveillance renal ltrasond, serm alphafetoprotein every  months ntil 4– years

 ncreased ris of malig nancy highest if hemihy pertrophy and nephro megaly

AD, Autosomal dominant; ADHD, attention decit hyperactivity disorder; ASD, atrial septal defect; CBC, complete blood count; CMA, chromosomal microarray analysis; CMV, cytomegalovirus; DNA, deoxyribonucleic acid; EEG, electroencephalogram; FISH, uorescence in situ hybridization; UPD, uniparental disomy; UTI, urinary tract infection; VSD, ventricular septal defect.

18

44

Genetics and Teratology

TERATOLOGY

Genetics and Teratology 18

 Denition: teratologi agents are heial phsial or biologial agents that an daage ebroni tissue resulting in alforations  tiologies a Maternal infections: see Congenitalerinatal Infetions in Chapter  Infetious Diseases b Maternal disease untreated or poorly controlled i Diabetes ellitus not gestational ide spetru of alforations eg neural tube defets NDs ongenital ardia defets saral agenesis if poor glei ontrol during earl ebrogenesis ii Hpertension groth restrition iii Maternal phenlketonuriahperphenlalanineia intelletual disabilit iroephal ongenital heart disease iv ©roid disease hper—groth restrition hpo—intelletual deits v stei lupus ertheatosus sall for gestational age neonatal lupus ongenital heart blok anti-RoA andor anti-a antibodies  adiation: exposure of about  rad an result in isarriages iroephal ognitive ipairent and skeletal alforations exposure to the fetus fro a to-vie hest x-ra of the other is , rad d Medications and chemicals i ee able 7 for seleted ediations and possible eets on the fetus ii Alohol onsuption . ounesda at risk of fetal alohol spetru disorder Aets groth height eight , faial features iroephal epianthal folds sall palpebral ssures at nasal bridge upturned nose sooth philtru thin upper lip neurodevelopent iii oking at risk of isarriage preter labor groth restrition sudden infant death sndroe able .7

446

Selected edications and Possile Effects

edication

Possile Effects on the ets

A inhibitors

enal tblar dysgenesis

sotretinoin

acial and ear anomalies, congenital heart disease

ithim

bstein anomaly

A

rematre closre of dcts arterioss

henobarbital

Vitamin  deciency

henytoin

, hypoplastic nails, facial and ear anomalies, microcephaly

Valproatecarbamaepine

ognitive disability, 

arfarin

acial dysmorphisms, chondrodysplasia, developmental disability

ACE, Angiotensin converting enzyme; IUGR, intrauterine groth restriction; NSAID, nonsteroidal antiinammatory drug; NTD, neural tube defects.

 ndication: undiagnosed hild ith dsorphiss andor ultiple ongenital anoalies ho is oribund or reentl deeased  orup a ank DNA b lood to spots on lter paper  lasa   heparinied saple d rine   to be froen at 2°C e Cerebrospinal uid CF   to be froen f kin biops for broblast ulture - punh biops plae in ell ulture ediu or sterile saline keep at roo teperature if , hours or 1°C if longer do not freee g Musle and liver biopsies froen at 27°C h Clinial photographs i keletal x-ras

Genetics and Teratology

PERIMORTEM WORKUP IN CHILD WITH DYSMORPHISMS

18

447

CHAPTER

19

Growth and Nutrition Justin Lam • Laura Kinlin • Meta van den Heuvel Dietitians: Mara Alexanian-Farr, Alisa Bar-Dayan, Kelsey Gallagher, Daina Kalnins, Alissa Steinerg, Lri uira, Laura res, Kellie elh, Jrdan Beaulieu Lactation Consultants: arline urrie, Laura MLean, Samantha Sullivan Occupational erapist: Ashley Graham

Common abbreviations Energy requirements Assessment of growth Enteral feeding Vitamin and mineral supplementation Parenteral nutrition Failure to thrive Obesity Micronutrient deciencies

448

COMMON ABBREVIATIONS BMI BMR CMPA RI BM R  R  I  IB PC P  I

body mass index basal metabolic rate cow’s milk protein allergy dietary reference intake expressed breast milk estimated energy reuirement failure to thrive gastroesophageal reux disease human immunodeciency virus human cell lymphotropic viruses ideal body weight polycystic ovary syndrome parenteral nutrition total energy expenditure total uid intake

Growth and Nutrition

Also see page xviii for a list of other abbreviations used throughout this book

ENERGY REQUIREMENTS  nergy reuirements can be estimated to help establish goals for adeuate calorie provision 19  Predictive euations provide only estimates for energy needs and should be used as a guideline only  A number of methods are used to estimate the energy needs of children in a clinical setting a Assessing energy reuirements using the orld ealth rganiation  prediction euations estimate basal metabolic rate BMR and total energy expenditure  i e  euation calculates BMR by gender age and weight able  ii otal daily energy reuirements are estimated by multiplying the calculated BMR by activity factor A 6 stress factor  to adust for physical activity medical status andor need for catch up growth 6 tone factor for patients with spasticity ee able  for factors b Assessing energy reuirements using dietary reference intakes RIs for energy i RIs are a set of reference values used in Canada and the nited tates for both individuals and groups RIs are based on chrono logical age and not on developmental stage RI values are for healthy individuals therefore adustments for disease and nutritional status may be reuired ee able  for estimated energy reuire 449 ments based on RIs for energy

ale 19.1

Euations for asal Metaolic Rate (FAH 1991)

Age Range (Years)

Males (calay)

Feales (calay)

0–

0.9 – 54

1.0 – 51

–10

22.7 1 495

22.5 1 499

10–18

17.5 1 51

12.2 1 74

FAO, Food and Agriculture Organization; UNU, United Nations University; W, weight in kg; WHO, World Health Organization.

Growth and Nutrition 19

Adapted ro he Hospital or ick hildren uidelines or the Adinistration o nteral and arenteral Nutrition in aediatrics, .

ale 19.2

Factors for Activity Stress an Muscle Tone in FA H Euations for Estiating Total Energy Eeniture (nstitute of Meicine )

Situation

Activity Factor

Situation

Activity Factor

aralye coatose

0.8–1

Sepsis

1.2–1.4

Conne to e

1.15

Elective surgery

1–1.1

epenent

1.2

Starvation

0.7–0.85

Craling

1.25

Cancer

1.1–1.45

Seentary

1.–1.5

Burns

1.2–2

oral activity

1.7

Multiple traua

1.4

tlete

2

1.1–1.

Situation

Stress factor

Multiple long one racture

ever

1.2 per 1°C .7°C

Situation

Muscle tone factor

ecrease tone

0.9

Severe inection

1.2–1.

oral tone

1

eritonitis

1.05–1.25

ncrease tone

1.1

FAO, Food and Agriculture Organization; UNU, United Nations University; WHO, World Health Organization. Adapted ro he Hospital or ick hildren uidelines or the Adinistration o nteral and arenteral Nutrition in aediatrics, .

PEAR otal energy epeniture 5 asal etaolic rate 3 activity actor 3 stress actor 3 tone actor Bracets inicate to inclue i applicale

450

Estiate Energy Reuireent (EER) for nfants an Cilren as er ietary Reference ntaes for Energy

ale 19.

EERa (kcal/kg/day) Age

Se

Pysical Activity evel (PA)a

0–2

M 

107 104–102

–

M 

95–82 95–82

7–9

M 

79–80 80

10–20

M 

79–82 82

21–5

M 

82–8 8 Seentary

o Active

Active

ery Active

Growth and Nutrition

nfants an Cilren (onts)

Cilren (years) –4

M 

81–75 78–72

9–8 89–8

104–97 100–94

117–109 118–111

5–

M 

9–4 –2

80–74 77–72

90–84 87–82

10–97 104–97

7–8

M 

0–57 57–5

70– 7–2

80–75 75–71

92–87 90–85

9–10

M 

54–50 49–45

–59 57–5

71–7 5–0

8–78 78–72

11–12

M 

47–44 41–9

55–52 49–4

4–0 5–5

74–70 7–4

1–14

M 

42–41 7–5

50–48 44–41

57–5 50–47

7–4 0–57

15–1

M 

40–8 –2

47–45 40–8

54–52 45–44

2–0 55–54

17–18

M 

7– 1–0

4–42 7–

50–49 4–42

58–57 52–51

19

a

A or inants not deterined.

F, Feale; M, ale. Adapted ro he Hospital or ick hildren uidelines or the Adinistration o nteral and arenteral Nutrition in aediatrics, .

451

ASSESSMENT OF GROWTH  Minimum parameters of growth assessment weight heightlength head circumference  ingle measurements can be compared with normal population values by plotting on  growth charts igures – Figure 191 oys irt– Monts engtforAge an eigtforAge Percentiles Growth and Nutrition 19

BOYS

WHO OWH H O  BIRTH TO 24 MONTHS: BOYS Length-for-age and Weight-for-age percentiles Birth

L E N G T H

in 39 38 37 36 35 34 33 32 31 30 29 28 27 26 25 24 23 22 21 20 19 18 17 16 15 14 13 12 11 10 9 8 7

2

4

6

8

10

c

NAME:

12

14

16

18

20

22

24

in 39 38 37 36 35 34 33 32 31 30 29

c

AGE (MONTHS)

95

95 97

90

90

85 50

85

85

15 3

80

80

75

75

70 65

17

60

16

55

15

97

38 36 34 32

14

50

30

85

45

13

40

28

12

50

26

11

24

10

22

25

9

20

20

8

35 15

30 3

18 16

7 14

6 10

5 10 8 6 4 l

12

14

g

AGE (MONTHS)

12 W E I G H T

L E N G T H

14

16

18

20

22

l

24

OH’ HH H’ HH

4



3

OL   H  H

LH

WH

W

O

2 kg Birth

2

4

6

8

SOURCE: Based on World Health Organization (WHO) Child Growth Standards (2006) and WHO Reference (2007) and adated for Canada  Canadian aediatric Societ Canadian ediatric ndocrine Gro College of ail hsicians of Canada Conit Health rses of Canada and ietitians of Canada © ietitians of Canada 20 Chart a e rerodced in its entiret (ie no changes) for noncoercial roses onl

rom  rowth Charts et  ietitians of Canada  Accessed at wwwdietitiansca

452 growthcharts

W E I G H T

Figure 19 irls irt– Monts engtforAge an eigtfor Age Percentiles GIRLS

WHO OWH H O  BIRTH TO 24 MONTHS: GIRLS Length-for-age and Weight-for-age percentiles

L E N G T H

2

4

6

8

10

14

12

c

16

18

20

22

24

c

AGE (MONTHS)

95

95 97

90

90

85 50

85

85

15

80

80

3

75

75

65

17

60

16

55

15

45

14 13

85

40

12 50

35

4 l

30 28 26

11

24

30

15

10

22

25

3

9

20

20

8

6

g

AGE (MONTHS)

10

10

6

36

32

97

50

5

8

38

34

12 W E I G H T

L E N G T H

70

7 14

in 39 38 37 36 35 34 33 32 31 30 29

12

14

16

18

20

22

Growth and Nutrition

Birth

in 39 38 37 36 35 34 33 32 31 30 29 28 27 26 25 24 23 22 21 20 19 18 17 16 15 14 13 12 11 10 9 8 7

NAME:

24

W E I G H T

19

18 16 14 l

OH’ HH OL   H

4 

3

 H

LH

WH

O

2 kg Birth

2

4

6

8

SOURCE: Based on World Health Organization (WHO) Child Growth Standards (2006) and WHO Reference (2007) and adated for Canada  Canadian aediatric Societ Canadian ediatric ndocrine Gro College of ail hsicians of Canada Conit Health rses of Canada andietitians of Canada © ietitians of Canada 20 Chart a e rerodced in its entiret (ie no changes) for noncoercial roses onl

rom  rowth Charts et  ietitians of Canada  Accessed at wwwdietitiansca growthcharts

45

Figure 19 oys irt– Monts Hea Circuference an eigtfor engt Percentiles BOYS

WH WH CH  C BIRTH TO 24 MONTHS: BOYS Head Circumference and Weight-for-length percentile Birth in

H   

Growth and Nutrition

C  C        C 

21 20 19 18 17 16

8

10

14

12

16

18

20

22

24 cm

AGE (MONTHS)

54

54

52

52 97

50

85

48

50 15

46

3

50

21 20

48

19

46

18

44

44

in

17 54

24

15

38

23

14

36

22

34

21

46

20

44

19

42

18

40

17

38

13

99.9

97

32 12

34 32

30

85

50

17 16

15

15

3

16 15

52 50 48

36 34 32

14

14

13

13

26

12

12

26

24

11

11

24

22

10

10

22

20

9

9

20

18

8

8

7

7

30 28

 H 

6

40

36

W 

4

cm

42

38

19

2

NAME:

16 14

6

12 10 8 6 lb

kg

LENGTH

70 72 74 78 76 80 82 84 86 88 90 92 94 96 98 100 102 104 106cm 108

5 27

4

28

29

30

31

32

GETATINAL AGE AT BIRTH DATE AGE BIRTH

3

33

34

35

36

37

38

39

40

41

42

30 28

18 16 14 12

in

lb

WEE LENGTH

WEIGHT

HEAD IR

ENT

2

kg cm in 18

46 48 50 52 54 56 58 60 62 64 66 19

20

21

22

23

24

25

26

SOURCE: Based on World Health Organization (WHO) Child Growth Standards (2006) and WHO Reference (2007) and adated for Canada  Canadian aediatric Societ Canadian ediatric ndocrine Gro College of ail hsicians of Canada Conit Health rses of Canada andietitians of Canada © ietitians of Canada 20 Chart a e rerodced in its entiret (ie no changes) for noncoercial roses onl

rom  rowth Charts et  ietitians of Canada  Accessed at wwwdietitiansca growthcharts

454

W   H 

Figure 19 irls irt– Monts Hea Circuference an eigtfor engt Percentiles GIRLS

WH RWH CHAR FR CANADA BIRTH TO 24 MONTHS: GIRLS Head Circumference and Weigfreng ercenie Birth in

C R C U M F E R E N C E

4

6

8

10

14

12

16

18

20

22

24 cm

AGE (MONTHS)

cm

52 20 19

52

50

97 85

48

18 17

46

15 3

44

20

48

19

46

18

16

40

15

38

14

36

13

40 38 36 34 32

17

25 24 99.9

34

97

30 28

18 50

17 16

15

15

54 52

23 22

32 12

44 42

42

85

50 48

21

46

20

44

19

42

18

40

17

38

16 15

3

36 34 32

14

14

13

13

26

12

12

26

24

11

11

24

22

10

10

22

20

9

9

20

8

8

7

7

30 28

G H T

50

50

11

W E

in

18 16 14

6

8 6 lb

kg

LENGTH

12 10

Growth and Nutrition

H E A D

2

NAME:

0 2 4 6 8 80 82 84 86 88 0 2 4 6 8 00 02 04 06 cm 08

5 2

4

28

2

30

3

32

EAINA AE A IRH DAE AE IRH

3

33

34

35

36

3

38

3

40

4

42

30 28

W E G H T

19

18 16 14 12

in

lb

WEE ENH

WEIH

HEAD CIRC

CMMEN

2

kg cm in

46 48 50 52 54 56 58 60 62 64 66 18

19

20

21

22

23

24

25

26

SOURCE: Based on World Health Organization (WHO) Child Growth Standards (2006) and WHO Reference (2007) and adated for Canada  Canadian aediatric Societ Canadian ediatric ndocrine Gro College of ail hsicians of Canada Conit Health rses of Canada andietitians of Canada © ietitians of Canada 20 Chart a e rerodced in its entiret (ie no changes) for noncoercial roses onl

rom  rowth Charts et  ietitians of Canada  Accessed at wwwdietitiansca growthcharts

455

Figure 19 oys –19 Years HeigtforAge an eigtforAge Percentiles BOYS

WHO OWTH CHT O C 2 TO 19 YEARS: BOYS Heightorage and Weightorage ercentiles 2 in

Growth and Nutrition

H E G H T

19

80 9 8   5  3 2 1 0 9 8   5  3 2 1 0 59 58 5 5 55 5 53 52 51 50 9 8   5  3 2 1 0 39 38 3 36 35 34 33 32 31

3

4

5

7 6

8

cm

9

NAME:

10

11

12

13

14

15

16

17

18

19 cm

AGE (YEARS) OTHE’ HEIGHT THE’ HEIGHT

195

195 97

190 185

85

180

190 185 180

50

175 170

15

175 170 165

165 3

160

160

155

155

150

150

145

145

97

135

90 85

130 85

125

80

120

5

115

0

50

5

110

G H T

190 180 170 160 150 140

0

130

55

120

95

50

110

90

5

100

85

0

80

35

105

15

100

3

90 80 70 60

25

20

20

15

15

20

10

10

20

lb

kg

kg

lb

40 30

WHO recommends I s e bes mesre er ge 10 de o rible ge o ber Trcking eig lone is no dised

AGE (YEARS)

2

3

4

5

7 6

8

9

10

11

12

13

14

15

16

17

18

50 40 30

19

SOURCE: The main chart is based on World Health Organization (WHO) Child roth tandards (200) and WHO eerence (200) adated or Canada b Canadian aediatric ociet Canadian ediatric ndocrine ro (C) College o amil hsicians o Canada Commnit Health rses o Canada andietitians o Canada The eightorage10 to 19 ears section as deeloed b C based on data rom the  ational Center or Health tatisticssing the same rocedres as the WHO groth charts © ietitians o Canada 201 Chart ma be rerodced in its entiret (ie no changes) or noncommercial roses onl

rom  rowth Charts et  ietitians of Canada  Accessed at wwwdietitiansca growthcharts

456

 H T

200

25

50

H 

140

30

W E

in

80 79 78 77 76 75 74 73 72 71 70 69 68 67 66 65 64 63 62 61 60 59 58 57 56

W   H T

Figure 19 irls –19 Years HeigtforAge an eigtforAge Percentiles GIRLS

WHO GROWTH CHRT OR C 2 TO 19 YEARS: GIRLS Height-for-age and Weight-for-age percentiles

H  G H T

78 77 7 75 7 7 72 71 70 9 8 7  5   2 1 0 59 58 57 5 55 5 5 52 51 50 9 8 7  5   2 1 0 9 8 7  35 34 33 32 31 30 29

3

4

5

7 6

8

cm

9

10

11

12

13

14

15

16

17

18

19 cm

AGE (YEARS) OTH’ HHT TH’ HHT

190

190

185

185

180

180

175

97

175

170

85

170

165

50

165 160

160 15

155

3

150

155 150

145

145

140

140

50

G H T

40 30

H  G H T

135 130

90

125

85

120

80 97

115

75 70

110 85

105 100

50

95 90

15

85

3

5

100

20

20

15

15

2

3

4

5

7 6

8

9

10

11

12

13

14

15

16

17

18

140

5

25

AGE (YEARS)

150

110

25

kg

160

50

35

lb

170

120

75

WHO recommends BMI as the best measure after age 10 due to variable age of puberty. racing eight alone is not advised.

180

55

0

10

190

130

80

20

200

0

0

W 

in

78 77 76 75 74 73 72 71 70 69 68 67 66 65 64 63 62 61 60 59 58 57 56 55 54

Growth and Nutrition

2 in

NAME:

W  G H T

19

90 80 lb

60 50 40 30

10

20

kg

lb

19

SOURCE: The main chart is based on World Health Organiation WHO hild roth tandards 2006 and WHO eerence 2007 adated or anada b anadian aediatric ociet anadian ediatric ndocrine ro  ollege o amil hsicians o anada ommnit Health rses o anada andietitians o anada The eightorage10 to 19 ears section as deeloed b  based on data rom the  ational enter or Health tatisticssing the same rocedres as the WHO groth charts © ietitians o anada 2014 hart ma be rerodced in its entiret ie no changes or noncommercial roses onl

rom  rowth Charts et  ietitians of Canada  Accessed at wwwdietitiansca growthcharts

457

Figure 19 oys –19 Years oy Mass neforAge Percentiles

BOYS

W W    2 TO 19 YEARS: BOYS Body mass index-for-age percentiles





W



NAME:

B



B 39 38 37

Growth and Nutrition

99.9

35 B talescalclator aailale at www.whogrowthcharts.ca To Cacat BM: Weigt g ÷ eigt cm ÷ eigt cm x 10000 OR Weigt l ÷ eigt in ÷ eigt in x 703

34 33

B

32

31

31

30

30

29

29

97

28

28

27

27

26

26 85

25

19

36

25

24

24

23

23

22

22

50

21

21 20

20 15

19 18

19 18

3

17

17

16

16

15

15

14

14

13

13

12

12

B

B AGE (YEARS)

2

3

4

5

6

7

8

9

10

11

12

13

14

15

16

17

18

19

SOURCE: Based on World ealt rganiation W ild rot tandards 2006 and W eference 2007 and adapted for anada y anadian aediatric ociety anadian ediatric ndocrine rop ollege of amily ysicians of anada ommnity ealt rses of anada andietitians of anada © ietitians of anada 2014 art may e reprodced in its entirety ie no canges for non-commercial prposes only

www.whogrowthcharts.ca

rom  rowth Charts et  ietitians of Canada  Accessed at wwwdietitiansca growthcharts

458

Figure 19 irls –19 Years oy Mass neforAge Percentiles GIRLS

W W    2 TO 19 YEARS: GIRLS Body mass index-for-age percentiles





W

NAME:



B



B 40 39 38

36

B talescalclator aailale at www.whogrowthcharts.ca To Cacat MI: Weigt g ÷ eigt cm ÷ eigt cm x 10000 OR Weigt l ÷ eigt in ÷ eigt in x 703

35

B

34

33

33

32

32

31

31

30

30

29

29

97

28

28

27

27

26

26

25

25

85

24

24

23

23

22

22 50

21

Growth and Nutrition

37

99.9

21

20

19

20

19

19

15

18

18 17

17

3

16

16

15

15

14

14

13

13

B

B AGE (YEARS)

2

3

4

5

6

7

8

9

10

11

12

13

14

15

16

17

18

19

SOURCE: Based on World ealt rganiation W ild rot tandards 2006 and W eference 2007 and adapted for anada y anadian aediatric ociety anadian ediatric ndocrine rop ollege of amily ysicians of anada ommnity ealt rses of anada andietitians of anada © ietitians of anada 2014 art may e reprodced in its entirety ie no canges for non-commercial prposes only

www.whogrowthcharts.ca

rom  rowth Charts et  ietitians of Canada  Accessed at wwwdietitiansca growthcharts

 erial measurements provide information about a child’s growth velocity and changes in growth pattern over time ee able  for expected gain by day for infants and children  Premature infants , weeks should be plotted on the enton growth chart see igures  and  in Chapter  eonatology until

459

 weeks gestational age once termcorrected growth parameters should be plotted on the  charts using corrected age until  years  pecic growth charts are available for conditions with particular growth patterns eg risomy  Praderilli syndrome urner syn drome achondroplasia cerebral palsy hen such charts are available children should be plotted on both  and conditionspecic charts  ains in weight height and head circumference may be used to compare with predicted norms able  and able  Growth and Nutrition

EHT  eigh infants without clothes and diaper  erm neonates may lose up to  of birth weight in rst week of life should regain weight by at least  weeks of age then gain approximately  to  gday for rst  months of life  eight is a sensitive indicator of energy intake however nonnutritional causes eg edema or dehydration of weight changes should be considered

ale 19.4

Reference ains in eigt engt an Hea Circuference of Ter nfants an Reference ains in eigt an Heigt of Cilren Boys

19 eigt (gay)

engt Heigt ( ay)

Hea Circuference (ay)

eigt (gay)

0–1 onts

0

1.10



2

1.0



1–2 onts

5

1.09

0.8

29

1.01

0.78

2– onts

2

1.02

0.50

2

0.94

0.47

–4 onts

20

0.84

0.

19

0.81

0.4

4–5 onts

17

0.8

0.29

1

0.7

0.28

5– onts

15

0.

0.24

14

0.

0.2

–9 onts

1

0.52

0.18

12

0.59

0.18

9–12 onts

10

0.4

0.1

10

0.47

0.1

12–18 onts

7.

0.

0.08

7.

0.7

0.09

18–24 onts

.2

0.0

0.05

.7

0.1

0.05

24–0 onts

5.7

0.25

0.04

5.

0.2

0.04

0– onts

5.

0.22

0.0

5.

0.2

0.0

.5 years

5.4

0.21

5.5

0.21

4 years

5.2

0.20

5.4

0.20

4.5 years

5.8

0.19

5.2

0.19

Age

460

Girls engt Heigt Hea ( Circuference ay) (ay)

Boys

Girls

eigt (gay)

engt Heigt ( ay)

5 years

5.8

0.19

5.7

0.19

5.5 years

.4

0.19

5.

0.19

 years

.5

0.18

.2

0.18

.5 years

.4

0.18

.5

0.17

7 years

.9

0.18

.5

0.17

7.5 years

7.

0.1

7.

0.17

8 years

8.4

0.17

7.5

0.17

8.5 years

7.9

0.1

8.1

0.1

9 years

8.

0.1

8.7

0.1

9.5 years

8.5

0.15

8.4

0.15

10 years

9.

0.15

8.

0.1

10.5 years

8.9

0.14

10.8

0.15

11 years

9.5

0.14

11.0

0.17

11.5 years

10.5

0.14

1.

0.18

12 years

11.0

0.15

15.1

0.18

12.5 years

1.5

0.15

15.2

0.18

1 years

15.4

0.18

11.8

0.15

1.5 years

1.8

0.19

12.7

0.11

14 years

18.

0.22

10.1

0.07

14.5 years

20.2

0.20

8.2

0.05

15 years

17.8

0.18

5.5

0.04

15.5 years

14.2

0.11

5.1

0.0

1 years

12.0

0.09

2.0

0.0

1.5 years

9.

0.05

4.1

0.01

17 years

.7

0.05

.5

0.01

17.5 years

.0

0.02

2.2

0.00

18 years

4.2

0.02

2.

0.00

Age

Hea Circuference (ay)

eigt (gay)

engt Heigt Hea ( Circuference ay) (ay)

Adapted ro he Hospital or ick hildren uidelines or the Adinistration o nteral and arenteral Nutrition in aediatrics, .

Growth and Nutrition

ale 19.4

Reference ains in eigt engt an Hea Circuference of Ter nfants ains in eigt an Heigt of Cilren—cont’

19

41

ale 19.5

Growth and Nutrition 19

Average rot Paraeters an Rate of rot irt

Rate of rot

eigt

–.5 g

2 3 irt eigt y 4–   3 irt eigt y 1 year 4 3 irt eigt y 2 year

eigt

50 c

25 c in rst year 12 c in secon year 2 3 irt eigt y age 4 year ten 5–8 cyear until puerty

ea circuerence

5 c

2 cont until  onts 1 cont ro  to  onts 0.5 cont ro  to 12 onts

ETH  Measure recumbent length until age  years with a length board then standing height using a stadiometer  o estimate child’s adult height from parental heights a Boy’s height father’s height in cm 1 mother’s height in cm 1  cm b irl’s height father’s height in cm 1 mother’s height in cm 2  cm HEA CRCMFERECE  ess sensitive indicator of shortterm nutritional status is closely related to brain growth and is inuenced by nutritional status until  months of age  Measurement is taken using a exible tape measure placed around the largest occipitofrontal circumference EHT FR HEHT A Y MASS E  creening tool for wasting overweight and obesity able   In children $ years use body mass index BMI for age  In children , years use weightforheight or percent ideal body weight IB o calculate IB a Plot height to determine height percentile b ind the same percentile and determine weight IB c IB 5 Child’s actual body weightIB 3   If height plots ,rd percentile or .th percentile IB can only be estimated   to  IB indicates normal nutrition status  to  IB mild risk of malnutrition  to  IB moderate risk of malnutrition , IB severe risk of malnutrition

462

H Cutff Points for asting vereigt an esity BIRTH25 YEARS

5219 YEARS

rot Status

nicator

Percentile

S

Percentile

S

asting

eigtorlengtaBM

r

,22

r

,22

Severe asting

eigtorlengtaBM

0.1st

,2

0.1st

,2

is o overeigt

eigtorlengtaBM

85t

.1





vereigt

eigtorlengtaBM

.97t

.12

.85t

.11

esity

eigtorlengtaBM

.99.9t

.1

.97t

.12

Severe oesity

BM





.99.9t

.1

a

Weightorlength in children , years.  in children $ years.



BMI, ody ass inde; SD, standard deviation; N/A, not applicale; WHO, World Health Organization. ata ro rooting Optial onitoring o child growth in anada. Using the new WHO growth charts. Paediatr Child Health. ;–.

MATRT CATRS  hen only a single data point is available use scores for weightfor heightlength body mass index for age or lengthheight for age or midupper arm circumference able   hen two or more data points are available include weight gain velocity , years of age weight loss – years of age decelerations in weight for lengthheight score and inadeuate nutrient intake able 

ale 19.7

Growth and Nutrition

ale 19.

19

nterreting Malnutrition Ris it a Single ata Point

nicator

Mil Malnutrition

Moerate Malnutrition

Severe Malnutrition

eigt or eigt score

–1 to –1.9

–2 to –2.9

– or less

Boy ass ine or age score

–1 to –1.9

–2 to –2.9

– or less

engteigt or age score

o ata

o ata

– or less

Miupper ar circuerence score

–1 to –1.9

–2 to –2.9

– or less

odied ro onsensus tateent o the Acadey o Nutrition and ieteticsAerican ociety or arenteral and nteral Nutrition ndicators ecoended or the dentication and ocuentation o ediatric alnutrition Undernutrition. Nutrition in Clinical Practice. ;–.

4

ale 19.8

Growth and Nutrition 19

464

nterreting Malnutrition Ris it To or More ata Points

nicator

Mil Malnutrition

Moerate Malnutrition

Severe Malnutrition

eigt gain velocity ,2 years

,75 o nor

,50 o nor

,25 o nor

eigt loss 2–20 years

5 B

7.5 B

10 B

ecline in eigt or lengt eigt score

g o 1 score

g o 2 scores

g o  scores

naeuate nutrient intae

51–75 estiate energyprotein

2–50 estiate energyprotein

#25 estiate energyprotein

UBW, Usual ody weight. odied ro onsensus tateent o the Acadey o Nutrition and ieteticsAerican ociety or arenteral and nteral Nutrition. ndicators recoended or the identication and docuenta tion o pediatric alnutrition undernutrition. Nutr Clin Pract. ;–.

ENTERAL FEEDING REASTFEE  Counselling—the importance of breastfeeding a Breast milk is optimal for infants xclusive breastfeeding is recom mended for the rst  months of life Continued breastfeeding with complementary foods is recommended for up to  years and beyond b nfant benets: transfer of antibodies and immune factors protec tion against respiratory and gastrointestinal I infections aids mat uration of the I tract low allergenicity supports neurodevelopment decreases risk of obesity infant intake is selfregulated at the breast decreases risk of type  and  diabetes and is protective against sudden infant death syndrome c aternal benets: decreases risk of type  diabetes heart disease osteoporosis and cancers convenient economical and environmen tally friendly  Counselling—practical support a In rst few days of life both breasts should be o¨ered at each feeding for a minimum of  minutes on each breast to establish a milk supply b Milk usually comes in between day  and  postpartum but can take up to  days especially if the baby was born by cesarean section c In the newborn period average time at breast is  to  minutes per feed arly freuent unrestricted feeding establishes the milk supply d nce milk comes in the rst breast should be emptied before o¨ering the second tart each feed on opposite breast







Growth and Nutrition



e If there is interruption of breastfeeding or parentinfant separation for any length of time parents need to be counselled on how to maintain milk supply with e¨ective milk expression as many times a day as the infant would be feeding arers of successful breastfeeding a ight breastfeeding events in  hours b wallows evident during feeds c umber of wet diapers matches day of age until day  a©er day  can expect six or more heavy wet diapersday d Return to birth weight by  weeks  to  gday weight gain during rst  months of life Composition of breast mil a Colostrum for rst  to  hours yellow small volume with increased electrolyte high protein immunoglobulin and lowfat content facilitates passage of meconium b Breast milk energy  kcalm composed of carbohydrate  calories fat  protein  Contraindications to breastfeeding a nfant: inborn errors of metabolism—in most cases of metabolic disorders except galactosemia infants can still breastfeed with close monitoring and a prescribed dose of specialied formula iet to be considered on a casespecic basis b other i Infections human immunodeciency virus I human cell lymphotropic viruses  herpes in breast region or active untreated tuberculosis these may not be absolute contraindications in some developing nations ii Most medications are safe to take while breastfeeding Caution regarding certain medications including chemotherapy immuno suppressants lithium ergot alkaloids radiopharmaceuticals bromocriptine and iodides peak with a pharmacist iii Current maternal alcoholdrug abuse roblems associated with breastfeeding: see able 

19

PEAR Maternal cytoegalovirus epatitis an antiiotictreate astitis are not contrainications to reasteeing.

45

ale 19.9

nset Caracteristics an Manageent of Coon Proles Associate it reastfeeing nset

Caracteristics

Manageent

ay

Growth and Nutrition 19

Breasteeing aunice

sually in te rst ee o lie

Breast il aunice

ter te rst ee o lie

ral caniiasis

See aunice in Capter 27 eonatology

ite plaues on oral suraces

reat ay it antiungal reat oter’s nipples topically

Moter Sorecrace nipples

nytie

oral nipple sensitiv ity typically susies itin 1 inute o sucling

Ensure proper positioning an goo latc. pply reast il to nipples ater eac ee an allo to air ry. voi soap. May apply creas e.g. lanolin.

Breast engorgeent

ccurs ost oten in te rst ee

Cause y accuu late il an eea in reast tissue ay e eaggerate y poor eeing

Encourage ore reuent ee ing col copresses eteen ees ay reuire puping

Mastitis

nytie

nection o te reast usually ecause o Staphylococcus aureus. ypically presents as a ar re tener sol len area o one reast associate it ever.

Continue reasteeing ensure aeuate eptying o aecte sie. reat oter it antiiotics copresses. Monitor or ascess ic ay reuire rainage.

ATERATE CHCE F FEE SEECT  If breast milk is unavailable contraindicated or mother chooses not to breastfeed commercially available infant formula is to be used as an alternative igure   nsure parent’s feeding choice is an informed one—refer to lifelong benets of breast milk and breastfeeding

466

Figure 199 Algorit for Clinically Aroriate Forula Selection Preterm infants

Normal GI function

Abnormal GI function

2000 g

Standard Preterm (hospital use only) Enfamil Premature A+ Enfamil  (to be mie with E) imilac pecial are

Discharge Preterm Enfamil Enfacare A+ imilac Neosure

Soy Enfamil oy A+ Goo tart Alsoy imilac Isomil

Milk protein allergy

Extensively hydrolyzed Nutramigen A+ imilac Alimentum

Milk & soy protein allergy

Amino acid–based Puramino A+ Neocate Infant

Severe intact protein allergy

Primary/ seconay lactase eficiency

Lactose-free nfamil  actose ree Similac Sensitive

Normal GI function

Normal GI function

Standard cow’s milk-ased

Intact protein allergy

Children >10 years

Children 1–10 years

Term infants

General malasorption it/itout intact protein allergy

Extensively hydrolyzed with >30 of fat CT Pregestimil 

Abnormal GI function

Standard casein-ased Nutren unior esource is essentials 

100 free amino acid Neocate unior Splas ivone Peiatric Puramino  unior

Intact protein allergy

il protein allergy eere intact protein allergy

General malabsorption (without intact protein allergy) /Dysmotility/ Intolerance

se adult ormulas Standard caseinbased eity (//) smolite  Ensure Ensure Plus Semi-elemental protein Peptamen (/) lended ood product ompleat Peiatric ompleat

emi-elemental rotein hydrolysate with CT Peptamen unior 0 Peptamen unior  Peiasure Peptie lended food rodct ompleat Peiatric

dditinal ormula selection considerations enal isease or electrolyte abnormalities

ow electrolytelow-renal solute load uplena Noasource enal

ymphatic abnormalities or chylous lea

at modiied with  o at  Portagen onogen

EBM xpressed breast milk GI gastrointestinal HMF human milk fortier MCT mediumchain triglycerides Provided with permission from epartment of ietetics ospital for ick Children

19

47

Growth and Nutrition

 If breast milk is unavailable because of a low milk supply ensure parent is connected to appropriate lactation support wwwontariobreastfeedsca  ee able  for feeding freuency and volume  or children . year of age igure  helps determine appropriate formula selection for children reuiring nutritional supplementation ale 19.10 Age

nfant Feeing Freuency an olue

Growth and Nutrition

uer of Feesay

Aroiate uantityFee

Birt–1 ee

–10

ay 1 5  ay 2 5–15  ay  15–0  ay 4–7 0–0  1–2 o

1 ee–1 ont

–8

90–120  –4 o

1–2 onts

–8

120–150  4–5 o

2– onts

5–

150–210  5–7 o

–9 onts

4–5

180–210  –7 o

7–12 onts

–4

210–240  –8 o

ata ro alnins , aa . Better Ba Food our ential Guide to Nutrition, Feedin and Cooin or All Baie and oddler. oronto, ON oert ose nc.; .

19

C’S M PRTE AERY (CMPA)  Denition: immune reaction to cow’s milk protein immunoglobin Ig type  hypersensitivity more immediate or nonIg mediated type III hypersensitivity or mixed  Clinical presentation a Immediate urticaria angioedema vomiting acute atopic dermatitis are b elayed  hours to  week—dysphagia vomiting regurgitation dyspepsia early satiety diarrhea rectal bleeding failure to thrive  abdominal pain severe colic persistent constipation c Chronic irondeciency anemia  reatment diagnostic approach and management: see igure   Diagnostic elimination diet a Breastfed infants continue breastfeeding mothers should avoid all milk and milk products from their own diet b onbreastfed infants cow’s milkbased formula should be avoided rst choice extensively hydrolysed infant formula e or infants with extreme or lifethreatening symptoms an amino acid–based formula AA may be considered as a rst choice c oddlers and older children foods and liuids containing cow’s milk protein or other unmodied animal milk proteins eg goat’s milk 468 sheep’s milk should be strictly avoided

d pen or blind oral food challenge under medical supervision e If conrmed elimination diet with reevaluation for cow’s milk pro tein every  to  months or – months for severe reactions for tolerance

History, physical examination +/– laboratory tests

naphylaxis or clear immediate type reaction

Diagnostic elimination diets Early and late reactions (e, omitin, atopic ecema – ees astrointestinal symptoms (e, diarrhea, constipation – ees

CMP elimination and test or speciic E

No improement in clinical symptoms

mproement o the clinical symptoms

pec E neatie

Growth and Nutrition

Figure 191 Treatent iagnostic Aroac an Manageent of Co’s Mil Protein Allergy

pec E positie

tandardied oral challene ith CMP (open, sinle and/or doubleblind

19 Negative

No CMP elimination diet

Positive

Therapeutic elimination diet

CMP cow’s milk protein I immunoglobulin  Modied from oletko  iggemann B Arato A et al iagnostic approach and management of cow’s milk protein allergy in infants and children PA I Committee Practical uidelines J Pediatr Gastroenterol Nutr –

EERYTRET ESTY F FEES A THCE  nergnutrient densit of feeds a nergy density of breast milk and standard fullstrength infant formulas 5  kcalm 5  kcalo 5  k b Most standard pediatric enteral formulas for children . year of age are ready to feed 5  kcalm 5  kcalo 5  k c Indications for increasing caloric content of feeds uid restriction weight losspoor weight gain increased metabolic demands im paired swallowingoral aversion impaired absorptiondigestion d Concentrating feeds increases energy density and provides additional nutrients however it also increases osmolality and renal solute load 49

e teps in fortication for expressed breast milk or infant formula i Advance volume to full uid allowance ii Increase concentration in a stepwise fashion  kcalm n  kcalm n  kcalm n  kcalm able  or breast milk add infant formula and for infant formula mix with less water to obtain desired concentration f Precaution in infants , year feeds should only be concentrated to a maximum of  g proteinkg unless warranted by medical condi tion because of risk of renal impairment from high solute load Growth and Nutrition 19

ale 19.11

Steise Progression for Energy ensity of Enteral Feeings



cal

calo

2800

0.8

20

00

0.8

24

800

0.9

27

4200

1.0

0

400

1.1



5000

1.2



Adapted ro he Hospital or ick hildren uidelines or the Adinistration o nteral and arenteral Nutrition in aediatrics, .

 icening feeds a Indications to thicken feeds known or suspected aspiration risk as indicated by clinical assessment or video uoroscopic feeding study history of aspirationsuspected aspiration pneumonia coughing chokinggagging with feeds severe neurological impairment b If aspiration suspected feeding assessment by occupational therapist or speech language pathologist is recommended SS  ransition to solids a At around  months of age solids can be introduced in the baby’s diet ollow the baby’s cues on how much to feed ry new foods many times Infants may not always accept at initial o¨ering b ¨er ironrich foods rst meat poultry sh wellcooked egg legumes and ironfortied infant cereals c ¨er a variety of textures igure  d omogenied cow’s milk may be introduced between  and  months e An infant should be eating a wide variety of foods from all the food 470 groups by  months of age

Figure 1911 ntroucing e Foo Tetures to nfants

Thin purée

Thicker purée

Minced • umpy, fine chopped foods • elps teach baby about chewing and coordinating tongue moement • Examples cottage cheese, soft moist ground meats, small well-cooked pasta pastina, stars Chopped • hicker, coarser texture of food • deal when teeth start coming, but many babies can manage with their gums • aby can use pincer grasp thumb and forefinger to pick up food • Examples pieces of toast, elbow macaroni, chopped cubes of meat or cheese

Growth and Nutrition

Puréed • Smooth, lump-free texture for baby’s first introduction to solids • Start with thin purée, gradually thicken when baby is ready • Use infant cereal to thicken, breast milk or formula to thin • Examples smooth applesauce, rice cereal, sweet potato mash

Courtesy of Aboutidsealth at e ospital for ick Children

19

 llergenic foods a Most common food allergens in children are cow’s milk egg soy wheat peanut tree nuts and seafood see ood Allergic Reactions in Chapter  Allergy b o not restrict maternal diet during pregnancy or lactation PEAR elaying introuction o tese coon allergenic oos eyon  onts o age oes not prevent allergy evelopent.

 Other uids a ater that meets safety standards including tap commercially bottled water and well water is safe to reconstitute powdered formula with o not use mineral or carbonated water b Bring water for feeding infants , months to boil for at least  minutes and let cool c imit uice intake to a maximum of  oday from open cup  ood safet a Avoid hard small round smooth and sticky solid foods eg hot dogs grapes peanuts and raw vegetables and fruits not cut in small 471

Growth and Nutrition 19

472

pieces in children , to  years of age because of choking and aspiration risk b eed infants and children in an upright position with supervision c o not give honey to infants , year of age risk of botulism  egetarian diet a A wellplanned vegetarian diet with adeuate nutrients and energy can meet growth and nutrition needs at any age b Commercial soybased formula is recommended until  years of age for vegan infants who are not breastfed a©er  years of age fortied soybased milk should be used c Review intake of total calories protein iron vitamin B inc calcium and vitamin  to ensure child is growing well

VITAMIN AND MINERAL SUPPLEMENTATION ee ables   and  for daily reference intakes for macronutrients vitamins and minerals TAM   itamin  is found in a limited number of foods eg egg yolks fatty sh itamin  deciency is common in children in Canada  Cow’s milk infant formula and margarine have added vitamin   ere is a high level of vitamin  deciency found in irst ations and Inuit peoples special attention needs to be given to vitamin  supplementation in these populations  upplementation a Infants , year goal itamin  intake minimum  Iday b oddlers and children $ year routine vitamin  supplementation not recommended c upplementation with  Iday may be considered for children who do not regularly consume vitamin containing foods or have other risk factors for vitamin  deciency

ale 19.12

Age

Suary of ietary Reference ntaes (Rs) for nfants an Cilren Macronutrients

Se

Protein (gay)

Caroyrate (igestile) (gay)

Total Fat (gay)

inoleic Aci ega (gay)

Alainolenic Aci ega (gay)

Total Fier (gay)

Total ater (ay)

nfants 0– onts

Bot

9.1

60

31

4.4

0.5



0.7

Bot

11

90

30

4.6

0.5



0.8

1– years

Bot

1

1



7

0.7

19

1.3

4–8 years

Bot

19

1



10

0.9

25

1.7

9–1 years

M 

 

1 1

 

12 10

1.2 1

31 26

2.4 2.1

14–18 years

M 

 

1 1

 

16 11

1.6 1.1

38 26

3.3 2.3

7–12 onts Cilren

ecoended daily allowances As in bold and adeuate intakes As in italic. F, Feale; M, ale; ND, not deterinale. Adapted ro Food and Nutrition oard, nstitute o edicineNational Acadey o ciences. ietary eerence ntakes or nergy, arohydrate, Fier, Fats, Fatty Acids, holesterol, rotein, and Aino Acids acronutrients. . Availale at www.nap.edu.

19

47

Growth and Nutrition

19

Suary of ietary Reference ntaes (Rs) for nfants an Cilren itains

Se

itain A (cg ay)

itain C (g ay)

itain  ( ay)

itain E (g ay)

0– onts

Bot

400

40

400

4

7–12 onts

Bot

500

50

400

1– years

Bot



1

4–8 years

Bot



9–1 years

M 

14–18 years

M 

Age

Growth and Nutrition

474

ale 19.1

itain  (cg ay)

Tiain (1) (g ay)

Rio§avin () (gay)

iacin () (gay)

Pyrioine () (g ay)

2

0.2

0.3

2

0.1

5

2.5

0.3

0.4

4

600



30







600



55



 

 

600 600

11 11

60 60

9 

 

600 600

1 1

75 75

Folate (9) (cg ay)

it 1 (cg ay)

Pantotenic Aci () (gay)

iotin (cg ay)

Coline (g ay)

65

0.4

1.7

5

125

0.3

80

0.5

1.8

6

150





1

9

2

8

200









1

3

12

250

9 9

9 9

1 1

1 1

 

1 1

4 4

20 20

375 375

1 1

1 1

1 1

1 1

 

 

5 5

25 25

550 400

nfants

Cilren

ecoended daily allowances As in bold and adeuate intakes As in italic. F, Feale; M, ale. odied ro Food and Nutrition oard, nstitute o edicineNational Acadey o ciences. Availale at www.nap.edu.

ale 19.14

Suary of ietary Reference ntaes (Rs) for nfants an Cilren Minerals

Age

Se

Cal ciu (g ay)

0– onts

Bot

7–12 onts

Croiu (cgay)

Coer (cg ay)

Flou rie (g ay)

200

0.2

200

0.01

110

0.27

30

0.003

2

100

15

2

0.4

0.12

0.18

Bot

260

5.5

220

0.5

130

11



0.6

3

275

20



0.7

0.37

0.57

1– years

Bot

700

11



0.7

9





1.2

1







3

1

4–8 years

Bot

1000

15



1

9

1

1

1.5









3.8

1.2

1.9

9–1 years

M 

1300 1300

25 21

 

2 3

1 1

 

 

1.9 1.6

 

1 1

 

 

4.5 4.7

1.5 1.5

2.3 2.3

14–18 years

M 

1300 1300

35 24

9 9

2 3

1 1

11 1

1 

2.2 1.6

 

1 1

 

11 9

4.5 4.7

1.5 1.5

2.3 2.3

oine ron (cg (g ay) ay)

Magne Pos siu orus Seleniu (g Manganese Molyenu (g (cg ay) (gay) (cgay) ay) ay)

inc (g ay)

Potas siu (g Soiu Clorie ay) (gay) (gay)

nfants

Cilren

ecoended daily allowances As in bold and adeuate intakes As in italic. F, Feale; M, ale. odied ro Food and Nutrition oard, nstitute o edicineNational Acadey o ciences. Availale at www.nap.edu.

19

475

Growth and Nutrition

1.5

R  ealthy terms infants have adeuate iron stores for the rst  months  At  months old complementary ironrich foods should be introduced  Preterm infants and small for gestational age infants , kg have a decit of total body iron preterm infants receiving breast milk should be started on an iron supplement by  month of age and small for gesta tional age infants by  weeks of age continued until dietary sources are adeuate Additional information in Chapter  eonatology Growth and Nutrition 19

PEAR n iportant ris actor or iron eciency is te consuption o ore tan 500 1 o o il per ay in cilren .1 year o age.

TAM 1  itamin B is only found naturally in animal products meat sh dairy products and eggs  It is added to soy infant formula cereals yeasts and fortied soy and nut beverages  Breastfed infants of vegan mothers and children who follow a vegan diet are at highrisk for vitamin B deciency  It is recommended that at least three servings of food rich in vitamin B be included in the daily diet or supplementation be provided at  to  mcgday FRE SPPEMETAT  luoride has been shown to decrease dental caries but excess uoride can result in uorosis  luoride supplementation recommended only for infants $ months if water uoride concentration , ppm teeth not brushed at least twice a day susceptibility to high caries activity eg family history geographic trends

PARENTERAL NUTRITION (PN)  ese guidelines are intended for the general inpatient pediatric population Prescribers are encouraged to use clinical assessment and udgement of their patients’ reuirements when prescribing P  Parenteral nutrition in the neonatal period is covered in Chapter  eonatology  ndications for N: P is nutrition delivered intravenously to patients who are unable to ingest or absorb nutrients via the enteral tract for a signicant period of time Patients should not be without adeuate nutrition for longer than a Premature infants  day b erm infants and beyond  to  days consider withholding P for 476  week in critically ill children

ale 19.15

Growth and Nutrition

 Although the I tract should be used whenever possible P may be used as a primary source of nutrition providing full nutrition support or as a partial source providing nutrition repletion or augmentation in patients unable to tolerate full enteral nutrition  arenteral nutrition reuirements a e energy cost of digestion of P is minimal therefore P fed patients reuire approximately  to  infantschildren to , nenates eer alries mared ith hen enterally ed b o account for the decreased energy reuirement when using P resting energy expenditure R plus activity andor stress factors are applied to estimate total caloric reuirements instead of the RI see able  c ee able  for amino acid protein rates able  for lipid fat rates able  for glucose rates able  for uid rates and ables  and  for electrolyte recommendations

Energy Reuireents (calgay) for Parenteral utrition in ifferent Pases of isease Acute Pase

Stale Pase

Recovery Pasea

0–1 years

45–50

0–5

75–85

1–7 years

40–45

55–0

5–75

7–12 years

0–40

40–55

55–5

12–18 years

20–0

25–40

0–55

19

a

eneral adeuate aounts to support growth. Higher or lower aounts ay e reuired in specic patient populations. Adapted ro he Hospital or ick hildren uidelines or the Adinistration o nteral and arenteral Nutrition in aediatrics, .

ale 19.1

Parenteral Aino Aci Recoenations for Stale Patients (ggay) nitiate

Aeuatea

Maa

er inants

1.5–2

1.5–

.5

2 onts to  years

1.5–2

1.5–2.5

.5

–18 years

1.5–2

1.5–2

2

a

atients ay reuire ore protein to support growth andor accoodate losses.

Adapted ro he Hospital or ick hildren uidelines or the Adinistration o nteral and arenteral Nutrition in aediatrics, .

477

ale 19.17

Parenteral ii Recoenations for Stale Patients (ggay) nitiatea

Min

Ma

0.25

4

0.1



er inant 0.5–1.5

2 onts–18 years

Aeuate Aount ntae o 25–50 o nonprotein calories is recoene in ully parenterally e patients

a

ipid delivery range or ages etween adolescents to neonates, respectively. iniu lipid delivery reuired to prevent atty acid deciency.



Growth and Nutrition

Adapted ro he Hospital or ick hildren uidelines or the Adinistration o nteral and arenteral Nutrition in aediatrics, .

ale 19.18

Parenteral lucose Recoenations (ggin)

Patient

Acute Pase

,10 g .1 ont o age

Recovery Pasea

2–4

4–

–10

11–0 g

1.5–2.5

2–4

–

1–45 g

1–1.5

1.5–

–4

1–2

2–

.45 g

19

Stale Pase

0.5–1

a

Adeuate aounts to support growth in the average pediatric patient. Higher glucose aounts ay e reuired to support edical needs. t is advisale to consider total acronutrient distriution within the total parenteral nutrition order. Adapted ro he Hospital or ick hildren uidelines or the Adinistration o nteral and arenteral Nutrition in aediatrics, .

ale 19.19

Maintenance Flui Reuireents (eyon te eonatal Perio)

eigt

gay

g

 te rst 10 g

100

4

B eigt eteen 10 an 20 g

150 etra gay

12 etra gour

C eigt aove 20 g

125 etra gay

11 etra gour

Su total reuireents

1B1C

1B1C

Adapted ro he Hospital or ick hildren uidelines or the Adinistration o nteral and arenteral Nutrition in aediatrics, .

478

ale 19.20

Parenteral Flui an Electrolyte Recoenations a  ays– 1 Year

1– Years

– Years

–1 Years

1–1 Years

lui gay

120–150

80–120

80–100

0–80

50–70

a olgay

2–

 olgay

1–

1–

Cl olgay

2–4

Adeuate aounts to support the average pediatric patient. Higher or lower aounts ay e reuired to support edical needs. Adapted ro he Hospital or ick hildren uidelines or the Adinistration o nteral and arenteral Nutrition in aediatrics, .

ale 19.21 Age

Recoene Parenteral ntae for Calciu Posorus an Magnesiu (olgay) Calciu

Posorus

Magnesiu

0– onts

0.8–1.5

0.7–1.

0.1–0.2

7–12 onts

0.–0.8

0.–0.8

0.1–0.2

0.25–0.4

0.2–0.7

0.05–0.15

1–18 years

Adapted ro he Hospital or ick hildren uidelines or the Adinistration o nteral and arenteral Nutrition in aediatrics, .

Growth and Nutrition

a

19

 Considerations in management a rdering P i Amino acids protein standard solutions contain  g amino acids per  m  for preterm or  g amino acids per  m  for infants and children ample calculation for a kg infant if starting at  gkgday of amino acids using  amino acid solution  gkg 3  kg 3  m g   h day 5 desired rate mh ii ipids standard solutions are fat emulsion   g lipids per  m or fat emulsion   g lipids per  m if uid restricted ample calculation for a kg infant and if ordering sepa rate lipid solution starting at  gkgday lipids using  lipid solution  gkg 3  kg 3  m g   hday 5 desired rate mh b eaning from P i onuid restricted patients rate of amino acid and dextrose solution should be tapered at a  ratio as enteral intake increases ii luid restricted patients incremental decreases in rate of amino acid and dextrose solution ie   etc based on enteral delivery or example enteral volume is  of goal decrease P solution by  479 iii ipids may be stopped completely without tapering the rate

Growth and Nutrition 19

c Cycling P i Cycling allows administration of incompatible medications increases patient freedom for ambulation and helps in weaning o¨ P ii udden cessation of P solution with high glucose concentration . or IR . mgkgmin may result in rebound hypoglyce mia apering P should take place over  hour with half the full P rate given over the last  hour Consider blood glucose check within  hour of abrupt decrease in P rate  See ale  r  mnitring

ale 19.22

Sale Monitoring Sceule for Stale Patients on Parenteral utrition

Paraeter

Monay

Tursay (3 ees nly)

Soiu potassiu clorie

es

es

lucose

es

es

Creatine urea

es

esno

ipi level

es

es

   conugate iliruina

es

o

onie calciu pospate agnesiu

es

o

a

arkers o hepatic inury A, A, conugated iliruin, A can e ollowed periodically once a week to twice a onth i known liver inury or other specic concerns. AP, Alkaline phosphatase; A, alanine ainotranserase; AS, aspartate ainotranserase; GG, gaaglutayl transerase; ICU, intensive care unit. Adapted ro he Hospital or ick hildren uidelines or the Adinistration o nteral and arenteral Nutrition in aediatrics, .

FAILURE TO THRIVE (FTT)  Denition: weight for age less than the third percentile decrease in growth velocity resulting in weight andor height crossing $ percentiles or weight , of ideal body weight  is the result of interaction between the child’s health development behavior and the environment  Dierential diagnosis: see able  PEAR ailure to trive is a not a iagnosis ut a sign o inaeuate grot.

480

ale 19.2

Causes of Failure to Trive

naeuate Caloric ntae Meical  astroesopageal reªu  naeuate appetite e.g. genetic synroes anoreia o cronic isease  naeuate aility to eat large aounts e.g. cronic constipation intestinal tract ostruction  nsucient lactation  nappropriate nutrition intae e.g. ecess il ilution o orula ecess ruit uice intae restricte iet Feeing Sills    

Mecanical proles e.g. clet palate nasal ostruction Sucing or salloing ysunction e.g. central nervous syste isorers neurouscular ral aversionypersensitivity euroevelopental elay e.g. gloal evelopent elay autis spectru isorer

Psycosocial        

oo insecurity isturance o caregivercil relation orceeeing naeuate nolege aout nutrition nees ailure to avance to ageappropriate eeing Caregiver ental ealt proles e.g. aniety epression syciatric isorers o cil e.g. oo isorers eating isorers eglectinaeuate provisions o calories

Growth and Nutrition

utritional

19

ncrease Eeniture of Calories  Enocrine isorers ypertyroiis  Cronic inections e.g. congenital inections urinary tract inections tuerculosis uan iunoeciency virus  Cronic inªaation e.g. inªaatory oel isease uvenile iiopatic artritis  Metaolic iseases e.g. galactoseia inorn errors o etaolis  Cronic respiratory insuciency e.g. cronic lung isease cystic rosis  Congenital or acuire eart isease  eatological iseases e.g. severe ironeciency aneia or alignancy naeuate utrient Asortion or ncrease osses  Malasorption e.g. cystic rosis co’s il protein allergy celiac isease  Biliary atresia  oiting e.g. intestinal tract ostructionatresia inectious gastroenteritis increase intracranial pressure  iarrea e.g. inectious inªaatory oel isease  enal iseases e.g. renal tuular aciosis  norn errors o etaolis

481

Growth and Nutrition 19

482

 istor a etailed diet history durationuantity of feeds sleep feeding forcefeeding feeding regardless of hunger cues distracting to feed dilution of formula other uid intake uice water etc dietary restrictionsbeliefs b Assessment of textures—feeding ability and appropriateness for age c Past medical history allergies developmental milestones detailed social history ie caregivers psychosocial stressors parental mental illnesssubstance abuse social services involvement food insecurity d Pregnancybirth history small for gestational age intrauterine growth restriction prematurity intrauterine infections maternal substance abuse  hsical eamination a Plot all anthropometrics length weight head circumference and compare with prior measurements b ydration status dysmorphic features wasting signs of chronic disease development signs of abusepoor hygiene c bserve parent–child interaction and child’s temperament  nestigations a reeday food record b Assessment by dietitian occupational therapist lactation consultant social worker as indicated c Consider clinical observation to assess weight gainobserve feeding d Basic laboratory assessment complete blood count CBC electro lytes blood gas urinalysis creatinine urea liver enymes albu min ferritin Creactive protein CRPerythrocyte sedimentation rate R e ther tests as indicated by history and physical examination f Consider insulinlike growth factor  I and bone age xray of le© hand and wrist if short stature length less than the third percentile  reatment a nergyboosting strategies use high fat foods such as butter oils margarine creams – high fat yogurt and milk $ milk fat nut butters sour cream or mashed avocado added to foods to increase energy density b Mealtime modications solids before liuids no meals on the go or chasing eliminate graing between meals encourage regular feeding

schedule eg feed every – hours encourage family mealtimes avoid force feeding eliminate distractions c Involvement of psychologistsocial worker to support parent–child interactions d Assess the need for tube feeding if attempts at increasing energy intake orally are unsuccessful

 Denition: see able  for cuto¨s  Dierential diagnosis a Idiopathicfamilial b ormonal hypothyroidism growth hormone deciency Cushing syndrome polycystic ovary syndrome hypogonadism c ypothalamic obesity craniopharyngioma d yndromic risomy  Praderilli BardetBiedl retinal dystrophy renal abnormalities mitral regurgitation ragile  macroorchidism large ears Albright hereditary osteodystrophy short stature skeletal defects e enetic melanocortin  receptor deciency congenital leptin deciency leptin receptor defect  istor: diet physical activity sedentary time sleep psychosocial factors mental health body image depression anxiety binge eating disorder  hsical eamination a ital signs heart rate R increases with higher BMI blood pressure BP by auscultation with cu¨ . of midarm circumference b ead and neck papilledema dental caries wide neck adenotonsillar hypertrophy c Chest gynecomastia . cm breast tissue cervicodorsal hump Cushing syndrome d I hepatomegaly nonalcoholic fatty liver disease A e enitourinary inconspicuous penis from enlarged suprapubic fat pad f Musculoskeletal gait scoliosis lordosis slipped capital femoral epiphysis genu valgumvarus pes planus g kin acanthosis nigricans hirsutism acne striae intertrigo pannus  nestigations: directed by history and physical examination igure   Complications: see able 

Growth and Nutrition

OBESITY

19

48

Figure 191 Evaluation of Cil or Aolescent it esity Onset of diabetes in early infancy

Yes

Possible genetic defect of leptin signalling pathway

Yes

Consider change of medication

No Drug associated weight gain No Yes

Growth and Nutrition

Poor linear growth despite weight gain

ypothyroidism rowth hormone deficiency Cushing syndrome Pseudohypoparathyroidism a

Consider endocrine disorders

No Congenital midline defect or history of intracranial radiation or surgery

Yes

Imaging and neuroendocrine screening for structural or functional hypothalamic dysfunction

No Developmental delay or dysmorphic features

Yes

Consider genetic syndromes

Yes

ssess modifiable lifestyle factors diet and physical activity

Praderilli syndrome ardetiedl syndrome lstrom syndrome Cohen syndrome Smithagenis syndrome ragile  syndrome SI mutation  syndrome

No Normal examination, normal or increased stature

19

Screen for comorbidities of obesity

AR ilms tumour anirida genitourinary anomalies intellectual disability rom an C awlor A imm  Childhood obesity ancet –

ale 19.24

Colications of Ciloo esity

CS  iopatic intracranial ypertension Psycosocial  epression  niety Cariovascular  Elevate loo pressure  yslipieia  terosclerosis Pulonary  structive sleep apnea  sta  Eercise intolerance

Enocrine  nsulin resistance2M  CS  recocious puerty astrointestinalnutrition  atty liver isease  astroesopageal reªu  Colelitiasis  ron eciency  itain  eciency rtoeic  Slippe capital eoral epipysis  steoartritis

Renal  loerulopaty CNS, entral nervous syste; PCOS, polycystic ovary syndroe; DM, type  diaetes ellitus.

484

ale 19.25

Growth and Nutrition

 Comorbidit screening a Children . years of age with BMI $th percentile lipid prole total cholesterol triglycerides high density lipoprotein  cholesterol non cholesterol every  years b Children with BMI $ th percentile with at least one risk factor fasting plasma glucose hemoglobin bAC and oral glucose tolerance test every  years i Risk factors family history of type  diabetes high risk ethnicity signs of insulin resistance or exposed to gestational diabetes mellitus in utero c Children . years with BMI th to th percentile and metabolic risk factors or BMI $th percentile alanine aminotransferase Aaspartate aminotransferase A every  years  anagement a Primary goal of pediatric obesity treatment is to improve health outcomes by increasing healthy behaviors able  b mphasie that weight loss in small increments no more than  kgweek can lead to large improved health outcomes c rastic weight loss with restrictive diets or extreme physical exercise should not be encouraged because it is not sustainable d Dietar: establish normalied eating—eat as a family decrease sugarsweetened beverages follow healthy portion sies increase consumption of vegetables and fruit limit fastfoodrestaurant meals e hsical actiit: integrate enoyable activities to complete as fam ily andor with teamsprograms encourage active living eg taking stairs vs elevator limit screen time appropriately promote sleep hygiene f harmacotherap: minimal evidence for e°cacy in weight loss g urger: bariatric surgery rare in pediatric population

19

Aroac to esity Counselling

Ste

Actions

s

s or perission to iscuss eigt Eplore reainess or cange

ssess

ssess oesity relate riss an potential root causes o eigt gain

vise

vise on oesity riss iscuss enets an options

gree

gree on a realistic plan ase on SMa goals

ssist

ssist in aressing rivers an arriers oer eucation an resources reer to provier an arrange olloup

a

A A goal is specic, easurale, achievale, realistic and tiely.

Adapted ro the anadian Oesity Network. Accessile at www.oesitynetwork.caAspediatrics

485

 Counselling a se phrases like “weight” “BMI” “weight issues” “excess weight” b ocus on entire family rather than the child alone c Avoid conversations which are forced or shaming create unrealistic expectations or practitioner driven goals

MICRONUTRIENT DEFICIENCIES Growth and Nutrition 19

 Micronutrients are vitamins and minerals that are obtained from the diet and are reuired in trace amounts to maintain physiological processes in the body and ensure normal growth and development  Micronutrient deciencies other than iron deciency are relatively rare in developed nations and o©en only seen in specic nutritional medical or developmental contexts typically as a result of inadeuate oral intake or malabsorption  Children with autism spectrum disorder are at risk of “rare” micronutri ent deciencies if restricted and repetitive behaviors manifest as restricted diet and limited food repertoire ERPHTHAMA  pectrum of eye disease caused by vitamin A deciency  Characteried by pathological dryness of the cornea and conunctiva Bitot spots abnormal keratiniation of the conunctiva xerosis dryness and keratiniation of cornea keratomalacia so©ening and liuefaction of cornea also manifests as night blindness and retinopa thy because of role of vitamin A in photoreception at the reception  iagnosis usually made clinically but may be supported by low serum retinol vitamin A level  May lead to permanent vision loss if not identied and treated promptly  reat as per  guidelines with agespecic doses of vitamin A ral route of administration is strongly preferred SCRY  isease resulting from severe vitamin C deciency  Clinical manifestations are dermatological petechiae ecchymosis follicular hyperkeratosis corkscrew hairs gingival bleeding swelling gingivitis hematological anemia and musculoskeletal arthralgias subperiosteal hemorrhages

486

RCETS  ecient mineraliation of bone resulting in abnormalities at growth plate usually classied based on underlying mineral deciency calcipenic versus phosphopenic  Calcipenic form caused by insu°cient intake of vitamin  andor calcium rare genetic defects leading to vitamin  resistance do exist  Phosphopenic form almost exclusively caused by renal phosphate wasting isolated or related to a tubular disorder eg anconi syndrome  Clinical ndings similar in calcipenic and phosphopenic rickets eg frontal bossing delayed fontanelle closure craniotabes prominence of anterior ribs at costochondral unctions wrist widening and bowing of legs pattern of deformity depends on age and weightbearing status of child  Radiological ndings include widening cupping and fraying of the metaphysis and an increase in thickness of the growth plate  Alkaline phosphatase is elevated in both forms of rickets serum para thyroid hormone calcium and phosphorus levels help to distinguish between the two able   In calcipenic rickets serum hydroxyvitamin  should be ordered to di¨erentiate between vitamin  deciency most common and other causes  reatment involves vitamin and mineral supplementation dependent on the underlying type of rickets

ale 19.2

Growth and Nutrition

 iagnosis is primarily clinical and is conrmed when symptoms resolve with vitamin C supplementation plasma ascorbic acid level may be supportive can be inuenced by recent vitamin C intake  reatment vitamin C replacement

19

istinguising Calcienic ersus Posoenic Ricets Calcienic Ricets

Posoenic Ricets

aratyroi orone

Elevate

sually noral ay e lo or illy elevate

Calciu

oral or lo

oral

osporus

oral or lo

o

487

R EFCECY ee Chapter  ematology

FURTHER RESOURCES  ietitians of Canada wwwdietitiansca  ntario ieticians in Public ealth wwwodphca  orth American ociety for Pediatric astroenterology epatology and utrition wwwnaspghanorg Growth and Nutrition 19

488

CHAPTER

Gynecology

20

Lauren Friedman • Heather Millar • Anjali Aggarwal

Common abbreviations Anatomy of the external genitalia Genital examination of pediatric and adolescent females Prepubertal gynecological issues Genital infections Ovarian/adnexal pathology Dysmenorrhea and pelvic pain Abnormal uterine bleeding

489

COMMON ABBREVIATIONS Also see page xviii for a list of other abbreviations used throughout this book

Gynecology 20

AFP AUB b-hCG BV CHC DHA DPA DUB UA FH FA-AB GU UD DH H AA A CP PC P  P-PA VD

alpha fetoprotein abnoral uterine bleeding b-huan horioni gonadotropin baterial vaginosis obined horonal ontraeption dehdroepiandrosterone depoedroxprogesterone aetate dsfuntional uterine bleeding exaination under anesthesia follile-stiulating horone uoresent treponeal antibod absorption test genitourinar intrauterine devie latate dehdrogenase luteiniing horone nulei aid apliation testing nonaidental inur oral ontraeptive pill polsti ovar sndroe rapid plasa reagin sexuall transitted infetion Treponema pallidum partile agglutination venereal disease researh laborator

ANATOMY OF THE EXTERNAL GENITALIA ee Figure  Figure 20.1 Female External Genitalia Mons pubis Prepuce (clitoral hood) Clitoris Labia minora Labia majora Anus

Urethra Openings for paraurethral (Sene’s) glands men Vestibule Vaginal orifice Openings for greater estibular (artholin’s) glands

odied fro Applegate  e Sectional Anatomy Learning System rd ed Philadelphia

490 aunders 

A. Genital examination of the prepubertal child  Preparation a btain assent fro the patient and onsent fro the patient’s aregiver ith the hild present b xplain the reasoning for the exaination and deonstrate the aterials that ill be used  Put hild at ease ith aregiver visible and appropriate draping and lighting  Examination techniques a Frog-leg position hild las supine ith feet together and knees apart either on exaination table or aregiver’s lap First inspet the external genitalia and then appl gentle tration at  and o’lok on the labia aora in a lateral and posterior diretion is allos visualiation of the loer third of the vagina and the henal ring b Prone knee–hest position patient kneels and leans forard to rest on forears hih allos for inspetion of the upper vagina and possibl the ervix  Special considerations a Never fore an exaination b Beause of a lak of estrogen prepubertal vaginal uosa and henal tissue are thinner redder and ore sensitive opared ith postpubertal uosa  ere are an henal variations Certain ongenital anoalies a reuire surgial onsultation d peulu exainations are not appropriate in prepubertal hildren f better visibilit is reuired a stosope or vaginosope a be used under anaesthesia or onsious sedation

Gynecology

GENITAL EXAMINATION OF PEDIATRIC AND ADOLESCENT FEMALES

20

PEARL Gynecological examinations should be modied based on pubertal stage and history of sexual activity.

B. Genital examination of the adolescent  Preparation a evie ondentialit and obtain onsent b Disuss and deonstrate the exaination proedures and instruents  edial haperone is strongl reoended for ediolegal reasons and to assist phsiian  Examination techniques a Position the patient in lithoto position in stirrups or in frog-leg position ith appropriate draping b First inspet external genitalia noting anner stage see Chapter  491 ndorinolog

Gynecology 20

 e exaination should be odied based on sexual ativit n virginal patients vaginal sabs henal inspetion and abdoinal or retoabdoinal palpation a be appropriate exuall ative patients a reuire speulu and bianual vaginal–abdoinal exainations to palpate the adnexa and uterus  Special considerations a Adust the sie of the speulu based on age henal status and sexual ativit b peulu and bianual exainations are less o¡en indiated in adolesent patients ith the advent of the ne vaginal sab hih patients an self-adinister and urine nulei aid apliation testing AA  tests as ell as ne Canadian Pap sear guidelines not reoended before age  ears

PREPUBERTAL GYNECOLOGICAL ISSUES NEONATAL VAGINAL BLEEING  enition Vaginal bleeding in a neborn feale that usuall ours beteen da of life  and  hould not ontinue past  onth of age  Etiology aternal estrogens ross the plaenta in utero and an ause various seuelae in the bab inluding breast buds nipple disharge and vaginal disharge Postdeliver aternal estrogen ithdraal an lead to endoetrial shedding and vaginal bleeding  ierential ther rare auses of neonatal vaginal bleeding inlude gneologial disease infetion tuor bleeding diathesis urethral prolapse and sexual abuse Heaturia is o¡en istaken for vaginal bleeding  reatment bservation and reassurane Further orkup is indiated onl if persistent or if there are suspiions of another ause PREPBERTAL VAGINAL BLEEING enition Aute or hroni vaginal bleeding in a hild ho has not et passed through enarhe ee Box  for di¢erential diagnosis Also see Chapter  ndorinolog

A. ularaginal trauma  Etiology a Can be blunt straddle inur fore of bod falling on an obet eg bike frae or penetrating inur  istory a nuire about ethod of inur keep a high level of suspiion for nonaidental inur A see Chapter  Child altreatent and ensure that ehanis of inur athes exaination b nuire about seuelae of inur aount of bleeding degree of pain 492 dsuria di¤ult urinating heaturia

Bx 20.1

Differential Diagnosis of Prepubertal Vaginal Bleeding

temi Caue • ndocrine conditions e.g. precocious puberty isolated precocious menarche • xogenous estrogen exposure • ypothyroidism • lood dyscrasia • varian tumors benign or malignant

 Physical examination a a need analgesia before exaination Can appl ie pak for  to  inutes or use loal anaestheti ore severe inuries a reuire sedation or exaination under anesthesia UA b traddle inuries exaination a reveal ehoseslaerations of the ons litoral hood labia aora and inora or periurethral areas andor vulvar heatoas f there is evidene of traua to the hen or posterior fourhette onsider A  Penetrating inuries henal tear iplies penetrating inur aidental or abuse and arrants investigation for vaginal or retal tears Deep tears ie involving hen and above deep perineal retal reuire UA b a gneologist to rule out ore extensive inur  reatment a uperial abrasion treat ith ie and opression b all laeration o¡en an be treated ith topial anaestheti and hgiene easures arel reuires suturing for osesis or heostasis  Heatoas onservative anageent ith ie opression and sit baths Heodnai instabilit arrants surgial treatent d eferral to gneolog for deep or penetrating laerations signiant bleeding inabilit to visualie extent of laeration and unooperative hild e f there is a true inabilit to void a reuire Fole atheter or assessent b urolog for urethral inur

Gynecology

Lal Caue • rauma—straddle or penetrating inury • oreign body • exual abuse • onspecic vulvovaginitis • nfections group  strep shigella sexually transmitted infections gonorrhea chlamydia etc. • rethral mucosal prolapse • ichen sclerosis • emangioma • terinevaginal pathology benign or malignant

20

B. rethral prolapse  enition protrusion of the distal urethra through the urethral eatus  Epidemiology ore oon in hildren of Afrian ethniit 493 age  to  ears

 linical presentation vaginal bleeding vaginal ass dsuria urinar freuen a have histor of reurrent Valsalva onstipation aute hroni ough  Examination friable redpurple annular ass ith urethra at enter  reatment treat preipitating fators Conservative easures inlude stool so¡eners topial estrogen reas eg Prearin rea applied tie dail for  eeks and sit baths hih o¡en resolve the prolapse in a fe eeks urgial exision a be reuired if the prolapse is large or persistent a¡er edial treatent

Gynecology 20

. oreign body  linical manifestations vaginal disharge foul odor interittent bleeding  Etiology ost oon foreign bod is toilet paper  Examination attept irrigation or diret visualiation 6 retoabdoinal exaination to palpate  reatment an irrigate vagina ith ar ater using atheter or feeding tube or use a algi sab to reove toilet paper UA reuired if sptos ith no visualied foreign bod despite irrigation PREPBERTAL VLVOVAGINITI  enition naation or irritation of the vaginal and vulvar tissue  Epidemiology ost oon gneologi oplaint in the prepubertal age group Unestrogenied prepubertal vaginal tissue is thinner alkaline and ore sensitive to irritation  linical manifestations Pain pruritus vaginal disharge or bleeding urinar freuendsuria and vulvar erthea  ierential  to  of ases are nonspei ee Box  for an expanded di¢erential Bx 20.2

Differential Diagnosis of Prepubertal Vulvovaginitis

Nnei 2– • oor hygiene • hemical irritant • hysiologic differences in prepubertal vaginal mucosa • ight clothing Freign B TraumaAue Inetiu • espiratory e.g. Group  strep Haemophilus inuenza or enteric e.g. Shigella ora • inorms • exually transmitted infections should raise ags for sexual abuse • Candida rare in prepubertal girls ho are no longer in diapers ithout predisposing factors e.g. diabetes recent antibiotic use.

494

temi Cnitin • iruses e.g. aricella psteinarr virus • rug reactions e.g. tevensohnson syndrome • ermatological conditions e.g. atopic dermatitis psoriasis lichen sclerosis

Bx 20. • • • • • • • • • • • •

Maintaining Vulvar Hygiene

o soap to labia void scented products void bubble baths ear cotton underear o underear at night void tight tting clothing void spending time in a et bathing suit void hair removal ppropriate voiding posture ipe front to bac se gentle underear detergent ith no fabric softener o not use pantiliners unless menstruating

Gynecology

 iagnosis a Clinial diagnosis b A vaginal ulture see able  a be indiated in selet ases  n ases of suspeted sexual abuse involve loal hild altreatent experts see Chapter  Child altreatent  reatment a General easures vulvar hgiene eduation and irritant avoidane Box  it baths use of barrier reas petroleu ell in oxide b reat spei organiss if identied  For severe inaation of nonspei origin onsider steroid rea d eferral to gneolog if unlear diagnosis nonresponsive to therap or if vaginosop is reuired for diagnosis or foreign bod reoval

20

PEARL ulvar yeast infections are uncommon in the prepubertal age group ithout other ris factors antibiotics diabetes etc..

LABIAL AHEION  enition Coon auired disorder in prepubertal hildren tpiall  onths to  ears here the labia adhere to eah other in the idline  linical presentation Asptoati or vulvovaginitis urinar trat infetion U urinar dribbling  reatment a ill o¡en spontaneousl resolve ith endogenous estrogen at pubert therefore no treatent needed if asptoati b General easures vulvar hgiene see Box  reove irritants appl bland eollient rea petroleu ell or in oxide  f sptoati an appl estrogen rea to fused area for  to  eeks eg Prearin rea applied tie dail using a ngertip 49

d f nonresponsive or aute urinar retention experiened liniian an perfor anual separation under topial anaestheti or sedation e eurrene is oon until patient goes through pubert

GENITAL INFECTIONS

Gynecology 20

496

nfetions of the feale genitalia an result in various linial sndroes and are aused b a ide variet of sexuall transitted and nonsexuall transitted infetions Priar are providers should revie sexual histor risk fators and sptos and onsider testing for infetions as appropriate “eportable” s eg hladia gonorrhoea sphilis HV should be reported to the publi health departent for ontat traing and to ensure notiation and treatent of sexual partners e presene of one  should raise suspiion for others s in all prepubesent and nonsexuall ative hildren is a red ag for hild abuse see Chapter  Child altreatent POTPBERTAL VLVOVAGINITI  enition naation or infetion of the vagina or vulva external feale genitalia  linical manifestations Vaginal or vulvar pain ithing disharge erthea dspareunia dsuria and spotting  ierential a nfetious Candida albicans Trichomonas vaginalis and baterial vaginosis Also onsider gonorrhea hladia herpes siplex virus HV genital arts and parasites eg sabies b oninfetious ontat deratitis retained tapon poor hgiene and deratologi disorders eg psoriasis  Phsiologi leukorrhea noral vaginal disharge seen in higher estrogen states idle pregnan patient taking CP  iagnosis a Phsial exaination perineal and gneologi exainations adusted based on sexual ativit b et ount pH irosop potassiu hdroxide H hi¢ test able   ther investigations ervial or vaginal sabs for gonorrhea hladia trihoonas should be sent based on sexual ativit  reatment efer to able  nsure proper vulvar hgiene see Box 

able .1

Clinial an Laratr Feature  Cmmn Caue  Vulaginiti

ClinialLaratr Feature

Pilgi

Candida

Trichomonas

Baterial Vagini

ppearance of discharge

hite grey or clear

hite curdlie

Greengrey frothy

hin homogeneous grey

ulvarvaginal inammation

one

resent erythema edema

ften erythema straberry cervix

are

ther clinical signs and symptoms

one

tching dysuria dyspareunia

ulvar itching burning dysuria pelvic discomfort

ishy odor

is factors

ecretion of estrogen

ntibiotics diabetes heatmoisture s pregnancy obesity immunodeciency

ther s

exual activity douching previous 

p of discharge

#4.

#4.

.4.

.4.

icroscopy

pithelial cells many lactobacilli fe s

ncreased s pseudohyphae and buds

ncreased s motile trichomonads

ncreased s decreased lactobacilli “clue cells”

hiff test shy odor on addition of 1  to sample

egative

egative

ometimes positive

ositive

anagement

eassurance

 ntravaginal iconaole lotrimaole or erconaole 3 3– nights OR   luconaole 1 mg 3 1 dose

 etronidaole  mg  bid 3  days OR  g  3 1 dose  reat sexual partners

 ral etronidaole  mg bid 3  days OR  ntravaginal etronidaole gel .  g gel daily 3  days OR  lindamycin cream   g gel daily 3  days

KOH, Potassium hydroxide; OCP, oral contraceptive pill; STI, sexually transmitted infection; WBC, white blood cell. Adapted from Zitelli BJ, Davis . Atlas of Pediatric Physical Diagnosis. rd ed. ondon osby olfe; .

20

49

Gynecology

LCERATIVE IORER ee Box  for list of infetious and noninfetious auses t should be noted that aphthous ulers are the ost oon ause of vulvarvaginal ulers in hildren Bx 20.

Differential Diagnosis of Genital Ulceration

Gynecology

Venereal • ainful  erpes herpes simplex virus  hancroid Haemophilus ducreyi • ainless  yphilis Treponema pallidum  Granuloma inguinale Klebsiella granulomatis  ymphogranuloma venereum Chlamydia trachomatis serotypes 1–3 Oter • pthous ulcers most common cause in children • iral varicella psteinarr virus • raumaoreign body • ehçet’s disease • dverse drug reaction • nammatory boel disease

20

A. erpes simplex irus  Etiology HV- HV-  linical manifestations nonpriar or reurrent disease presents ith painful genital ulers radiulopath dsuria and tender inguinal lphadenopath Priar infetion an have additional severe sstei sptos fever alaise asepti eningitis  nestigations viral ulture or PC of lesion ust unroof lesion to sab pe-spei serologies have liited utilit given high arriage rates of virus  reatment oral antivirals alovir valalovir failovir For reurrent episodes an be treated episodiall or ith hroni suppressive therap B. Syphilis  Etiology aused b spirohetal bateria Treponema pallidum  linical manifestations a Priar sphilis painless uler hanre at inoulation site ith inguinal lphadenopath b eondar sphilis disseinated infetion auses rash fever lphadenopath uous lesions ondloa lata alopeia and asepti 498 eningitis

Gynecology

 atent sphilis earl , ear late . ear a last  to ears d ertiar sphilis presents ith guatous ardia or neurologial disease  nestigations erologi testing to diagnose sphilis should inlude the use of both nontreponeal P VD and treponeal treponea pallidu partile agglutination uoresent treponeal antibod absorption tests e use of onl one test is insu¤ient for diagnosis beause serologi testing espeiall nontreponeal tests an be assoiated ith false positive results False negative results an also our in earl disease or advaned iunosuppression  reatment a Priar seondar and earl latent sphilis one tie  dose of Benathine peniillin G  illion units b ate latent and tertiar sphilis exluding neurosphilis Benathine peniillin G  illion units  eekl 3  eeks  P uantitative assa an be repeated at   and  onths for treatent response CERVICITI enition naation of the endoervix A. hlamydia trachomatis  Epidemiology ost oonl reported   linical manifestations asptoati or an present ith uopurulent ervial disharge interenstrual bleeding urethritis dsuria puria vulvovaginitis oon in prepubertal patients protitis pelvi inaator disease PD pelvi pain sstei sptos andor perihepatitis  iagnosis AA of rst-ath urine vaginal sabs or endoervial sabs are the ost sensitive tests

20

PEARL rine samples submitted for nucleic acid amplication testing should be collected from the initial urine stream ithout precleansing the genital area.

 reatment see able  for antibiotis est of ure in  eeks reoended for prepubertal patients pregnant patients or suspeted nonadherent patients  omplications hroni pelvi pain infertilit etopi pregnan reative arthritis 499

Gynecology

B. eisseria gonorrhea  linical manifestations asptoati or an present ith purulent ervial disharge interenstrual bleeding urethritis dsuria puria vaginitis oon in prepubertal patients bartholinitis protitis PD pelvi pain sstei sptos perihepatitis pharngitis andor onuntivitis  iagnosis urine vaginal and endoervial AA is oonl used Cultures an deterine suseptibilities a be negative if , hours fro exposure Cultures an be obtained fro endoervial vaginal retal and pharngeal loations depending on the tpe of sexual ativit  reatment see able  for antibiotis Cobination therap reoended beause of high resistane rates and for hladia otreatent est of ure is reoended a¡er  to  eeks in prepubertal hildren pregnant patients and hen there is a high likelihood of resistane  omplications hroni pelvi pain infertilit etopi pregnan reative arthritis disseinated gonooal infetion arthritis deratitis endoarditis and eningitis

able .

20

Treatment  nmliate Angenital an Parngeal exuall Tranmitte Inetin

ieae

Treatment

hlamydia 9 years and .4 g

ithromycin 1 g  3 1 dose  doxycycline 1 mg   3  days

a

Gonorrhea ,9 years  #4 g

eftriaxoneb  mgg  3 1 dose max  mg  cexime 8 mgg   3  doses max 4 mgdose  aithromycin  mgg  3 1 dose max dose 1 g

a

eftriaxoneb  mg  3 1 dose  cexime 8 mg  3 1 dose  aithromycin 1g  3 1 dose

Gonorrhea 9 years  .4 g a

Dosin reimen also recommended for postexposure prophylaxis, as it covers for both chlamydia and onorrhoea. b eftriaxone is the preferred cephalosporin. exime should only be used if ceftriaxone is unavailable. IM, ntramuscular; PO, orally. Adapted from he ospital for ic hildren eormulary, .

500

PELVIC INFLAATOR IEAE  enition Asending infetion of the feale reprodutive trat hih an inlude endoetritis salpingitis oophoritis perihepatitis and pelvi peritonitis

Bx 20.

Diagnostic Criteria for Pelvic Inammatory Disease

Gynecology

 Etiology Polirobial infetion ith baterial auses inluding s C. trachomatis, Neisseria gonorrhoeae endogenous genital organiss Mycoplasma genitalium, Ureaplasma urealyticum anaerobi organiss and faultative bateria a be auired sexuall postabortal or postinstruentation  linical manifestations a present ith pelvi pain abnoral uterine bleeding postoital interenstrual enorrhagia dspareunia vaginal disharge andor inreased urinar freuen ore severe disease an be assoiated ith sstei sptos and peritoneal signs  iagnosis Clinial diagnosis ee Box  for riteria Pregnan test and other  tests a be helpful to rule out other diagnoses

exuall atie emale it eli ain an n alternatie aue it • ervical motion tenderness  • terine tenderness  • dnexal tenderness urtie riteria • emperature .38.3° • bnormal cervical or vaginal discharge • s on et mount of vaginal discharge • levated  • onrmed gonorrhea or chlamydia infection

20

CRP, reactive protein; ESR, erythrocyte sedimentation rate; WBC, white blood cell. Data from enters for Disease ontrol and Prevention  exually ransmitted Diseases reatment uidelines.

 anagement a ee able  for antibioti guidelines o threshold for treatent given the severit of the disease reat all sexual ontats b Consult gneolog  Hospitalie if onerns about opliane severe illness iunosuppression pregnan tuboovarian absess unable to tolerate oral ediations or failed oral therap d Patients treated in abulator setting ust be folloed up for iproveent in  to  hours

1

able .3

Treatment  Peli Inammatr ieae

Inatienta

Outatient

• referred regimen efoxitin  mgg dose  h max  gdose  doxycyclineb 1 mgdose  1h • lternative regimen lindamycin 13 mg gdose  8h max 9 mgdose  obramycin  mgg  4h max 8 mgdose

• referred regimen eftriaxone  mg  as a single dose  doxycyclineb 1 mg   3 14 days 6 metronidaole  mg   3 14 days • lternate regimen evooxacin  mg  daily 3 14 days 6 metronidaole  mg   3 14 days

a

 therapy can be discontinued  h after clinical improvement. ral therapy should consist of doxycycline  m P BD or clindamycin  mdose D to complete a day course. b Doxycycline is not recommended in children under  years of ae.

Gynecology 20

IM, ntramuscular; IV, intravenous; PO, orally. Adapted from he ospital for ic hildren eormulary, .

 omplications Chroni pelvi pain infertilit etopi pregnan or tuboovarian absess GENITAL ART CONLOATA ACINATA  Etiology Caused b HPV subtpes eg    linical manifestations ide variet of appearanes but are o¡en esh-olored and aulioer-shaped or plaue-like a be loated on vulva perineu perianal area vagina ervix or periurethral area ¡en asptoati but a have ild sptos irritation dsuria  iagnosis Clinial if unertain an do shave biops  reatment a defer treatent if asptoati as an ill spontaneousl regress f sptoati or distressing an treat ith topial rst-line or proedural ethods opial ethods inlude trihloroaeti aid iiuiod  or  rea and Podophllotoxin Crotherap laser ablation and surgial exision reserved for large or realitrant arts reatent is o¡en prolonged and arts a reur  Preention oe forulations of the HPV vaine prevent against genital arts

OVARIAN/ADNEXAL PATHOLOGY OVARIAN AE ee ables  and  able .4

imle Veru Cmlex Oarian a n ltraun

imle • nilateral • hin alled • o septations • ystic • o vascular o

502

Cmlex • ilateral • hic alls • eptations • olid components • ascular o ithin alls • ssociated ascites

able .

Oarian ae  Age

etalneonatal follicular cysts

repubertal ovarian masses

Clinial anietatin

ierential iagni

iagni

anagement

 etected on routine fetal  or as abdominal mass in neonatal period  ostly unilateral

 G and G tract anomalies as ell as other intraabdominal pathologies.  varian malignancy is rare

our  criteria female sex nonmidline regular cystic structure normal G tract normal G tract

 bservation ith serial ultrasounds until 4– months.  urgical management may be arranted for acute torsion enlarging complex or symptomatic cysts and for those that have not regressed by 4– months.  otential role for postnatal aspiration of simple cysts . cm to reduce the ris of torsion.  9 of simple cystic masses ill have resolved on repeat .

aries asymptomatic abdominal mass chronic abdominal pain sense of abdominal fullness or acute abdominal pain

road hormonesecreting cysts cune lbright syndrome neoplasms paraovarianparatubal cysts mesothelial cysts and true precocious puberty

 plus oppler if concern for torsion. orup for precocious puberty if secondary sex characteristics present

 aries observation recommended for simple cystic masses ith repeat  in 4–8 ees 9 of simple cystic masses ill have resolved on repeat .  urgery may be reuired for torsion or prevention of torsion.  oncerning  features solid compo nents bilaterally ascites septations me tastases mandate orup for malig nancy tumor marers bhG   6 1  or  scan surgical removal and pathological examination

Cmliatin

varian torsion higher ris ith cysts . cm intracystic hemorrhage rupture and GG obstruction

Continued

20

3

Gynecology

Gynecology

20

504

able .

Oarian ae  Age—nt’

ostmenarchal ovarian masses

Clinial anietatin

ierential iagni

iagni

anagement

aries incidental nding abdominal fullnesspain menstrual irregularities or GG obstruction

 road functional cysts follicular corpus luteal  as ell as benign and malignant neoplasms  onovarian etiologies in clude tubal tuboovarian abscess paratubal cyst ectopic pregnancy G appendiceal abscess rohn’s disease and G pelvic idney causes

  plus oppler if concern for torsion of malignancy  regancy test  f complex or persistent cyst can order tumor marers bhG   1 and 

 bservation analgesia and repeat ultrasound in 4–8 ees for asymptomatic simple cysts most ill involute spontaneously  ombined hormonal contraception can be used to prevent ne cyst formation.  or cysts that are symptomatic persis tent increasing in sie or ith features concerning for malignancy surgical management may be arranted

Cmliatin

AFP, Alpha fetoprotein; b hCG, bhuman chorionic onadotropin; CA-125, cancer antien ; CT, computed tomoraphy; GI, astrointestinal; GU, enitourinary; LDH, lactate dehydroenase; MRI, manetic resonance imain; PCOS, polycystic ovary syndrome; US, ultrasound.

Bx 20. Differential Diagnosis of Pelvic Pain of Gynecological rigin • • • • • • • •

rimary dysmenorrhea ndometriosis natomic obstructions e.g. imperforate hymen vaginal septum mullerian anomalies ittelschmer midcycle “ovulation pain” elvic inammatory diseasetuboovarian abscess varian cysts ruptured hemorrhagic dnexal torsion regnancy complications ectopic pregnancy spontaneous abortion

Gynecology

ANEAL TORION  enition isting of the ovarfallopian tube ausing olusion of the ovarian artervein a be oplete or interittent  Etiology nfants and hildren a have torsion of noral adnexa n postenarhal patients ovarian torsion is usuall assoiated ith adnexal ass neoplas or funtional sts  linical manifestations “aves” of aute pelvi pain o¡en assoiated ith nausea Patients a have a palpable pelvi ass and a be febrile  ierential ee Box  for a di¢erential diagnosis of pelvi pain

20

 nestigations a aborator studies noral to ildl elevated BC b-hCG to rule out pregnan b aging Doppler ultrasound a deonstrate enlargeent and edea of the involved ovar an ovarian ass absene of Doppler o to the ovar andor peripheraliation of ovarian folliles PITFALL dnexal torsion is a clinical diagnosis that is nt rule ut by the presence of ovarian blood o on oppler ultrasound.

 anagement Urgent laparosopi detorsion 6 ovarian steto ophoropex a be onsidered in selet ases POLCTIC OVARIAN NROE PEARL ince 3 of adolescents have polycystic ovaries ultrasound is not reuired as part of the diagnostic evaluation of polycystic ovarian syndrome in this age group unless there is a clinical concern for other causes of amenorrheaoligomenorrheahyperandrogenism.



Gynecology

 linical presentation Hperandrogenis hirsutis ane or bioheial hperandrogenis and evidene of anovulation infreuent andor irregular enses a also involve obesit and etaboli sndroe  iagnosis videne of anovulation infreuent andor irregular enses AD linial or bioheial hperandrogenis n adolesents polsti ovaries are not part of the diagnosti riteria  reatment ¡en under the are of a gneologist treatent inludes eight loss le regulation ith obination or progesterone-onl ontraeptives and ediations to redue hirsutis ie obined horonal ontraeption CHC spironolatone and protet the endoetriu fro long-ter risk of endoetrial hperplasia and alignan PC is also assoiated ith etaboli sndroe and patients should be sreened for diabetesgluose intolerane dslipideia and hperholesteroleia

DYSMENORRHEA AND PELVIC PAIN

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 Etiology Can be priar not aused b pelvi patholog or seondar as a result of pelvi patholog  istory Histor of the pelviabdoinal pain inluding assoiated sptos eg dsuria dsheia pelvi pressure dspareunia if sexuall ative other G or GU sptos enstrual histor sexual histor histor of ontraeptive use anageent to date ediations tried et  Physical examination hould inlude usuloskeletal and abdoinal exainations f patient is sexuall ative a gneologi exaination inluding  testing a be appropriate A retoabdoinal or bianual exaination a douent ervial otion tenderness andor adnexal tenderness asses  ierential ee Box  for gneologial auses of pelvi pain and dsenorrhea  nestigations A b-hCG to rule out pregnan Consider urinalsis and ulture to rule out U f the patient is sexuall ative ervialvaginal sabs an be sent for gonorrhea hladia and trihoonas f there is diagnosti unertaint or a assuterine anoal is suspeted a pelvi ultrasound should be ordered f a patient fails edial anageent a diagnosti laparosop a be arranted

PRIAR ENORRHEA  enition Crap pelvi pain that ours during enstruation and is not a result of patholog Generall ours a¡er the establishent of a regular enstrual le hih a our  to  ears a¡er enarhe Dsenorrhea that ours at the onset of enarhe is less oon and 506 should raise suspiion for an obstrutive anoal

ENOETRIOI  enition Presene of endoetrial glands and stroa outside of uterus usuall earl stage in adolesents but a be assoiated ith signiant pain endoetrioas less oon at this age  linical manifestations Dsenorrhea lassiall a start ust before enses li andor non-li pain deep dspareunia pain during sexual interourse dsheia pain during defeation dsuria and abdoinal tenderness A bianual exaination a reveal ervial otion tenderness a xed retroverted uterus uterosaral nodularit and adnexal tenderness 6 ass ¡en no ndings on bianual exa in adolesents t a eventuall ause infertilit and hroni pelvi pain  iagnosis Diagnosed liniall based on sptos and response to edial anageent Denitive diagnosis reuires laparosop ith biops and histologi onration but this is not reuired for anageent  anagement Conservative easures inlude ADs obined horonal ontraeption pill path or ring progesterone-onl ethods norethindrone aetate dienogest DPA progestinontaining UD and suppression of the hpothalai-pituitarovarian HP axis ith gonadotropin-releasing horone agonists urger a be reuired for treatent-resistant ases or for infertilit nvolveent of ultidisiplinar hroni pain teas should be onsidered on an individualied basis

Gynecology

 Etiology xess prodution andor sensitivit to uterine prostaglandins hih auses uterine raping as ell as other sptos inluding diarrhea nausea voiting and headahe  anagement Pain anageent ith ADs Cobined horonal ontraeption pill path ring dienogest depoprovera progestinontaining UD

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IPERFORATE HENTRANVERE VAGINAL EPT  enition An iperforate hen is hen the hen laks an opening and is obstruting the vaginal orie A transverse vaginal septu results fro a failure of fusion and analiation of the upper and loer vagina  linical manifestations Both onditions a result in abdoinal pain and heatoolpos blood lled vagina Vaginal distension a result in obstrutive urinar sptos dsheia and bak pain n phsial exaination an iperforate hen a be seen as bluish bulge at the vaginal orie A transverse vaginal septu results in a shortened vagina and possible vaginal ass as a result of heatoolpos Figure    reatment Both onditions reuire surgial repair

Figure 20.2 Hematl Uterus Hematometra Cervix

Hematocolpos

Gynecology

Complete occlusion of lower vagina by transverse septum or imperforate hymen

odied fro ith P Netter’s bstetrics  ynecology rd ed lsevier 

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ABNORMAL UTERINE BLEEDING Abnoral uterine bleeding AUB refers to enstrual bleeding that ours outside the noral range and inludes absene of enses bleeding that is abnoral in freuen duration or volue

AENORRHEA Also see Chapter  ndorinolog  enition Primary amenorrhea absene of enses b age ears in the presene of seondar sex harateristis or b age ears in the absene of seondar sex harateristis or secondary amenorrhea absene of enses for . onths a¡er douented enarhe or for . onseutive les rregular enstrual bleeding is dened as enstrual le variation . das All three onditions have a siilar di¢erential and orkup  istory enstrual histor sexual histor pubertal histor groth presene or absene of pain ediations histor of hroni or sstei illness sptos of other horonal onditions throid disease hperprolatineia hperandrogenis priar ovarian insu¤ien et stressors eg eating exerise  Physical examination anner staging signs of hirsutis ane aanthosis signs of Cushing sndroe throid exaination neurologi and gneologi exainations for signs of viriliation and to assess paten 508 of outo trat

ierential iagni  AmenrreaIrregular entrual Bleeing

ypothalamic

• • • • • • •

nterior pituitary

• hyroid • rolactin • eoplasm

drenal

• eoplasm • ushing • ongenital adrenal hyperplasia

varygonadal

• • • • • •

novulation olycystic ovarian syndrome eoplasm ysgenetic gonads remature ovarian insufciency genetic autoimmune idiopathic ancer therapy

terus

• • • •

regnancy ullerian anomaly ndrogen insensitivity syndrome edications

• • • • •

ullerian anomaly mperforate hymen ransverse vaginal septum ndrogen insensitivity syndrome aginal agenesis

uto tract

iet tress xercise hronic illness entral nervous system lesion allman syndrome onstitutional

Gynecology

able .

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 ierential diagnosis ee able  for di¢erential diagnosis and Figure  for diagnosti approah  nestigations a abs CBC b-hCG H FH estradiol testosterone DHA androstenedione prolatin H and -HP ther investigations depending on linial presentation and initial test results inlude karotpe Fragile  antiadrenal antibod antithroid Ab A ortisol ACH stiulation test et b f H is noral and prolatin is elevated need to investigate for other auses of hperprolatineia histor phsial 6  brain  Progesterone hallenge  g oral 3 – das to ii luteal phase of enstrual le and attept to indue ithdraal bleed bleeding iplies adeuate endogenous estrogen and funtional outo trat Aenorrhea is likel attributed to anovulation 9  reatment pei to underling proess

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Gynecology

510 Figure 20. iagnti Ara t Amenrrea

History Physical examination Pelvic ultrasounda

External or internal genital anomaly

Diff Dx: PCS, ate onset CH, mors

Do: Urine hCG FSH, E, Prolactin, SH, F

Do: Urine hCG FSH, H, SH, Free , Prolactin ndrogens: estosterone , free , DHES  HProgesterone HC Fasting lipids, inslin, glcose  hr G

↓SH, ↑F

↑SH, ↓F

Uters asent

↑Prolactin, ormal SH

ormal FSH, Prolactin, SH, Free

↑FSH

mperforate hymen ranserse aginal septm aginal agenesis genesis of the cerix

Diff Dx: ndrogen insensitiity S , genesis of the ters H

Uters asent and iriliation

Diff Dx: αredctase deficiency Partial androgen insensitiity ntersex disorders

Do: estosterone, E, H, FSH aryotype for S, intersex disorder Pelic 

Hypothyroidism C

C

Diff. Dx: Prolactinoma diopathic hyperprolactinemia ther C tumors Drugs Pregnancy

Do: repeat Prolactin Cranial 

ormal ithdraal to progestin normal E

o ithdraal to progestin

Diff. Dx: HA Chronic disease Celiac disease PC

Diff. Dx: P urner yndrome adiation chemotherapy Autoimmune oophoritis Fragile  premutation carrier alactosemia ther

Do: aryotype and Fragile  testing creen for thyroid and adrenal antiodies celiac disease autoimmune disorders

Normal E2 Diff. Dx: HA Failure to take progestin Asherman’s syndrome rare

Low E2 Diff. Dx: HA Anorexia nerosa Chronic disease Pituitary lesion

Consider: Estrogen/Progestin trial Hysteroscopy a

Pelic ultrasound may not e needed in all cases of secondary amenorrhea.

AS Androgen insensitivit sndroe T free   estradiol A hpothalai aenorrhea TT oral gluose tolerane test  priar ovarian insu¤ien M aer–okitansk–üster–Hauser sndroe Diagnosti Approah to Aenorrhea Adapted fro ans aufer oldstein’s ediatric  Adolescent ynecology th ed ippinott illias  ilkins 

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11

Gynecology

HEAV ENTRAL BLEEING  Pathophysiology Heav enstrual bleeding refers to inreased enstrual losses that interfere ith ualit of life hen this bleeding ours at regular intervals but ith exessive volue or duration it is onsidered ovulator in nature  Etiology ee Box  for di¢erential diagnosis Bx 20.

Gynecology

• • • • • • • • • • •

Differential Diagnosis of Heavy Menstrual Bleeding

novulatory bleeding e.g.  immature  axis at onset of menarche regnancy complications e.g. ectopic pregnancy threatenedincompletecomplete abortion lood dyscrasia e.g.  von illebrand disease iatrogenic liver disease nfections e.g.  endometritis ndocrine disorders e.g. hypothyroidism  tructural lesions e.g. polyp broid—rare in adolescents rauma accidental vs. abuse eoplasms ovarian vaginal cervical opper  edications ongenital e.g. uterine vascular malformation

HPO, ypothalamicpituitaryovarian; ITP, immune thrombocytopenic purpura; IUD, intrauterine device; PCOS, polycystic ovary syndrome; PID, pelvic inammatory disease.

20

 istory enstrual histor onset tiingpattern aount of bleeding pubertal histor sexual and  histor bleeding histor freuent epistaxis eas bruisingbleeding bleeding into oints heaturia fail histor of bleeding disorders past edial histor and ediations and assoiated sptosrevie of sstes boel or bladder sptos sptos of hpothroidis  Physical examination hould inlude vital signs for assessent of heodnai stabilit exaination to look for stigata of sstei illnesses ie hpothroidis bleeding disorders anner staging exaination to rule out other soures of bleeding retal urethral vaginal ervial onsider a pelvi and bianualretoabdoinal exaination to look for strutural auses of bleeding pregnan and alignan based on historlinial presentation  nestigations ust do a b-hCG to rule out pregnan ther tests inlude a CBC oagulation prole H prolatin Consider tests for platelet funtion and Von illebrand disease if severe bleeding heav enstrual bleeding present sine enarhe andor other features on histor onerning for bleeding disorder esting for hladia gonorrhea and trihoonas should be done if the patient is sexuall ative Although strutural abnoralities broids polps et are rare in ado512 lesents a pelvi U a be onsidered if rst-line tests are negative

able .

Treatment r Aute Hea entrual Bleeing

il inimal drop in b hemodynami cally stable mild bleeding on examination

Gynecology

 reatment a Aute heav enstrual bleeding ensure heodnai stabilit reat bleed ith tranexai aid in onuntion ith tapering ourse of horonal therap for spei treatent regiens see ables  and  Follo aute treatent ith aintenane therap see later b Chroni heav enstrual bleeding iron suppleentation ADs tranexai aid CHC pill path ring dienogest o¢-label for AUB progesterone-onl options norethindrone edroxprogesterone aetate progesterone-onl UD

• rovide reassurance • onsider s tranexamic acid  progesterone only methods depending on impact on uality of life and desire for contraception • onsider oral iron therapy

erate ignicant drop in b hemodynam ically stable moderate bleeding on examination

• • • •

ombined  or progesterone only taper ranexamic acid onsider oral or  iron therapy aintenance therapy folloing stabiliation

20

eere ignicant drop in b hemody namic instability severe bleeding on examination

• emodynamic stabiliation • ombined  or progesterone only taper   estrogen folloed by progesterone taper • ntiemetic therapy • ranexamic acid • onsider blood transfusion  iron infusion • n severe circumstances dilation and curettage may be reuired andor intrauterine balloon tamponade • aintenance therapy folloing stabiliation

CHC, ombined hormonal contraception; Hb, hemolobin; IV, intravenous; NSAID, nonsteroidal antiinammatory dru; OCP, oral contraceptive pill.

13

able .8

Hrmnal Teraie r Aute Hea entrual Bleeing

Tera

e

Rute

Initial Freuen

onugated estrogen

 mg



very 4– h

3–3 mcg ethinyl estradiol combined pilla

1 tablet

ral

very  h

edroxyprogesteronea

1– mg maximum 8 mgday

ral

very –1 h

orethindrone acetatea

–1 mg

ral

very  h

a

Gynecology

xamples of taperin reimens with plan for shortterm followup to determine plan for loner term therapy - 30–35 mcg ethinyl estradiol combined pill:  tablet h 3  days, then  tablet h 3  days, then  tablet h 3  days, then continue  tablet daily - Progesterone-based pill (medroxyprogesterone or norethindrone acetate):  dose –h 3  days, then  dose h 3  days, then  dose daily IV, ntravenous. odied from aamid , ass A, Dietrich J. eavy menstrual bleedin in adolescents. APA ommittee pinion. J Pe Ae Ge. ;–.

20

514

IRREGLAR ENTRAL BLEEING  Pathophysiology xessive nonli irregular uterine bleeding is onsidered anovulator previousl referred to as dysunctional uterine bleeding U Unopposed estrogen stiulation of the endoetriu an lead to endoetrial hpertroph and unoordinated shedding of the endoetrial lining is an lead to irregular and infreuent enstrual bleeding that is o¡en heav and prolonged  Etiology n the rst  to  ears postenarhe this is ost oonl beause of an iature hpothalai-pituitar-ovarian axis but an also be due to other etiologies ie PC pregnan stress eight hanges endorinopathies ote overlap beteen etiolog of aenorrhea partiularl seondar and irregularinfreuent enstrual bleeding ee able  for di¢erential diagnosis  istory enstrual histor onset tiingpattern aount of bleeding pubertal histor sexual histor past edial histor and ediations assoiated sptosrevie of sstes ie sptos of hpothroidis hperprolatineiaprolatinoa hperandrogenis soial histor stress anxiet disordered eating

PITFALL

 Physical examination Assess heodnai stabilit look for stigata of sstei illnesses eg aanthosis nigrians hirsutis in PC buffalo hup abdoinal striae anner staging exaination to rule out other soures of bleeding retal urethral vaginal ervial onsider a pelvi and bianualretoabdoinal exaination to look for strutural auses of bleeding or pregnan  nestigations b-hCG CBC oagulation prole H prolatin FH H estradiol testosterone androstenedione DHA -HP  reatment anageent of aute bleeding as per able  longer-ter anageent ith lifestle hanges exerise diet CHC pill path ring progesterone-onl ediations norethindrone edroxprogesterone aetate depo provera progesterone-onl UD

Gynecology

 urine pregnancy test bhG should be performed in any postmenarchal female presenting ith heavy vaginal bleeding.

20

1

CHAPTER

21

Hematology Adam Yan • Vanja Cabric • Soumitra Tole • Michaela Cada

Common abbreviations Anemia Hemoglobinopathies Thrombocytopenia Neutropenia Bleeding disorders Thrombosis Transfusion medicine

516

COMMON ABBREVIATIONS ACS ANC BMA BMT DAT DIC DT    IIIC D  IT  S I IT

I II M

acute chest syndrome absolute neutrophil count bone marrow aspirate bone marrow transplant direct antiglobulin test disseminated intravas cular coagulation deep vein thrombosis erythropoietin actor eg  5 actor  resh roen plasma actor III coagulant glucosephosphate dehydrogenase growth hormone heparininduced thrombocytopenia hydroyurea hemolytic uremic syndrome insulinlie growth actor  immune thrombocytopenia ormerly idiopathic thrombocytopenic purpura intravenous uids intravenous immunoglobulin lowmolecularweight heparin

MC MC NAIT NAT NBS NTDT  T pBC D SCD S TDT TIA TIBC TT  C  T D v

mean corpuscular hemoglobin mean corpuscular volume neonatal alloimmune thrombocytopenia neonatal autoimmune thrombocytopenia newborn screen nontransusion dependent thalassemia pulmonary embolism platelets paced red blood cell red blood cell distribution width sicle cell disease systemic lupus erythematosus transusion dependent thalassemia transient ischemic attac total ironbinding capacity thrombotic thrombocy topenic purpura unractionated heparin vasoocclusive crisis ventilationperusion venous thromboembolic event von illebrand disease von illebrand actor

Hematology

Also see page viii or a list o other abbreviations used throughout this boo

21

ANEMIA APPROACH TO ANEMIA  Denition: reduction in hemoglobin concentration hematocrit or red blood cell number Normal b and hematocrit values vary with age  Clinical presentation: pallor atigue weaness irritability decreased eercise tolerance shortness o breath  Diagnostic approach: see igure 

517

21

Hematology

518 Figure 21.1 Approach to Anemia

Blood smear & MCV

Hypochromic microcytic (MCV↓

Iron studies (Fe, TIBC, Ferritin) Inflammatory markers (CRP, ESR) Hb electrooresis ead leel Iron deficiency anemia Anemia of chronic disease Thalassemia Lead poisoning Siderolastic anemia

↑)

ormochromic (MCV )

eticlocyte ↓

eticlocyte ↑

BC, PT Iron studies Cr, rea Tyroid function BBB bdominal S

Hatolobin, H Bilirubin T, lutinins Hb analysis RBC enyme assays

BC  PT ↓

BC  PT 

Leemia Aplastic anemia Infection Hypersplenism

Infection arly iron deficiency rgs enal disease Hypothyroidism Acte lood loss TC iamondBlacfan

BC  PT ↑ Infection

Acte lood loss Hemolysis (see Fiure )

onmegalolastic

FTs Tyroid function PTT, IR BBB Lier disease Hypothyroidism Asplenia Aplastic anemia rgs MS on syndrome anconi anemia iamondBlacfan Actie hemolysis

Megalolastic

B Folate B deficiency olate deficiency rgs Inorn errors of metaolism

BMA Bone marrow aspirate BMB bone marrow biopsy Cr creatinine CRP Creactive protein DAT direct antiglobulin test ESR erythrocyte sedimentation rate Hb hemoglobin INR international normalied ratio LDH lactate dehydrogenase LFTs liver unction tests MCV mean corpuscular volume MDS myelodysplastic syndrome N normal PLT platelets PTT partial thromboplastin time RBC red blood cell TEC transient erythroblastic anemia TIBC total ironbinding capacity US ultrasound WBC white blood cell  g low h high

 Laboratory ndings: see Table 

aorator Features o arious Tpes o Anemia

Test

Aplastic Anemia

Thalassemia

Iron ecienc Anemia

Anemia o Chronic isease

Hb

g

g

g

g

MCV

h

g

g

N or g

MCH

N

g

g

N

RDW

N or h

N or h

h

N

Iron

N

N or h

g

N or g

TIBC

N

N or g

h

g

Ferritin

N

N or h

g

N or h

Transferrin

N

N or g

h

g

Reticulocte count

g

N or h

g

N or g

Hematology

Table 11

Hb, Hemoglobin; MCH, mean corpuscular hemoglobin; MCV, mean corpuscular volume; N, normal; RDW, red blood cell distribution width; TIBC, total iron-binding capacity.

PHIOOICA ANEMIA OF INFANC  Healthy infants: b is high at birth then rapidly declines to nadir o  to  g at  to  wees o age because o decreased erythropoiesis  Premature infants: may have more rapid and etreme decline to  to  g at  to  wees  nce at the nadir  production is stimulated and b increases  Exaggerated nadir: can occur with BC transusions mild hemolytic diseases and prematurity  reatment: generally not reuired iron supplementation may be bene¡cial

21

IRON EFICIENC ANEMIA  ost common pediatric anemia ith to peas a Toddlers ecessive mil intae poor dietary iron intae b Teenagers rapid growth poor dietary iron intae menstrual losses in emales  Presentation: pallor irritability anoreia lethargy pica glossitis angular stomatitis oilonychia 519

Hematology 21

 Preention: many cases preventable with nutritional counseling a Breasted inants introduce ironrich oods at  to  months o age b Nonbreasted inants ironorti¡ed ormula c Toddlers limit mil intae to , mday introduce ironrich oods  reatment a Dietary counseling and education b Trial o iron supplementation in toddlers with hypochromic microcytic anemia without urther investigation i typical history o nutritional de¡ciency i Administer  hour beore or  hours a¢er dairy eggs tea wholegrain bread cereal ii itamin C coadministration enhances absorption c Monitoring chec b in  to  wees to ensure response and adherence d Duration treat or  months a¢er b normalies to replenish iron stores e ailed response to therapy , g increase in  month i nsure correct dose and administration ii I ully adherent investigate or chronic inammatory diseases blood loss thalassemia concurrent B or olate de¡ciency PITFA oi transfusion in iron ecienc aneia unless atient is eonaicall unstable

HEMOTIC ANEMIA  Pathophysiology: BCs destroyed and removed rom circulation dropping b  stimulates BC production in bone marrow increas ing reticulocyte count  History: aundice anemia atigue headaches syncope dar urine recent inection abdominal or bac pain gallstones transusions ever splenomegalysplenectomy amily history medications  Physical: tachycardia dyspnea pallor aundice splenomegaly growth retardation thalassemic acies eg rontal bossing leg ulcers  Diagnostic approach: see igure  520

Figure 21.2 Approach to Hemoltic Anemia History and physical exam concerning for hemolytic anemia nitial investigations to confirm hemolysis B  differential Elevated H, bilirubin Elevated reticulocytes ecreased haptoglobin Abnormal blood smear

DAT negative ed cell abnormalities on blood smear Enyme assays Hemoglobin analysis I, P, fibrinogen Membrane defects Hereditary spherocytosis Hereditary elliptocytosis Hereditary stomatocytosis Paroxysmal nocturnal hemoglobinuria Enzyme defects P deficiency Pyruvate inase deficiency Hb abnormality icle cell disease halassemia

DAT positive, ie, immne hemolysis AB and cross match AA Primary arm autoimmune (Ig old autoimmune (IgM Paroxysmal cold hemoglobinuria (Ig Secondary Autoimmune disorders Evans syndrome Immunodeficiency Malignancy Infection (Mycoplasma, EB, postviral Posttransplant

Hematology

DAT

21

ragmentation hemolysis I, P, H Prosthetic heart valve asabachMerritt EM Burn Systemic process rugs  toxins iver disease Infection ilson disease Hypersplenism

ANA Antinuclear antibody CBC, complete blood count DAT direct antiglobulin test DIC disseminated intravascular coagulation EBV, psteinBarr virus ECMO etracorporeal membrane oygenation G6PD glucosephosphate dehydrogenase HUS hemolytic uremic syndrome Ig immunoglobin INR international normalied ratio LDH lactate dehydrogenase PTT partial thromboplastin time TTP thrombotic thrombocytopenic purpura

HEMOGLOBINOPATHIES ICE CE IEAE C  Pathophysiology: mutation in bglobin gene results in hemoglobin S bS ormation deoygenated bS molecules orm characteristic sicle shape  Presentation: o¢en diagnosed during newborn screening a I not maority present with pain inants commonly present with 51 dactylitis

 Diagnosis: suggested by anemia with sicle cells target cells polychro masia on blood smear a Con¡rmed by b analysis see Table  Hemogloin Analsis in icle Cell isease

Table 1

% Hb H ariant

N

Hematology

H g

MC

HA

H

HA2

HF

HC

Hb sicle cell trait

N

N

N

55–6

–

–

—

—

Hb sicle cell aneia

F

6–

N or h



5–95

–

5–15

—

Hbb° talasseia

F

7–9

g



7–

–5

1–

—

Hbb1 talasseia

F

9–1

g

1–

6–75

–5

1–

—

HbC isease

FC

1–1

N



5–5

—

—

5–5

Hb, Hemoglobin; MCV, mean corpuscular volume; NBS, newborn screen. Modied from Driscoll MC. icle cell disease. Pediatr Rev. ;–.

21

 Clinical manifestations and complications: see Table  Table 1

Clinical Maniestations o icle Cell isease

Presentation

escription

cute cest snroe C

Ne inltrates on CR it $1 ne resirator stos feer cou snea sutu rouction oia

creening

ee Fiure 1 Consier RBC transfusion if Hb 1–15  belo baseline cane transfusion if rail eterioratin

Ma be seconar to infec tion ulonar infarct oentilation fat ebolis ulonar eea surer asta cronic oeia or a cobination of te aforeentione neia

Cronic onset at – onts of ae

lastic crisis

aroirus infection causes RBC alasia an reticuloctoenia resent it feer

522

Management

CBC it reticulocte count eer –6 onts

Folic aci rourea cronic transfusion if colications or stoatic uortie care an trans fusion as neee Monitor for colications C VC stroe seuestration

Table 1

Clinical Maniestations o icle Cell isease—cont’

Presentation

escription

creening

Carioo at

resents as eart failure

co  earl fro ae 1 ears

Cerebroas cular eents

Isceic stroe TI silent infarct because of siclin an trobosis in essels Heorraic seen ore in aults Hiest incience 1–9 ears of ae

Cronic lun isease

ulonar brosis restrictie lun isease a lea to cor ulonale

Dactlitis

ellin of orsal asects of ans an feet ,5 ears of ae ea 6–1 onts

astrointestinal colications

Bilirubin stones colecstitis eatoat esenteric asoocclusion

rot failure ubertal ela Infections

Transcranial Doler earl fro –16 ears of ae it initia tion of cronic transfusions for abnoral results

Hea MRIMR or CT cane transfusion analesia ration Control seiures B teerature

Hematology

Rare tout to be cause b brosis

Management

 saturation at eac isit Consier baseline FT at 1 ears nalesia ration

FTs an bilirubin earl

lectie colecstecto for recurrent colecstitis an allstones

ntrooetrics earl

Nutritional suleents

bnoral iune function because of slenic infarcts

rolactic enicillin until at least 5 ears of ae

t ris for infection it encasulate bacteria neuococcus enino coccus Hemophilus inuenza an osteoelitis it Salmonella an Staphylococcus aureus

Routine iuniations incluin annual in¢uena accine

e ulceration

nilateral or bilateral eial an lateral alleolar areas

cular

Retinoat ea

21

itional neuococcus eninococcus an H. inuenza accines cute see Fiure 1

taloloical ealuation earl fro 1 ears Continued

5

Table 1

Clinical Maniestations o icle Cell isease—cont’

Presentation

escription

riais

rolone erection . in

creening

Hration ar bat eer cise asturbation analesia  an transfusions

ea ae 5–1 ears ften in earl ornin can be reciitate b seual actiit

rolo consult for ossible enile asiration an irria tion or surical sunt

entual iotence if rolone

areneric aents seuoeerine orall or intracaernous

Hematology

scoloical

niet eression neuro conitie iairent oor scool erforance

Neuroscoetric testin if clinical concerns

Renal colications

Heaturia renal aillar necrosis nerotic sn roe renal infarcts renalconcentratin efect ostenuria enuresis renal eullar carcinoa

eru creatinine an urinalsis earl fro 1 ears

lenic seuestration

oolin of lare uantities of sicle RBCs in sleen

lenic alation b careiers at oe

een in Hb 6 onts– 5 ears an HbC Hbb an ae

21

resent it snea soc feer iral recii tant aboinal ain assie slenoeal Hb ro .  it reticuloctosis VC affectin bone

Isceic tissue inur fro obstruction of bloo ¢o b sicle RBCs reciitate b infection aciosis oia era tion slee anea eosure to etree teeratures

Management

Multiiscilinar care inolin social or sciatr s colo scool ainistration

Volue eansion an transfusion Totirs recur itin 6 onts consier cronic transfusions an slenec to after . eents

nalesia see Table 1 ration Monitor for colications e C

DD osteoelitis aascular necrosis ACS, cute chest syndrome; BP, blood pressure; CBC, complete blood count; CT, computed tomography; CXR, chest -ray; DDx, differential diagnosis; Hb, hemoglobin; LFTs, liver function tests; MRA, magnetic resonance angiography; MRI, magnetic resonance imaging; PFT, pulmonary function test; pRBC, paced red blood cell; TIA, transient ischemic attac; VOC, vaso-occlusive crisis.

524  anagement: see igure  Table  and later or urther details

Figure 21. Management o Ill Patients ith icle Cell isease History • Fever, infectious symptoms • Breathing difficulties • ature, duration, and severity of pain • edications already used

• • • •

Associated symptoms Vaccination history Baseline hemoglobin Previous SCD complications

Physical • Vital signs,  saturation • Pain score • ydration status • aundice

• • • •

Spleen sie Cardiorespiratory eurologic eam Signs of infection

f  °C oral or °C aillary, treat empirically ith V antibiotics ithin  minutes Don’t delay treatment to obtain reuired investigations

Investigations Blood culture CBC  diff, reticulocytes, type  screen C if concerns for ACS rinalysis  culture for all infants nfluena and P sabs as indicated

Initial infection management ighdose Ceftriaone  if V not possible evofloacin VP if severe betalactam allergy Add Vancomycin if seriously ill

Other symptoms anage as per able  and pain as per able 

21

55

Hematology

Suspected Pneumococcal meningitis: high-dose Ceftriaxone Seriously unwell: add ancomycin Concern for atypical pneumonia or positive ycoplasma PC: add Clarithromycin ild pneumonia: step down to Cefuroxime after  h Positive blood culture: narrow antibiotic choice based on susceptibility egative blood culture at  h and no other bacterial source identified: stop antibioticss

Yes

Red flags? Patient appears unwell Suspected sepsis, meningitis, stroe, ACS, aplastic crisis, splenic seuestration Shoc, cardiovascular instability, or respiratory distress  °C Age  year Previous severe sepsis or meningitis C  or  ×  or P  ×  Poor adherence missed antibiotics or clinic visits  return visits to E for same episode ncertain follow-up or family coping

Hematology

21

526 Inpatient management Analgesia, hydration, transfusions, and respiratory support as needed Early mobilisation and incentive spirometry

No

Outpatient management bserve for – h in E P analgesia with ibuprofen and acetaminophen Encourage P hydration  day course P antibiotics with Cefuroxime or evofloxacin Adust duration if source identified A, , etc eview home fever management Ensure follow-up arranged eview when to return to E Provide procedure for notification of positive blood cultures Provide clinic contact information

Discharge olerating P fluids and medications o respiratory distress, vitals stable ollow-up arranged

ACS Acute chest syndrome AOM acute otitis media CBC complete blood count CXR chest ray ED, emergency department PCR, polymerase chain reaction PLT platelets UTI urinary tract inection WBC white blood cell © ¥e ospital or Sic Children Adapted rom ever Guidelines for Management in Cildren it Sile Cell Disease rd ed Toronto N ospital or Sic Children 

Pain Management in asoocclusie Crisis

Pain eerit

Management

Comments

ischarge Planning

Mil

Acetaminophen ND iuproen  sceule

ie acetainoen an iburofen reularl for 1– as ten sitc to RN

If ain control aeuate itin 6 in of ei cation ainistration can iscare oe

tart it IV if oe  anaeent as not aeuate

Discare en toler atin  ¢uis an ain controlle on  eications

Morphinea 1–5    –6 RN a 15 ose Moerate seere

Morphinea 1–5   –6 a 15  ose or 5–1  IV – a 5  ose sceule Consier intranasal entanl ile establis in IV access if in seere ain

eere inatient

tart morphineb 1   IV loain ose a 75  folloe b continuous IV infusion  c Morphineb bolus 1–   IV 1–  RN a 15 ose

If no ain relief fro  itin –6 in ie IV bolus If ain relief itin   or it 1– IV oses cane to euialent  ose

If ain uncontrolle on  analesics or if ot erise unell ait to osital consier if $ IV oses reuire

Increase infusion rate in increents of  c   RN a 1 c

Discare en toler atin  ¢uis an ain controlle on  eications

Decrease infusion b 1 c as tolerate once ain controlle

Hematology

Table 1

21

Consier atient con trolle analesia u ll atients

Flui management if not toleratin  start IVF at 13 aintenance TFI If erate ie 1  bolus 9 NaCl o not bolus if concerns for C Monitor electroltes Nonpharmacological pain management eatin as ar bats assae structure actiit iaeristraction tecniues AC preention incentie siroetr siotera earl obiliation Managing opioi sie eects 5 or ocusate to reent constiation ntiistaines RN for ruritus Folloup arrane outatient Heatolo follou

a

mmediate-release form lternatively can use hydromorphone

b

ACS, cute chest syndrome; IV, intravenous; IVF, intravenous uid; PO, orally; PRN, as needed; TFI, total uid intae.

PITFA Runnin IVF at 153 aintenance a increase te ris of C Routine IVF for CD atients soul be run at 13 aintenance

57

PEAR se incentie siroetr siotera an earl obiliation in all ositalie CD atients to reent C

Hematology 21

 Hydroxyurea H  echanism: increases production o etal b thereby decreasing bS and sicling tendency  enet: decreases reuency o Cs dactylitis ACS blood transusions hospitaliation and death  ide eects: neutropenia bone marrow suppression macrocytosis hepatic enyme elevation anoreia nausea vomiting inertility no proven increased ris o malignancy  onitoring: monthly CBC di¦erential reticulocytes bilirubin AT urea creatinine while titrating dose  C transfusion  cute indications: ACS aplastic crisis splenic seuestration stroe  Chronic indications: splenic seuestration prevention primary and secondary stroe prevention treatment o symptomatic anemia  onitoring: or side e¦ects o repeat transusions Table  a Alloantibody ormation etended crossmatching o pBCs reuired b Iron ecess consider iron chelation therapy a¢er  mg pBCs  transusions or erritin consistently above  ngm Table  Table 15

ureillance uring Chronic Transusion Therap

stem

pecic Inestigations an Freuenc

Heatoloical

Ferritin  onts

Renal

Cr an urea 6 onts

nocrine

H IF1 TH free TT annuall

Heatic

T T T  6 onts Ferriscan annuall to assess lier iron concentration

Cariac

Cariac MRI annuall

Hearin

nnual assessent

taloloical

nnual assessent

Infectious

Heatitis B Heatitis C an HIV serolo annuall

ureillance reuire because of ris of iron loain in te aforeentione orans an ris of infection transission trou bloo Ferritin is a eneral arer of iron loain

528

P laline phosphatase; T alanine aminotransferase; SP aspartate aminotransferase; GGT, gamma-glutamyl transferase; GH, growth hormone; HIV, human immunodeciency syndrome; IG, insulin-lie growth factor ; MRI, magnetic resonance imaging; T, triiodothyronine; T, thyroine; TSH, thyroid stimulating hormone.

Agent Trae Name

Options or Iron Chelation Therap Route

ie Eects

Monitoring

Comments

eeroamine Desferal

ien CIV 5–7 nits er ee oer 1–1 

totoicit retinal canes truncal sortenin

earl earin an ee eaination

Fast for  in before ainistra tion

eerasiro ae aenu

Tablet taen ail

Nerotoicit eatotoicit ototoicit cataracts

Montl renal function T an urinalsis

If transitionin fro ae to aenu te aenu ose soul be  loer tan te ae ose

Neutroenia lier toicit

Montl CBC an T

eeriprone Ferriro

Tablet taen TID

earl earin an ee eaination

Best for cariac eosierosis

T lanine aminotransferase; CBC, complete blood count; IV, intravenous; SC, subcutaneous; TID, three times per day.

C Perioperatie management of CD  atients with SCD are more liely to reuire surgical procedures than the general population especially cholecystectomy tonsillectomy adenoidectomy splenectomy  Increased ris o postoperative complications eg C ACS post operative ever stroe splenic seuestration death  Preoperatie management a ematologist should review  wees beore procedure b Consider correcting anemia and reducing  bS preoperatively via simple transusion c Admit  day preoperatively or CBC reticulocytes etended cross match reuest blood on hold or surgery and postoperatively d nsure adeuate hydration with I or at least  hours preprocedure e reoygenate with  at  min or  minutes preprocedure  Postoperatie management a ptimie respiratory supports and bedside spirometry target   saturation $ b Maintain hydration with I at maintenance rate c rovide adeuate analgesia d ncourage early mobiliation e Avoid hypothermia

Hematology

Table 16

21

THAAEMIA  Pathophysiology: imbalance in aglobin and bglobin chain synthesis un paired globin chains precipitate with hemolysis and ine¦ective erythropoiesis  Classication: historically based on genetic abnormality now also classi ¡ed based on transusion reuirement because o phenotypic variability 59  Clinical features and treatment: see Table 

Features o Thalassemia nromes

Table 17

% Hb enetics Noral ab

HA

HA2

HF

Other

Clinical nrome

96–9

–

,1

—

None

Treatment

bThalassemias

Hematology

b°b°



–5

95

—

Dianose in late infanc g gcain eere aneia al lor failure to trie eatoslenoeal

Reular transfusions Iron celation BMT Fail counselin

b1b1

–

—

6–

—

Moerateseere ane ia beon infanc nisoctosis oiiloctosis

No or irreular transfusions 6 Iron celation Fail counselin

stoatic il aneia

Fail counselin

bb° or bb1

9–95

5–7

–1

h RBC count

Hocroic icroctic sear basoilic stilin aThalassemias

21

Hooous atalasseia – –– –

—

—

—

HbH b Hb Bart g

Hros fetalis aorit stillborn

ntenatal an reular transfusions Iron celation

– 9

BMT Fail counselin

HbH isease – –– a

6–9

–5

–5

HbH – 

Neonatal icroctic aneia Hb 7–1  Hein boies

Transfusion in eoltic or alastic crisis 6 slenecto Folic aci Fail counselin

aTalasseia trait – a – a aa– –

9–9

–

–

—

Hocroic icroctic sear no aneia

Fail counselin

ilent carrier – aaa

9–9

–

–

—

Noral

Fail counselin

°Denotes alleles that mae no globulin chains 1 Denotes alleles that mae reduced globulin chains BMT, one marrow transplant; Hb, hemoglobin; RBC, red blood cell.

530

 ransfusiondependent thalassemia D: reuire regular pBC transusions a euire regular monitoring see Table  and iron chelation therapy see Table   ontransfusiondependent thalassemia D: do not reuire regular pBC transusions may reuire occasional transusions

THROMBOCYTOPENIA Hematology

 Diagnostic approach: see igure  Figure 21. Approach to Thromoctopenia Isolated thrombocytopenia on CBC and differential History & physical examination Blood smear to assess platelet morphology Normal Proceed according to clinical examination findings

Abnormal Differential diagnosis Bernard-Soulier syndrome Wiskott-Aldrich syndrome May-Hegglin anomaly Glanmann thromasthenia Gray platelet syndrome

ymphadenopathy andor splenomegaly

ormal physical examination

ongenital anomalies

Differential diagnosis Malignancy nfection H Storage disease Hypersplenism

Differential diagnosis P iraldrug-induced H ollagen-ascular disease nfant A AP hemangiomas may e isceral Aplastic anemia anconi anemia amilial

Differential diagnosis Skeletal anormalities anconi anemia A syndrome cema Wiskott-Aldrich syndrome Hemangiomas asaachMerritt syndrome yanotic heart disease

Proceed to BMA

21

CBC, Complete blood count HIV human immunode¡ciency syndrome ITP immune thrombocytopenia NAIT neonatal alloimmune thrombocytopenia NATP neonatal autoimmune thrombocytopenia TAR thrombocytopenia and absent radius Modi¡ed rom astings CA ubin B Blood In udolph AM amel  eds Rudo’s Fuds o Pdrs nd ed Norwal CT Appleton  ange – eprinted with permission o ¥e Mcrawill Companies Inc

51

Hematology 21

IMMNE THROMOCTOPENIA ITP  Denition: antibodymediated platelet destruction resulting in isolated thrombocytopenia platelet count , 3   Classication: newly diagnosed – months persistent – months or chronic . months  Pea incidence:  to  years o age  Presentation: minor bleeding such as epistais bruising petechial rash a No history o constitutional symptoms bony or oint pain lymphade nopathy hepatosplenomegaly dysmorphism congenital anomalies developmental or growth delay amily history o hematological malignancy or thrombocytopenia b Approimately hal o cases occur  to  wees ollowing a viral illness  nestigations a CBC and di¦erential normal apart rom low platelet count low b i signi¡cant blood loss b Blood smear platelets o varying sies many large platelets c Bone marrow eamination not recommended in children with typical eatures o IT  reatment a ery low ris o maor bleeding , develop intracranial hemorrhage with high rates o spontaneous remission b Conservative management children with no or mild bleeding eg petechiae can be observed regardless o platelet count c Active management see Table  or options d Management o maor bleeding episodes intravenous immunoglobu lin II 1 highdose I steroids 1 platelet transusion e Management o chronic IT includes thrombopoietin receptor agonists rituimab low dose corticosteroids splenectomy PEAR Treatent ecisions in IT soul be uie b clinical stos an tae into account fail reference Tere is no latelet count tresol for ecision to treat

532

Management Options or Immune Thromoctopenia

rug

ose

enets

ie Eects

IVI

inle ose of –1 

Rai resonse in latelet count

Infusion reaction asetic en initis renal iairent tro boebolic eents eolsis

Corticosterois

renisone   a iie BIDID a 15 a 3  as no taer reuire

No IV treatent reuire no aission reuire

lee isturbance erten sion erlceia

BID, wice per day; ITP, immune thrombocytopenia; IV, intravenous; IVIG, intravenous immunoglobulin; ID, four times per day.

NEONATA AOIMMNE THROMOCTOPENIA NAIT  Pathophysiology: maternal Ig alloantibodies against etal platelets epressing paternal platelet antigens  Most common cause in Caucasians is maternoetal incompatibility o human platelet antigen a Aa  Presentation: wellappearing neonate with petechiaepurpura and isolated usually severe thrombocytopenia platelets ,– 3 at  to  hours o lie a No history o maternal thrombocytopenia  Complications: high ris o prenatal or perinatal intracranial hemorrhage  nestigations a Serological testing demonstrates the presence o maternal antiA Ig antibodies which react with neonatal platelets b Chec maternal CBC or thrombocytopenia consider neonatal autoimmune thrombocytopenia NAT i maternal platelets low c Screening head ultrasound S or intracranial hemorrhage  reatment a educe ris o etal hemorrhage with antenatal II and planned cesarean section delivery b old standard transusion o washed maternal platelets o¢en not practical or easily obtained c Secondbest option transusion o Amatched or Aa negative platelets d andom donor platelets o¢en given as a reasonable and practical alternative e Consider II to improve response to random donor platelets or i severely thrombocytopenic despite matched platelets  Prognosis: resolves spontaneously in  to  months a Need to counsel or uture pregnancies

Hematology

Table 1

21

5

Hematology 21

534

NEONATA ATOIMMNE THROMOCTOPENIA NATP  Pathophysiology: transplacental transer o maternal Ig antiplatelet antibodies resulting in maternal AND neonatal IT  Presentation: neonatal thrombocytopenia with maternal history o IT systemic lupus erythematosus S or autoimmune disease and mater nal thrombocytopenia beore delivery  reatment a Treat maternal IT antenatally with II 6 corticosteroids b Treat neonatal thrombocytopenia with II 6 corticosteroids c Maternal and random donor platelets destroyed i transused  Prognosis: resolves spontaneously in  to  months

NEUTROPENIA  bsolute neutrophil count C: total BC count 3  o segmented neutrophils 1 bands  eutropenia: decrease in ANC below epected range or age and ethnicity  Classication: acute , months or chronic lasting . months  Clinical features a Systemic high ever chills irritability because o inection b Mucocutaneous necrotic and ulcerative lesions o oropharyngeal and nasal mucosa sin gastrointestinal I tract vagina uterus c is o inection inversely proportional to ANC particularly when ANC , 3  d is o inection proportional to the duration o neutropenia  Diagnostic approach a Acute neutropenia see igure  b Chronic neutropenia see igure 

Figure 21. Approach to Acute Neutropenia Acute ANC