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WALKER’SPEDIATRIC
GASTROINTESTINAL DISEASE
PATHOPHYSIOLOGY • DIAGNOSIS •MANAGEMENT
SIXTH EDITION
2
WALKER’SPEDIATRIC
GASTROINTESTINAL DISEASE
PATHOPHYSIOLOGY • DIAGNOSIS •MANAGEMENT
SIXTH EDITION
Ronald E. Kleinman, MDProfessor of Pediatrics
Harvard Medical SchoolMassachusetts General Hospital for Children
Boston, Massachusetts
Olivier-Jean Goulet, MDProfessor of Pediatrics
Head of the Division of Pediatric Gastroenterology-Hepatology-NutritionNational Reference Center for Rare Digestive Disease
Reference Center for Home Parenteral NutritionHôpital Necker Enfants Malades-University Paris Descartes
Paris, France
Giorgina Mieli-Vergani, MDDirector, Pediatric Liver ServiceProfessor in Pediatric Hepatology
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Guy’s, King’s and St. Thomas School of MedicineKing’s College
London, UK
Ian R. Sanderson, MSc, MDProfessor of Paediatric Gastroenterology
Head, Center for GastroenterologyCenter for Digestive Disease
Blizard Institute of Cell & Molecular ScienceLondon, UK
Philip M. Sherman, MDProfessor of Pediatrics, Microbiology and Dentistry
The Hospital for Sick ChildrenUniversity of Toronto
Toronto, Ontario, Canada
Benjamin L. Shneider, MDProfessor of Pediatrics
Director of Pediatric HepatologyChildren’s Hospital of Pittsburgh
Pittsburgh, Pennsylvania
2018PEOPLE’S MEDICAL PUBLISHING HOUSE—USA
RALEIGH, NORTH CAROLINA
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People’s Medical Publishing House-USA5711 Six Forks Road, Suite 210Raleigh, North Carolina 27609Tel: 919-502-4220Fax: 919-502-7673E-mail: [email protected]
© 2018 PMPH-USA, Ltd.
All rights reserved. Without limiting the rights under copyright reserved above, nopart of this publication may be reproduced, stored in or introduced into a retrievalsystem, or transmitted, in any form or by any means (electronic, mechanical,photocopying, recording, or otherwise), without the prior written permission of thepublisher.
18 19 20 21/KING/9 8 7 6 5 4 3 2 1
ISBN-13 978-1-60795-181-0ISBN-10 1-60795-181-9eISBN-13 978-1-60795-083-7
Printed in the United States of America by King Printing Company, Inc.Editor: Linda H. Mehta; Copyeditor/Typesetter: diacriTech; Cover designer:Allison Dibble
Library of Congress Cataloging-in-Publication Data
Names: Kleinman, Ronald E., editor.Title: Walker’s pediatric gastrointestinal disease / Ronald E. Kleinman, Olivier-Jean Goulet, Giorgina Mieli-Vergani, Ian R. Sanderson, Philip M. Sherman, Benjamin L. Shneider.Other titles: Pediatric gastrointestinal diseaseDescription: Sixth edition. | Raleigh, North Carolina : People’s Medical Publishing House-USA, 2018. | Includes bibliographical references and index.Identifiers: LCCN 2018006003 | ISBN 9781607951810 | ISBN 1607951819 |ISBN 9781607950837
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Subjects: | MESH: Gastrointestinal Diseases | Infant | Child | AdolescentClassification: LCC RJ446 | NLM WS 310 | DDC 618.92/33—dc23 LC recordavailable at https://lccn.loc.gov/2018006003
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Notice: The authors and publisher have made every effort to ensure that thepatient care recommended herein, including choice of drugs and drug dosages,is in accord with the accepted standard and practice at the time of publication.However, since research and regulation constantly change clinical standards, thereader is urged to check the product information sheet included in the packageof each drug, which includes recommended doses, warnings, andcontraindications. This is particularly important with new or infrequently useddrugs. Any treatment regimen, particularly one involving medication, involvesinherent risk that must be weighed on a case-by-case basis against the benefitsanticipated. The reader is cautioned that the purpose of this book is to informand enlighten; the information contained herein is not intended as, and shouldnot be employed as, a substitute for individual diagnosis and treatment.
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DEDICATIONS
With deepest gratitude to Allan Walker who has mentored and supportedme for all of my professional life. And to my family, Martha, Emily, Scott,Ellie, Avery, Adam, Monica, Maya and Jesse for their constant love andsupport.
—Ronald E. Kleinman, MD
With my deep appreciation to Professor Allan Walker who invited me tojoin the editorship of this book; to my senior colleagues, Professors JeanRey, Claude Ricour and Jacques Schmitz, who encouraged me to enterpediatric gastroenterology and nutrition; and to my wife, Véronique, andour children, Pierre-Arthur, Charles, Alix and Marine, who have supportedme in my work for so many years.
—Olivier J. Goulet, MD, PhD
To Alex P Mowat, formidable mentor, and to all patients and scientistswho have contributed to our research in juvenile autoimmune liver disease.
—Giorgina Meili-Vergani
To Julia and Vita.
—Ian R. Sanderson, MD
This book is dedicated to trainees—past, current, and future—who makethe journey through life in academic medicine so interesting, meaningful,and rewarding.
—Philip M. Sherman, MD
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To my wife, Abigail, and my daughters, Elizabeth and Caitlin, for theirtremendous love, support, and sacrifices without which this and my otherprofessional accomplishments would not be possible. I would also like toacknowledge my friend, Peter Whitington for his mentorship and in honorof his retirement.
—Benjamin L. Shneider, MD
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CONTENTS
CONTRIBUTORSPREFACE
VOLUME ONE
SECTION I: MOUTH AND ESOPHAGUS
CHAPTER 1 Anatomy, Embryology, Congenital Anomalies of theMouth and EsophagusJonathan E. Teitelbaum
CHAPTER 2 Disorders of the Oral CavityStephen R. Porter and Paul Ashley
CHAPTER 3 Disorders of DeglutitionDavid N. Tuchman
CHAPTER 4 ESOPHAGEAL MOTILITY
CHAPTER 4.1 Normal Motility and Development of theEsophageal Neuroenteric SystemSalvatore Cucchiara, Giovanni Di Nardo and Salvatore Oliva
CHAPTER 4.2 Gastroesophageal RefluxColin D. Rudolph and Eric Hassall
CHAPTER 4.3 Other Esophageal Neuromuscular Disorders
10
Benjamin D. Gold
CHAPTER 5 EsophagitisMike Thomson
CHAPTER 6 Toxic and Traumatic Injury of the EsophagusEmmanuel Mas, Anne Breton, and Jean-Pierre Olives
SECTION II: THE STOMACH AND DUODENUM
CHAPTER 7 Anatomy and Embryology and Congenital AnomaliesSanne L. Rosekrans, Nikè V.J.A. Büller, Nikhil Thapar and Gijs R.van den Brink
CHAPTER 8 Nausea, Vomiting, and Pyloric StenosisB U. K. Li and Katja Kovacic
CHAPTER 9 GASTRITIS
CHAPTER 9.1 Helicobacter pylori and Peptic Ulcer DiseaseMarion Rowland and Billy Bourke
CHAPTER 9.2 Acid-Peptic Disease (Excluding H. pylori)Frédéric Gottrand
CHAPTER 9.3 Other Causes of GastritisKeith Chee Y. Ooi, D.A. Lemberg and Andrew S. Day
CHAPTER 10 Esophageal and Gastric Polyps and NeoplasmsDavid F. Schaeffer, Richard Kirsch, Glenn P. Taylor, and RobertH. Riddell
CHAPTER 11 DEVELOPMENT OF THE GASTRICNEUROENTERIC SYSTEM
CHAPTER 11.1 Normal Motility and Development of the GastricNeuroenteric SystemMiguel Saps and Carlo Di Lorenzo
11
CHAPTER 11.2 Gastric Motility DisordersMiguel Saps and Carlo Di Lorenzo
SECTION III: THE INTESTINE
CHAPTER 12 Anatomy and EmbryologyPascal de Santa Barbara
CHAPTER 13 Congenital Anomalies Including HerniasDavid J. Wilkinson and Simon E. Kenny
CHAPTER 14 Lymphatic DisordersPierre-Yves von der Weid
CHAPTER 15 ABSORPTION AND DIGESTION
CHAPTER 15.1 Normal Physiology of Intestinal Digestion andAbsorptionJean Pierre Cézard
CHAPTER 15.2a Acute DiarrheaStefano Guandalini and Sona Y. Young
CHAPTER 15.2b Persistent and Chronic DiarrheaAlfredo Guarino and Roberto Berni Canani
CHAPTER 15.3a Genetically Determined DisaccharidaseDeficiencyHassan Y. Naim, Mahdi Amiri, and Klaus-Peter Zimmer
CHAPTER 15.3b Congenital Intestinal Transport DefectsMartín G. Martín and Ernest M. Wright
CHAPTER 15.3c Congenital EnteropathiesOlivier Goulet and Julie Salomon
CHAPTER 16 IMMUNE ENTEROPATHIES
CHAPTER 16.1 Celiac Disease
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Alessio Fasano and Carlo Catassi
CHAPTER 16.2a Eosinophilic Gastrointestinal DiseasesCalies Menard-Katcher, Vincent A. Mukkada, Chris A. Liacouras,Glenn T. Furuta, and Marc E. Rothenberg
CHAPTER 16.2b Food Allergic EnteropathyFranco Torrente and Simon Murch
CHAPTER 16.3 Autoimmune Enteropathy and IPEX SyndromeFrank M. Ruemmele, Nicole Brousse, and Olivier Goulet
CHAPTER 17 SURGICAL DISORDERS
CHAPTER 17.1 Intestinal ObstructionsElizabeth Fialkowski and Brad W. Warner
CHAPTER 17.2 AppendicitisKevin Johnson, J. Craig Egan and Dennis P. Lund
CHAPTER 17.3 The Pediatric OstomyCatherine Cord-Udy
CHAPTER 18 Benign Perianal LesionsJeremy Fisher, Biren P. Modi, Sidney Johnson, and Tom Jaksic
CHAPTER 19 MODULATION OF THE INTESTINAL FLORA
CHAPTER 19.1 Microbial Interactions With Gut EpitheliumEytan Wine, Séamus Hussey and Nicola L. Jones
CHAPTER 19.1a ProbioticsDominique Babin-Muise and David R. Mack
CHAPTER 19.1b Prebiotics: Composition and Potential FunctionsDimitrios Tzimorotas, Sofia Kolida, Robert A. Rastall, and Glenn R.Gibson
CHAPTER 19.1c AntimicrobialsMichael R. Millar and Mark Wilks
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CHAPTER 19.2 INFECTIONS
CHAPTER 19.2a Bacterial Infections, Food- and WaterborneInfectionsMitchell B. Cohen
CHAPTER 19.2b Viral InfectionsDorsey M. Bass and Mitchell B. Cohen
CHAPTER 19.2c Parasitic and Fungal InfectionsPaul Kelly and Beatrice Amudi
CHAPTER 19.2d Small-Bowel Bacterial OvergrowthSteven N. Lichtman
CHAPTER 20 IMMUNE AND INFLAMMATORY DISORDERS
CHAPTER 20.1 InflammationChien-Chang Chen, Thomas Blanchard, Claudio Fiocchi, andSteven J. Czinn
CHAPTER 20.2 Gastrointestinal Manifestations ofImmunodeficiency DisordersAmel Hassan and Delane Shingadia
CHAPTER 20.3 Necrotizing EnterocolitisErika C. Claud and Michael S. Caplan
CHAPTER 20.4 CRONIC INFLAMMATORY BOWEL DISEASE
CHAPTER 20.4a Crohn’s DiseaseAnne M. Griffiths and Johan Van Limbergen
CHAPTER 20.4b Ulcerative and Indeterminate Colitis (IncludingInflammatory Bowel Disease Unclassified)Nicholas M. Croft
CHAPTER 20.4c Atypical Colitis and Other Inflammatory DiseasesRanjana Gokhale and Barbara S. Kirschner
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CHAPTER 20.4d Surgical Aspects of Inflammatory Bowel Diseasein ChildrenJacob c. Langer
CHAPTER 20.5 Peritonitis and Intra-abdominal AbscessesDavid J. Hackam and Ori D. Rotstein
CHAPTER 21 INTESTINAL FAILURE
CHAPTER 21.1 Short Bowel SyndromeYigael Finkel, John Puntis, and Olivier Goulet
CHAPTER 21.2 Intestinal Failure-Associated Liver DiseaseJulie E. Bines and Prue M. Pereira-Fantini
CHAPTER 21.3 Intestinal TransplantationStuart S. Kaufman, Cal S. Matsumoto, Eddie R. Island, Nada A.Yazigi, Khalid M. Khan, and Thomas M. Fishbein
CHAPTER 22 INTESTINAL TUMORS
CHAPTER 22.1 Intestinal Polyps and PolyposisJean-François Mougenot, Sylviane Olschwang, and MichelPeuchmaur
CHAPTER 22.2 Other NeoplasmsKamran Badizadegan and Rhea J. Birusingh
CHAPTER 23 INTESTINAL MOTILITY
CHAPTER 23.1 Normal Motility and Development of the IntestinalNeurenteric SystemGabriella Boccia, Massimo Martinelli, and Annamaria Staiano
CHAPTER 23.2 MOTILITY DISORDERS
CHAPTER 23.2a Functional ConstipationRiad Rahhal and Aliye Uc
CHAPTER 23.2b Hirschsprung Disease
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Steven L. Ciciora and Cheryl E. Gariepy
CHAPTER 23.2c Other Dysmotilities Including Chronic IntestinalPseudo-obstruction SyndromeKhoa Tran and Jaime Belkind-Gerson
CHAPTER 23.3 Chronic Abdominal Pain: Including FunctionalAbdominal Pain, Irritable Bowel Syndrome, and Abdominal MigraineJuliette M.T.M. Rutten, Arine M. Vlieger, and Marc A. Benninga
CHAPTER 24 Gastrointestinal Manifestations of EndocrineDiseasesKarolina Maria Burghardt and Yaron Avitzur
CHAPTER 25 BOWEL INJURY
CHAPTER 25.1 Drug-Induced Bowel InjuryReo Tanoshima and Shinya Ito
CHAPTER 25.2 Radiation-Induced Bowel InjuryAgnès François
VOLUME TWO
SECTION IV: THE LIVER
CHAPTER 26 The Liver: Anatomy and EmbryologyMark Davenport
CHAPTER 27 PHYSIOLOGY
CHAPTER 27.1 Bile Formation and CholestasisNitika A. Gupta and Saul J. Karpen
CHAPTER 27.2 Bilirubin MetabolismGlenn R. Gourley
CHAPTER 27.3 Fibrogenesis and CirrhosisRoss W. Shepherd and Grant A. Ramm
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CHAPTER 27.4 Normal Hepatocyte Function and Mechanisms ofDysfunctionEdgar N. Tafaleng, Alicia Watson, and Ira J. Fox
CHAPTER 28 Diagnostic Approaches to Common Pediatric LiverProblemsSara Nóbrega, Sharat Varma, Xavier Stephenne, and Etienne M.Sokal
CHAPTER 29 DISORDERS OF THE BILIARY TRACT
CHAPTER 29.1 Disorders of the Intrahepatic Bile DuctsEkkehard Sturm and Henkjan J. Verkade
CHAPTER 29.2 Biliary AtresiaJorge A. Bezerra
CHAPTER 29.3 Primary Sclerosing CholangitisRuby Mehta and Dennis D. Black
CHAPTER 29.4 Choledochal Cysts and Other Biliary DisordersPhilip Rosenthal
CHAPTER 30 INFECTIONS
CHAPTER 30.1a Hepatitis B VirusMei-Hwei Chang
CHAPTER 30.1b Hepatitis C VirusDaniel H. Leung
CHAPTER 30.1c Other Viral InfectionsJaime Chu and Ronen Arnon
CHAPTER 30.2 Bacterial, Parasitic, and Other InfectionsDinesh S. Pashankar and Richard A. Schreiber
CHAPTER 30.3 Immune Deficiency and the LiverNedim Hadžić and Colin Ball
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CHAPTER 31 Autoimmune Liver DiseaseDiego Vergani and Giorgina Mieli-Vergani
CHAPTER 32 Drug-induced HepatotoxicityEve A. Roberts
CHAPTER 33 Liver TumorsDolores López-Terrada and Milton J. Finegold
CHAPTER 34 GENETIC AND METABOLIC DISORDERS
CHAPTER 34.1 Genetic TestingMatthew C. Dulik, Ian F. Slack, Judith R. Kelsen, and Nancy B.Spinner
CHAPTER 34.2 Disorders of Carbohydrate MetabolismMatthew J. Giefer and Simon P. Horslen
CHAPTER 34.3 Disorders of Amino Acid MetabolismJarrad M. Scarlett and Karen F. Murray
CHAPTER 34.4 Disorders of Mitochondrial and Fatty AcidMetabolismRoshni Vara and Mike Champion
CHAPTER 34.5 α1-Antitrypsin Deficiency
David H. Perlmutter
CHAPTER 34.6 Zellweger Syndrome and Other Disorders ofPeroxisomal MetabolismGerald V. Raymond, Steven J. Steinberg, and Paul A. Watkins
CHAPTER 34.7 Lysosomal Acid Lipase Deficiencies: WolmanDisease/Cholesteryl Ester Storage DiseaseGregory A. Grabowski and Carlos E. Prada
CHAPTER 34.8 Wilson DiseasePiotr Socha and Stuart Tanner
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CHAPTER 34.9 HemochromatosisPeter F. Whitington and Robert E. Fleming
CHAPTER 34.10 Disorders of Biliary TransportLaura Bull and Lee M. Bass
CHAPTER 34.11 Bile Acid Syntesis and MetabolismKenneth D. R. Setchell, James E. Heubi, and Kevin E. Bove
CHAPTER 35 Fatty Liver Disease in ChildrenValentina Giorgio, Anna Alisi, Alberto Villani, and Valerio Nobili
CHAPTER 36 Systemic Conditions Affecting the LiverSamar Ibrahim, Deborah K. Freese, and Mounif El-Youssef
CHAPTER 37 Acute Liver FailureSanjay R. Bansal and Anil Dhawan
CHAPTER 38 End-Stage Liver DiseaseSuzanne V. McDiarmid
CHAPTER 39 Liver TransplantationHector Vilca-Melendez and Nigel D. Heaton
CHAPTER 40 Gallbladder DiseaseAnnemarie Broderick and Brice Antao
SECTION V: THE PANCREAS
CHAPTER 41 Pancreatic TumorsMichael Hii and Assad M. Butt
CHAPTER 42 PANCREATITIS
CHAPTER 42.1 Acute and ChronicDavid Whitcomb and Mark E. Lowe
CHAPTER 42.2 Tropical Calcific PancreatitisCapecomorin S. Pitchumoni and Viswanathan Mohan
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CHAPTER 43 EXOCRINE PANCREATIC DYSFUNCTION
CHAPTER 43.1 Cystic FibrosisMichael Wilschanski
CHAPTER 43.2 Shwachman–Diamond SyndromePeter R. Durie and Michael Wilschanski
CHAPTER 43.3 Other Hereditary and Acquired PancreaticDisordersMichael Wilschanski
SECTION VI: DIAGNOSIS OF GASTROINTESTINALDISORDERS
CHAPTER 44 GASTROINTESTINAL ENDOSCOPY
CHAPTER 44.1 Patient Preparation and General ConsiderationsVictor L. Fox
CHAPTER 44.2a Upper Gastrointestinal EndoscopySimon Murch
CHAPTER 44.2b Upper Gastrointestinal BleedingMark A. Gilger and Kristin Whitfield Van Buren
CHAPTER 44.3a Ileocolonoscopy and EnteroscopyMike Thomson
CHAPTER 44.3b Lower Gastrointestinal BleedingDominique Turck and Laurent Michaud
CHAPTER 44.4 Gastrointestinal EndosonographyVictor L. Fox
CHAPTER 44.5 Endoscopic RetrogradeCholangiopancreatographyClaude Liguory, Jean-François Mougenot, Gustavo Andrade dePaulo, and Douglas S. Fishman
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CHAPTER 44.6 Interventional Endoscopy: Recent InnovationsNadeem Ahmad Afzal and Mike Thomson
CHAPTER 45 Liver Biopsy InterpretationAlex S. Knisely
CHAPTER 46 Intestinal BiopsyAlan David Phillips, Olivier Goulet, and Virpi V. Smith
CHAPTER 47 Gastrointestinal Manometry: Methodology andIndicationsSamuel Nurko
CHAPTER 48 Esophageal pH and Impedance MeasurementYvan Vandenplas and Silvia Salvatore
CHAPTER 49 IMAGING
CHAPTER 49.1 Imaging of Pediatric Gastrointestinal DiseaseRahul A. Sheth, Debra A. Gervais, and Michael S. Gee
CHAPTER 49.2 Cross-sectional Imaging of Abdominal Masses:Liver, Pancreas, and SpleenKeith B. Quencer, Debra A. Gervais, and Michael S. Gee
CHAPTER 49.3 Interventional Gastrointestinal RadiographyAmy R. Deipolyi, Michael S. Gee and Debra A. Gervais
CHAPTER 49.4 Radionuclide ScintigraphyReza Vali and Martin Charron
SECTION VII: PSYCHOSOCIAL ASPECTS OFGASTROINTESTINAL DISORDERS
CHAPTER 50 Pediatric Illness Falsification: GastrointestinalFeaturesBrenda Bursch and Paul Hyman
CHAPTER 51 Psychological Aspects of Chronic Disease
21
Steven C. Schlozman and Suzanne L. Bender
CHAPTER 52 Adherence to Medical RegimensEyal Shemesh
SECTION VIII: PRINCIPLES OF CLINICAL INVESTIGATION
CHAPTER 53 Outcomes Research in Pediatric GastroenterologyMaireade E. McSweeney and Jenifer R. Lightdale
CHAPTER 54 The Statistics and Interpretation of Screening andDiagnostic TestsPatricia L. Hibberd and Andrew B. Cooper
CHAPTER 55 Ethics and Regulatory IssuesP. Pearl O’Rourke and Jennifer P. Stevens
INDEX
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CONTRIBUTORS
Mahdi Amiri, PhDDepartment of GastroenterologyHannover Medical SchoolHannover, GermanyGenetically Determined Disaccharides Deficiency (15.3a)
Beatrice C. Amudi, MDDepartment of Pediatrics and Child HealthUniversity of Zambia School of MedicineUniversity Teaching HospitalLusaka, ZambiaParasitic and Fungal Infections (19.2c)
Brice Antao, MD, MBBSConsultant Paediatric SurgeonBeacon Hospital for KidsGoa UniversityCounty Dublin, IrelandGallbladder Disease (40)
Ronen Arnon, MDProfessor, Ichan School of MedicinePediatric Hepatology and Liver TransplantationIcahn School of Medicine at Mount SinaiNew York, New York
23
Other Viral Infections (30.1c)
Paul Ashley, MDPediatric DentistryUCL Eastman Dental Institute for Oral Health Care SciencesLondon, EnglandDisorders of the Oral Cavity (2)
Yaron Avitzur, MDAssociate Professor of Pediatrics, University of TorontoMedical Director, Intestinal Rehabilitation and TransplantationDivision of Gastroenterology, Hepatology and NutritionDirector, Pediatric Gastroenterology Training ProgramThe Hospital for Sick ChildrenToronto, Ontario, CanadaGastrointestinal Aspects of Systemic Endocrinopathies (24)
Dominique Babin-Muise, MDHarbour South Medical ClinicYarmouth, Nova ScotiaProbiotics (19.1a)
Kamran Badizadegan, MDProfessor, Department of PathologyOhio State University Wexner Medical CenterChief of Pathology and Laboratory MedicineNationwide Children’s HospitalColumbus, OhioOther Neoplasms (22.2)
Colin Ball, MDConsultant PaediatricianClinical Director General PaediatricsDepartment of PaediatricsKing’s College HospitalLondon, EnglandImmune Deficiency and the Liver (30.3)
24
Sanjay K. Bansal, MDAssistant Professor, Pediatric EndocrinologyTufts University School of MedicineBoston, MassachusettsAcute Liver Failure (37)
Dorsey M. Bass, MDAssociate Professor, Pediatric GastroenterologyThe Lucille Salter Packard Children’s HospitalStanford School of MedicinePalo Alto, CaliforniaViral Infections (19.2b)
Lee M. Bass, MDAssociate Professor of PediatricsDepartment of Pediatrics, Gastroenterology, Hepatology and NutritionNorthwestern University Feinberg School of MedicineChicago, IllinoisDisorders of Biliary Transport (34.10)
Jaime Belkind-Gerson, MDDigestive Health InstituteChildren’s Hospital of ColoradoColorado University School of MedicineAurora, ColoradoOther Dysmotilities Including Chronic Intestinal Pseudo-obstruction
Syndrome (23.2c)
Suzanne L. Bender, MDAssistant Professor of Psychiatry (part time)Harvard Medical SchoolSenior Consulting Child Psychiatrist, Pediatric Gastroenterology and Child
Nutrition UnitMassachusetts General Hospital for ChildrenBoston, MassachusettsPsychological Aspects of Chronic Disease (51)
25
Marc A. Benninga, MD, PhDProfessor of PediatricsUniversity of Amsterdam Faculty of MedicineDepartment of Pediatric GastroenterologyEmma Children’s HospitalAmsterdam, NetherlandsChronic Abdominal Pain Including Functional Abdominal Pain, Irritable
Bowel Syndrome, and Abdominal Migraine (23.3)
Jorge A. Bezerra, MDProfessor, University of Cincinnati Department of PediatricsDirector, Division of Gastroenterology, Hepatology and NutritionMedical Director, Pediatric Liver Care CenterCincinnati Children’s Hospital Medical CenterCincinnati, OhioBiliary Atresia (29.2)
Julie E. Bines, MDVictor and Loti Smorgon Professor of PediatricsDeputy Head, Department of PediatricsUniversity of MelbourneDirector Clinical Nutrition ProgramRoyal Children’s HospitalIntestinal Failure and Clinical Nutrition Research GroupMurdoch Children’s Research InstituteVictoria, AustraliaIntestinal Failure: Associated Liver Disease (21.2)
Rhea J. Birusingh, MDDirector of Anatomical PathologyNemours Children’s HospitalAssistant Professor of PathologyUniversity of Central Florida College of MedicineOrlando, FloridaOther Neoplasms (22.2)
Dennis D. Black, MD
26
J. D. Buckman Professor of PediatricsProfessor of PhysiologyDirector, Children’s Foundation Research InstituteVice President for Research, Le Bonheur Children’s HospitalThe University of Tennessee Health Science CenterMemphis, TennesseeSclerosing Cholangitis (29.3)
Thomas G. Blanchard, PhD, JDAssociate Professor, PediatricsUniversity of Maryland School of MedicineBaltimore, MarylandInflammation (20.1)
Gabriella Boccia, MDAssistant Professor, Department of PediatricsUniversity of Naples Federico IINaples, ItalyNormal Motility and Development of the Intestinal Neuroenteric System
(23.1)
Billy Bourke, MD, FRCPIAssociate Professor, Pediatric GastroenterologyUCD Conway Institute of Biomolecular and Biomedical ResearchOur Lady’s Children’s Hospital, CrumlinDublin, IrelandHelicobacter Pylori and Peptic Ulcer Disease (9.1)
Kevin E. Bove, MDProfessor, Department of PediatricsUniversity of CincinnatiCincinnati Children’s Hospital Medical CenterCincinnati, OhioBile Acid Synthesis and Metabolism (34.11)
Anne Breton, MDDivision of Gastroenterology, Hepatology, and Nutrition
27
Department of PediatricsChildren’s Hospital of the University of ToulouseToulouse, FranceTraumatic and Toxic Injury of the Esophagus (6)
Anne Marie Broderick, MDClinical Associate Professor, Pediatric GastroenterologyUniversity College of Dublin School of MedicineOur Lady’s Children’s Hospital, CrumlinDublin, IrelandGallbladder Disease (40)
Nicole Brousse, MD, PhDProfessor of PathologyHopital Necker-Enfants MaladesParis Descartes UniversityParis, FranceAutoimmune Enteropathy and IPEX Syndrome (16.3)
Laura Bull, PhDProfessor of Medicine, Department of Medicine/GastroenterologyUCSF Helen Diller Comprehensive Cancer CenterUniversity of California School of MedicineSan Francisco, CaliforniaDisorders of Biliary Transport (34.10)
Nikè V. J. A. Büller, PhDTytgat Institute for Liver and Intestinal ResearchDepartment of Gastroenterology and HepatologyAcademic Medical CenterAmsterdam, The NetherlandsAnatomy and Embryology and Congenital Anomalies (7)
Brenda Bursch, PhDProfessor, Psychiatry and Biobehavioral Sciences and PediatricsClinical Director, Pediatric Consultation and Liaison ServiceDavid Geffen School of Medicine at University of California
28
Los Angeles, CaliforniaGastrointestinal Features of Pediatric Illness Falsification (50)
Karolina Maria Burkhardt, MD, PhDAdjunct Assistant Professor, Department of PediatricsQueens UniversityBrockville General Hospital, University of TorontoPediatric Associate, Children’s Hospital of Eastern OntarioToronto, Ontario, CanadaGastrointestinal Aspects of Systemic Endocrinopathies (24)
Assad M. Butt, MD, RCPCH, BSPGHANDepartment of Pediatric GastroenterologyRoyal Alexandra Children’s Hospital,Brighton and Sussex University HospitalsBrighton, United KingdomPancreatic Tumors (41)
Roberto Berni Canani, MD, PhDAssociate Professor of PediatricsDepartment of Translational Medical ScienceEuropean Laboratory for the Investigation of Food-Induced DiseasesUniversity of Naples Federico IINaples, ItalyPersistent and Chronic Diarrhea (15.2b)
Michael S. Caplan, MDClinical Professor of PediatricsUniversity of Chicago, Pritzker School of MedicineChairman, Department of PediatricsChief Scientific Officer, Research InstituteNorthshore University Health SystemEvanston, IllinoisNecrotizing Enterocolitis (20.3)
Carlo Catassi, MDVisiting Scientist
29
Center for Celiac Research and Mucosal ImmunologyBiology Research CenterMassachusetts General Hospital for ChildrenHarvard Medical SchoolBoston, MassachusettsDepartment of PediatricsUniversità Politecnica delle MarcheAncona, ItalyCeliac Disease (16.1)
Jean Pierre Cézard, MD, PhDDepartment of Pediatric GastroenterologyHôpital Universitaire Robert DebréParis, FranceNormal Physiology of Intestinal Digestion and Absorption (15.1)
Mei-Wei Chang, PhD, RNAssociate Professor, College of NursingMichigan State UniversityEast Lansing, MichiganHepatitis B Virus (30.1a)
Martin Charron, MD, FRCP(C)Professor, Department of RadiologyUniversity of TorontoDivision Head of Nuclear MedicineHead of Research for Diagnostic ImagingSenior Associate Scientist, Research InstituteThe Hospital for Sick ChildrenToronto, Ontario, CanadaRadionucleotide Scintigraphy (49.4)
Chien-Chang Chen, MDDivision of GastroenterologyDepartment of PediatricsChang Gung Memorial HospitalTaoyuan, Taiwan
30
Inflammation (20.1)
Jaime Chu, MDAssistant Professor, Pediatrics and HepatologyIcahn School of Medicine at Mount SinaiPediatric Hepatology and Liver TransplantationMt. Sinai HospitalNew York, New YorkOther Viral Infections (30.1c)
Steven L. Ciciora, MDAssistant Professor of PediatricsThe Ohio State University College of MedicineDivision of Gastroenterology, Hepatology and NutritionNationwide Children’s HospitalColumbus, OhioHirschsprung Disease (23.2b)
Erika C. Claud, MDAssociate ProfessorDepartments of Pediatrics and MedicineSections of Neonatology and GastroenterologyThe University of Chicago School of MedicineChicago, IllinoisNecrotizing Enterocolitis (20.3)
Mitchell B. Cohen, MDChair, Department of PediatricsUniversity of Alabama at Birmingham School of MedicinePhysician-in-Chief and DirectorGastroeneterology, Hepatology and NutritionChildren’s of AlabamaBirmingham, AlabamaBacterial Infections, Food- and Waterborne Infections (19.2a)Viral Infections (19.2b)
Andrew B. Cooper, PhD
31
School of Resource and Environmental ManagementSimon Fraser UniversityBurnaby, British Columbia, CanadaThe Statistics and Interpretation of Screening and Diagnostic Tests (54)
Catherine Cord-Udy, MBBS, FRACSPediatric SurgeonWomen’s and Children’s HospitalNorth Adelaide, AustraliaThe Pediatric Ostomy (17.3)
Nicholas M. Croft, MBBS, PhDReader and Consultant Pediatric GastroenterologistCentre for Digestive DiseasesBlizard InstituteBarts and The London School of Medicine and DentistryQueen Mary University of LondonLondon, United KingdomUlcerative and Indeterminate Colitis (20.4b)
Salvatore Cucchiara, PhDDepartment of PediatricsPediatric Gastroenterology and Liver UnitSapienza University of RomeRome, ItalyNormal Motility and Development of the Esophageal Neuroenteric System
(4.1)
Steven J. Czinn, MDDrs. Rouben and Violet Jiji Professor of PediatricsChair, Department of PediatricsDirector of the University of Maryland Children’s HospitalUniversity of Maryland School of MedicineBaltimore, MarylandInflammation (20.1)
Mark Davenport, MD
32
Professor and Head, Department of Pediatric SurgeryKing’s College HospitalLondon, United KingdomThe Liver: Anatomy and Embryology (26)
Andrew S. Day, MDPediatric GastroenterologistHead, Department of PediatricsUniversity of Otago, ChristchurchDunedin, New ZealandOther Causes of Gastritis (9.3)
Gustavo Andrade de Paulo, MD, PhDEndoscopy Unit, Gastroenterology DepartmentCancer Institute of São PauloUniversidade Federal de São PauloSão Paulo, BrazilEndoscopic Retrograde Cholangiopancreatography (44.5)
Pascal de Santa Barbara, PhDResearch ScientistTeam Leader, Physiology and Experimental Biology of Heart and MusclesUniversity of MontpellierMontpellier, FranceAnatomy and Embryology (12)
Anil Dhawan, MBBSProfessor of Pediatric HepatologyDirector, Pediatric Liver, Gastroenterology, and Nutrition CenterClinical Director of Child HealthKing’s College HospitalLondon, United KingdomAcute Liver Failure (37)
Carlo Di Lorenzo. MDProfessor, Clinical PediatricsOhio State University College of Medicine
33
Division Chief, Gastroenterology, Hepatology and NutritionMedical Director, Center for Colorectal and Pelvic ReconstructionNationwide Children’s HospitalColumbus, OhioNormal Motility and Development of the Gastric Neuroenteric System
(11.1)Gastric Motility Disorders (11.2)
Giovanni Di Nardo, MDPediatric GastroenterologyDepartment of Medical and Surgical SciencesSantobono-Pausilipon Children’s HospitalNaples, ItalyCh. 4.1: Normal Motility and Development of the Esophageal
Neuroenteric System
Matthew C. Dulik, PhDDirector, Division of Genomic DiagnosticsThe Children’s Hospital of PhiladelphiaPhiladelphia, PennsylvaniaGenetic Testing in Clinical Practice (54.1)
Peter R. Durie, MD, FRCP(C)Professor of PediatricsUniversity of TorontoSenior Scientist, Program in Physiology and Experimental MedicineThe Research InstituteDepartment of Gastroenterology, Hepatology and NutritionThe Hospital for Sick ChildrenToronto, CanadaShwachman-Diamond Syndrome (43.2)
J. Craig Egan, MDClinical Assistant Professor of SurgeryUniversity of Arizona College of MedicineInstructor of Surgery, Mayo ClinicSurgical Director, Pediatric Intensive Care Unit
34
Phoenix Children’s HospitalPhoenix, ArizonaAppendicitis (17.2)
Mounif El-Youssef, MDPediatric GastroenterologyDirector of Pediatric Liver TransplantsMayo Clinic RochesterRochester, MinnesotaSystemic Conditions Affecting the Liver (36)
Alessio Fasano, MDW. Allan Walker Chair in Pediatric Gastroenterology and NutritionDirector, Center for Celiac Research and TreatmentDirector, Mucosal Immunology and Biology Research CenterAssociate Chief for Basic, Clinical, and Translational ResearchMassachusetts General HospitalBoston, MassachusettsCeliac Disease (16.1)
Elizabeth Fialkowski, MDAssistant Professor of SurgeryDivision of Pediatric SurgeryOregon Health and Science UniversityPortland, OregonIntestinal Obstructions (17.1)
Milton J. Finegold, MDProfessor, Pathology and Immunology and Pediatrics,Baylor College of MedicineChief Emeritus, Pathology Service, Texas Children’s HospitalPathologist Reviewer, Children’s Oncology GroupHouston, TexasLiver Tumors (33)
Yigael Finkel, MD, PhDAdjunct Professor, Pediatrics and Gastroenterology
35
Department of Clinical Science and EducationKarolinska Institutet, SolnaStockholm, SwedenShort-Bowel Syndrome (21.1)
Claudio Fiocchi, MDProfessor, Clinical and Translational Science CollaborativeMolecular Medicine, Case Western Reserve UniversityDepartment of PathobiologyCleveland Clinic Lerner Research InstituteCleveland, OhioInflammation (20.1)
Jeremy Fisher, DOHenry Ford Macomb HospitalHenry Ford HospitalClinton Township, MichiganBenign Perianal Lesions (18)
Thomas M. Fishbein, MDExecutive director, MedStar Georgetown Transplant InstituteDirector of Liver Diseases and Transplant ProgramProfessor, Departments of Surgery and PediatricsMedStar Georgetown Transplant InstituteWashington, DCIntestinal Transplantation (21.3)
Douglas S Fishman, MDAssociate Professor of Pediatrics, Baylor College of MedicineDirector, Gastrointestinal Endoscopy and Therapeutic EndoscopyMedical Director, Pancreaticobilary ProgramTexas Children’s HospitalHouston, TexasEndoscopic Retrograde Cholangiopancreatography (44.5)
Robert E. Fleming, MDDoisy Department of Biochemistry & Molecular Biology
36
Saint Louis University School of MedicineCardinal Glennon Children’s HospitalSt. Louis, MissouriHemochromatosis (34.9)
Ira H. Fox, MDProfessor of Surgery, University of Pittsburgh School of MedicineDirector of Center for Innovative Regenerative TherapiesChildren’s Hospital of Pittsburgh of UPMCMedical Director, McGowan Institute for Regenerative MedicinePittsburgh, PennsylvaniaNormal Hepatocyte Function and Mechanisms of Dysfunction (27.4)
Victor L. Fox, MDAssociate Professor of Pediatrics, Harvard Medical SchoolDirector, Gastrointestinal EndoscopyDirector, Pediatric Polyposis ProgramMedical Director, Gastroenterology Procedure Unit;Co-Director, Pancreatic Disorders ProgramBoston Children’s HospitalBoston, MassachusettsPatient Preparation and General Considerations (44.1)Gastrointestinal Endosonography (44.4)
Agnès François, PhDDepartment of Cell Biology and GeneticsInstitute for Integrative Biology of the CellUniversity of ParisParis, FranceRadiation-Induced Bowel Injury (25.2)
Deborah K. Freese, MDAssociate Professor of PediatricsPediatric GastroenterologyMayo ClinicRochester, MinnesotaSystemic Conditions Affecting the Liver (36)
37
Glenn T. Furuta, MDProfessor of Pediatrics, Colorado University School of MedicineLa Cache Endowed Chair for Gastroenterology, Allergic and Immunologic
DiseasesDirector, Gastrointestinal Eosinophilic Diseases ProgramChildren’s Hospital ColoradoAurora, ColoradoEosinophilic Gastrointestinal Disease (16.2a)
Cheryl Gariepy, MDAssociate Professor, Gastroenterology, Hepatology and NutritionAssociate Director for Research of the Pediatric Gastroenterology
FellowshipNationwide Children’s HospitalOhio State University College of MedicineColumbus, OhioHirschsprung Disease (23.2b)
Michael S. Gee, MD, PhDAssociate Professor of Radiology,Harvard Clinical and Translational Science CenterChief, Pediatric RadiologyMassachusetts General Hospital for ChildrenBoston, MassachusettsImaging of Gastrointestinal Tract Disease (49.1)Cross-Sectional Imaging of Abdominal Masses (49.2)Interventional Gastrointestinal Radiography (49.3)
Debra A. Gervais, MDDirector, Division of Pediatric RadiologyMassachusetts General Hospital for ChildrenBoston, MassachusettsImaging of Gastrointestinal Tract Disease (49.1)Cross-Sectional Imaging of Abdominal Masses (49.2)Interventional Gastrointestinal Radiography (40.3)
Glenn R. Gibson, PhD
38
Professor and Head, Food and Microbial Sciences Research UnitUniversity of ReadingReading, United KingdomResearch Scientist at MRC Dunn Clinical Nutrition CenterCambridge, United KingdomPrebiotics: Composition and Potential Functions (19.1b)
Mark A. Gilger, MDChief, Gastroenterology, Hepatology and NutritionChief, Gastrointestinal InstituteTexas Children’s HospitalHouston, TexasUpper Gastrointestinal Bleeding (44.2b)
Ranjana Gokhale, MDAssociate Professor of PediatricsThe University of Chicago MedicineDirector, Pediatric Gastroenterology Fellowship ProgramSection of Pediatric Gastroenterology, Hepatology and NutritionChicago, IllinoisAtypical Colitis and Other Inflammatory Diseases (20.4c)
Benjamin D. Gold, MDProfessor of Pediatrics and MicrobiologyChief of Gastroenterology ServiceChildren’s Center for Digestive Healthcare, LLCChair Emeritus, Division of Pediatric Gastroenterology, Hepatology and
NutritionEmory UniversityAtlanta, GeorgiaOther Esophageal Neuromuscular Disorders (4.3)
Frédéric Gottrand, MD, PhDProfessor of PediatricsDivision of Gastroenterology, Hepatology and NutritionJeanne de Flandre HosptialLille University
39
Lille, FranceAcid-Peptic Disease (9.2)
Olivier Goulet, MD, PhDProfessor of PediatricsDepartment Pediatric Gastroenterology, Hepatology and NutritionNational Reference Center for Rare Digestive DiseasesNecker-Enfants Malades HospitalParis, FranceCongenital Enteropathies (15.3c)Autoimmune Enteropathy and IPEX Syndrome (16.3)Short Bowel Syndrome (21.1)
Glenn R. Gourley, MD, AGAFProfessor of PediatricsResearch Director, Division of Pediatric GastroenterologyProgram Director, Pediatric Gastroenterology Fellowship Training
ProgramUniversity of MinnesotaMinneapolis, MinnesotaBilirubin Metabolism (27.2)
Gregory A. Grabowski, MDProfessor of Pediatrics and Molecular Genetics, Biochemistry and
MicrobiologyCincinnati Children’s Hospital Medical CenterCincinnati, OhioLysosomal Acid Lipase Deficiencies (34.7)
Anne M. Griffiths, MD, FRCPProfessor of PediatricsDivision of Gastroenterology, Hepatology and NutritionSenior Associate Scientist, Child Health Evaluative SciencesMember, Research Ethics BoardThe Hospital for Sick ChildrenUniversity of TorontoToronto, Ontario, Canada
40
Crohn’s Disease (20.4a)
Stefano Guandalini, MDProfessor of PediatricsChief, Pediatric Gastroenterology, Hepatology, and NutritionFounder and Medical Director, University of Chicago Celiac Disease
CenterUniversity of ChicagoChicago, IllinoisAcute Diarrhea (15.2a)
Alfredo Guarino, MDProfessor of PediatricsDepartment of Translational Medical ScienceUniversity of Naples Federico IINaples, ItalyPersistent and Chronic Diarrhea (15.2b)
David J. Hackam, MD, PhDProfessor of Pediatric SurgerySurgeon-in-Chief, Hackam Lab for Pediatric SurgeryTranslational and Regenerative MedicineJohns Hopkins Children’s CenterBaltimore, MarylandPeritonitis and Intra-abdominal Abscesses (20.5)
Nedim Hadžić, MD, PhDProfessor of Pediatric HepatologyGuthrie ClinicKing’s College HospitalLondon, United KingdomImmune Deficiency and the Liver (30.3)
Eric Hassall, MBChBCalifornia Pacific Medical Center,San FranciscoEmeritus Professor of Pediatrics
41
University of British ColumbiaVancouver, BC, CanadaGastroesophageal Reflux (4.2)
Amel Hassan, MDGreat Ormond Street Hospital for ChildrenNHS Foundation TrustLondon, United KingdomGastrointestinal Manifestations of Immunodeficiency Disorders (20.2)
Patricia L. Hibberd, MD, PhDProfessor of Global HealthProfessor of Medicine in Infectious DiseasesBoston University School of Public HealthChief, Division of Global HealthMassachusetts General HospitalBoston, MassachusettsThe Statistics and Interpretation of Screening and Diagnostic Tests (54)
Michael Hii, MBBSClinical Lecturer, University of MelbourneConsultant Surgeon, St Vincent’s Public Hospital and the Royal
Melbourne HospitalUpper Gastrointestinal and Bariatric SurgeonParkville, Victoria, AustraliaPancreatic Tumors (41)
James Heubi, MDProfessor of PediatricsAssociate Dean, Clinical and Translational ResearchDirector, Center for Clinical and Translational Science and TrainingUniversity of Cincinnati College of MedicineDirector, Division of Gastroenterology, Hepatology and Nutrition,Cincinnati Children’s Hospital Medical CenterCincinnati, OhioBile Acid Synthesis and Metabolism (34.11)
42
Simon Horslen, MB ChBProfessor and Medical Director, Solid Organ TransplantationDivision of Gastroenterology, Hepatology, and NutritionDirector of the Hepatobiliary and Intestinal Failure ProgramsSeattle Children’s HospitalSeattle, WashingtonDisorders of Carbohydrate Metabolism (34.2)
Séamus Hussey, MB, FRCPIConsultant, Department of Paediatric GastroenterologyOur Lady’s Children’s HospitalUniversity College DublinDublin, IrelandCh. 19.1: Microbial Interactions with Gut Epithelium (19.1)
Paul E. Hyman, MDPediatric Gastroenterology, Hepatology, and NutritionChildren’s Hospital of New OrleansLouisiana State University Health Sciences CenterNew Orleans, LouisianaGastrointestinal Features of Pediatric Illness Falsification (50)
Samar Ibrahim, MB, ChBAssistant Professor of PediatricsConsultant, Division of Pediatric Gastroenterology and HepatologyDepartment of Pediatric and Adolescent MedicineConsultant, Division of Gastroenterology and HepatologyDepartment of Internal MedicineMayo ClinicRochester, MinnesotaSystemic Conditions Affecting the Liver (36)
Eddie R. Island, MDAssociate Director, Pediatric TransplantationMedStar Georgetown Transplant InstituteWashington, DCIntestinal Transplantation (20.4)
43
Shinya Ito, MD, FRCP©Professor, Medicine, Pharmacology and Pharmacy,Department of Pediatrics, University of TorontoSenior Scientist, Translational MedicineResearch InstituteDivision Head, Clinical Pharmacology and ToxicologyThe Hospital for Sick ChildrenToronto, Ontario, CanadaDrug-Induced Bowel Injury (25.1)
Tom Jaksic, MD, PhDW. Hardy Hendren Professor of SurgeryHarvard Medical SchoolVice-Chairman, Pediatric General Surgery,Boston Children’s HospitalSurgical Director of the Center for Advanced Intestinal RehabilitationBoston, MassachusettsBenign Perianal Lesions (18)
Kevin N. Johnson, MDSection of Pediatric SurgeryMott Children’s HospitalUniversity of MichiganAnn Arbor, MichiganAppendicitis (17.2)
Sidney Johnson, MDPediatric SurgeryKasier Permenente Moanalua Medical CenterKapiolani Medical Center for Women and ChildrenHonolulu, HawaiiBenign Perianal Lesions (18)
Nicola L. Jones, MD, PhDProfessor and Senior ScientistDepartments of Paediatrics and PhysiologyCell Biology Programme
44
Research InstituteThe Hospital for Sick ChildrenToronto, Ontario, CanadaMicrobial Interactions with Gut Epithelium (19.1)
Khalid M. Khan, MDPediatric GastroenterologyMedStar Georgetown Transplant InstituteWashington, DCIntestinal Transplantation (21.3)
Saul J. Karpen, MD, PhDRaymond F. Schinazi Distinguished Biomedical ChairProfessor of PediatricsDivision Chief, Pediatric Gastroenterology, Hepatology & NutritionEmory University School of MedicineChildren’s Healthcare of AtlantaAtlanta, GeorgiaBile Formation and Cholestasis (27.1)
Stuart S. Kaufman, MDMedical Director, Pediatric TransplantationMedStar Georgetown University HospitalWashington, DCIntestinal Transplantation (21.3)
Paul Kelly, MDProfessor of Tropical GastroenterologyCenter for Immunobiology, Blizard InstituteBarts and the London School of Medicine and DentistryQueen Mary, University of LondonLondon, United KingdomParasitic and Fungal Infections (19.2c)
Judith R. Kelsen, MDAssistant Professor of PediatricsProgram Director of the Very Early Onset Inflammatory Bowel Disease
45
(VEO-IBD) ClinicDivision of Gastroenterology, Hepatology and NutritionChildren’s Hospital of PhiladelphiaPhiladelphia, PennsylvaniaGenetic Testing (34.1)
Simon Edward Kenny, MDHonorary Senior Lecturer in Child Health,University of LiverpoolDepartment of HealthConsultant Pediatric Surgery and Urology, NHSLiverpool, United KingdomCongenital Anomalies Including Hernias (13)
Richard Kirsch, MBChB, PhDAssociate ProfessorDepartment of Pathobiology and Laboratory MedicineMount Sinai Hospital/Research InstituteUniversity of TorontoToronto, Ontario, CanadaEsophageal and Gastric Polyps and Neoplasms (10)
Barbara S. Kirschner, MDProfessor Emerita of Pediatrics and MedicineThe University of Chicago MedicineCo-founder of PEDi-IBD Collaborative Research GroupChicago, IllinoisAtypical Colitis and Other Inflammatory Diseases (20.4c)
Alex S. Knisely, MDProfessor of PathologyInstitute of Liver StudiesKing’s College HospitalLondon, United KingdomLiver Biopsy Interpretation (45)
Sofia Kolida, PhD
46
Department of Food and Nutritional SciencesUniversity of ReadingWhiteknights, Reading, United KingdomPrebiotics: Composition and Potential Functions (19.1b)
Katja Kovacic, MDAssistant Professor of PediatricsCenter for Pediatric Neurogastroenterology, Motility and Autonomic
DisordersChildren’s Hospital of WisconsinMedical College of WisconsinMilwaukee, WisconsinNausea, Vomiting, and Pyloric Stenosis (8)
Jacob C. Langer, MDProfessor of Surgery, University of TorontoHead, Division of General and Thoracic SurgeryHospital for Sick ChildrenToronto, Ontario, CanadaSurgical Aspects of Inflammatory Bowel Disease in Children (20.4d)
Daniel A. Lemberg, MDAssociate Professor, School of Women and Children’s HealthUniversity of New South WalesHead of Pediatric GastroenterologyDirector, Children’s Inflammatory Bowel Diseases ClinicSydney Children’s HospitalSydney, AustraliaOther Causes of Gastritis (9.3)
Daniel H. Leung, MDAssociate Professor of Pediatrics, Baylor College of MedicineDirector, Clinical Research, Gastroenterology, Hepatology, and NutritionDirector, Viral Hepatitis ProgramTexas Children’s HospitalHouston, TexasHepatitis C Virus (30.1b)
47
B U.K. Li, MDProfessor, Pediatrics and GastroenterologyDirector, Cyclic Vomiting ProgramMedical College of WisconsinChildren’s Hospital of WisconsinMilwaukee, WisconsinNausea, Vomiting, and Pyloric Stenosis (8)
Chris A. Liacouras, MDProfessor of Pediatrics, Perelman School of Medicine,University of PennsylvaniaCo-director, Center for Pediatric Eosinophilic DisordersDivision of Gastroenterology, Hepatology and NutritionThe Children’s Hospital of PhiladelphiaPhiladelphia, PennsylvaniaEosinophilic Gastrointestinal Diseases (16.2a)
Steven N. Lichtman, MD, EdDProfessor, Division of Pediatric GastroenterologyUNC Hospitals Children’s Specialty ClinicChapel Hill, North CarolinaSmall-Bowel Bacterial Overgrowth (19.2d)
Jenifer R. Lightdale, MD, MPHProfessor of PediatricsUniversity of Massachusetts Medical SchoolDirector, Patient Safety and QualityDivision of Gastroenterology and NutritionBoston Children’s HospitalBoston, MassachusettsOutcomes Research in Pediatric Gastroenterology (53)
Claude Liguory, MDRetired as Director, Endoscopy UnitAmerican Hospital of ParisParis, FranceEndoscopic Retrograde Cholangiopancreatography (44.5)
48
Johan Van Limbergen, MD, PhDInflammatory Bowel Diseases CentreDivision of Pediatric GastroenterologyIWK Health CentreDalhousie UniversityHalifax, Nova Scotia, CanadaCrohn’s Disease (20.4a)
Mark E. Lowe, MD, PhDProfessor of PediatricsChief, Division of Gastroenterology, Hepatology and NutritionProfessor of Molecular Genetics and BiochemistryChildren’s Hospital of PittsburghUniversity of Pittsburgh and UMPCPittsburgh, PennsylvaniaPancreatitis Acute and Chronic (42.1)
Dennis P. Lund, MDProfessor of Surgery (Pediatric Surgery)Associate Dean of Faculty of Pediatrics and ObstetricsChief Medical Officer, Lucile Salter Packard Children’s HospitalStanford University School of MedicinePalo Alto, CaliforniaAppendicitis (17.2)
David R. Mack, MDProfessor and Chief, Division of Gastroenterology Hepatology and
Nutrition,Children’s Hospital of Eastern OntarioSenior Scientist, CHEO Research InstituteUniversity of OttawaOttawa, Ontario, CanadaProbiotics (19.1a)
Martin G. Martin, MDProfessor, Department of PediatricsDivision of Gastroenterology
49
David Geffen School of Medicine at UCLARonald Reagan UCLA Medical CenterSanta Monica, CaliforniaCongenital Intestinal Transport Defects (15.3b)
Massimo Martinelli, MDDepartment of Translational Medical ScienceSection of PediatricsUniversity of Naples Federico IINaples, ItalyNormal Motility and Development of theIntestinal Neurenteric System (23.1)
Emmanuel Mas, MD, PhDProfessor and Hospital PractitionerDivision of Gastroenterology, Hepatology and NutritionDepartment of PediatricsHôpital des EnfantsUniversité de ToulouseToulouse, FranceTraumatic and Toxic Injury of the Esophagus (6)
Cal S. Matsumoto, MDDirector, Center for Intestinal Care and TransplantationMedStar Georgetown Transplant InstituteWashington, DCIntestinal Tranplantation (21.3)
Suzanne V. McDiarmid, MBChBDepartment of PediatricsRonald Reagan UCLA Medical CenterLos Angeles, CaliforniaTreatment of End-Stage Liver Disease (38)
Maireade E. McSweeney, MD, MPHInstructor, Harvard Medical SchoolDivision of Gastroenterology and Nutrition
50
Co-director Enteral Tube ProgramBoston Children’s HospitalBoston, MassachusettsOutcomes Research on Diagnostic and Therapeutic Procedures (53)
Calies Menard-Katcher, MDAssistant Professor, Pediatrics – Gastroenterology, Hepatology and
NutritionGastrointestinal Eosinophilic Diseases ProgramUniversity of Colorado School of MedicineChildren’s Hospital ColoradoAurora, ColoradoEosinophilic Esophagitis (16.2a)
Laurent Michaud, MDDivision of Gastroenterology, Hepatology and NutritionPediatric ClinicHospital Jeanne de Flandre, CHU de LilleLille, FranceLower Gastrointestinal Bleeding (44.3b)
Michael R. Millar, MDHead, Department of InfectionBarts Health NHS TrustQueen Mary College, University of LondonLondon, United KingdomAntimicrobials (19.1c)
Biren P. Modi, MD, MPHAssociate Surgical DirectorCenter for Advanced Intestinal RehabilitationDirector, Thoracic Outlet Syndrome ProgramBoston Medical CenterAssistant Professor of SurgeryHarvard Medical SchoolBoston, MassachusettsBenign Perianal Lesions (18)
51
Viswanathan Mohan, MD, PhD, DScChairman and Chief DiabetologyDr. Mohan’s Diabetes Specialties CenterPresident and Director, Madras Diabetes Research FoundationChennai, IndiaJuvenile Tropical Pancreatitis (42.2)
Jean-François Mougenot, MDDirector, Pediatric Digestive Endoscopy UnitRobert Debré Hospital and Necker-Enfants MaladesUniversity of ParisParis, FranceIntestinal Polyps and Polyposis (22.1)Endoscopic Retrograde Cholangiopancreatography (44.5)
Vincent A. Mukkada, MDAssociate Professor, Department of PediatricsPediatric GastroenterologyUniversity of CincinnatiCincinnati Children’s HospitalCincinnati, OhioEosinophilic Gastrointestinal Diseases (16.2a)
Simon Murch, PhDProfessor of Pediatrics and Child HealthDivision of Metabolic and Vascular HealthWarwick Medical SchoolThe University of WarwickCoventry, United KingdomFood Allergic Enteropathy (16.2b)Upper Gastrointestinal Endoscopy (44.2a)
Karen F. Murray, MDChief, Division of Gastroenterology and HepatologySeattle Children’s HospitalProfessor of PediatricsUniversity of Washington School of Medicine
52
Seattle, WashingtonAmino Acid Metabolism (34.3)
Hassan Y. Naim, PhDProfessor and Chair, Department of BiochemistryUniversity of Veterinary MedicineHannover, GermanyGenetically Determined Disaccharides Deficiency (15.3a)
Valerio Nobili, MDChief, Hepato-Metabolic Diseases UnitHead, Liver Research UnitBambino Gesù Children HospitalRoma, ItalyFatty Liver Disease in Children (35)
Samuel Nurko, MD, MPHProfessor of Pediatrics, Harvard Medical SchoolDirector, Center for Motility and Functional Gastrointestinal DisordersDirector, Functional Abdominal Pain ProgramGastroenterology, Hepatology and NutritionChildren’s HospitalBoston, MassachusettsGastrointestinal Manometry (47)
Salvatore Oliva, MD, PhDDepartment of PediatricsPediatric Gastroenterology and Liver UnitSapienza University of RomeStaff Physician, Umberto I University HospitalRome, ItalyNormal Motility and Development of the Esophageal Neuroenteric System
(4.1)
Jean-Pierre Olives, MDProfessor of Pediatrics,Gastroenterology and Nutrition Department
53
Children’s HospitalUniversity of ToulouseToulouse, FranceToxic and Traumatic Injury of the Esophagus (6)
Sylviane Olschwang, MD, PhDDepartment of Bio-PathologyINSERM-Institute Paoli-CalmettesMarseille, FranceIntestinal Polyps and Polyposis (22.1)
Keith Chee Y. Ooi, PhDConsultant Pediatric GastroenterologistSydney Children’s HospitalUniversity of New South Wales School of MedicineSydney, AustraliaOther Causes of Gastritis (9.3)
P. Pearl O’Rourke, MDAssociate Professor of PediatricsHarvard Medical SchoolDirector of Human Research Affairs,Partners HealthCare SystemsBoston, MassachusettsEthics and Regulatory Issues (55)
Dinesh S. Pashankar, MDAssociate Professor of PediatricsGastroenterology and HepatologyDirector, Fellowship ProgramDirector, Pediatric IBD programYale School of MedicineNew Haven, ConnecticutBacterial, Parasitic, and Other Infections (30.2)
David H. Perlmutter, MDExecutive Vice Chancellor of Medical Affairs
54
Dean, Washington University School of MedicineSt. Louis, MissouriFormerly Distinguished Professor and Vira I. Heinz Endowed Chair in
PediatricsUniversity of Pittsburgh School of MedicineUniversity of Pittsburgh Medical Centerα1-Antitrypsin Deficiency (34.5)
Prue M. Pereira-Fantini, PhDHead of Laboratory ResearchIntestinal Failure and Clinical Nutrition GroupMurdoch Children’s Research InstituteDepartment of Gastroenterology and Clinical NutritionRoyal Children’s HospitalParkville, Victoria, AustraliaIntestinal Failure–Associated Liver Disease (21.2)
Michel Peuchmaur, MD, PhDDepartment of PathologyUniversity of ParisRobert Debré HospitalParis, FranceIntestinal Polyps and Polyposis (22.1)
Alan David Phillips, PhDConsultant Clinical ScientistDepartment of PediatricsRoyal Free Hospital NHS TrustUniversity College LondonLondon, United KingdomIntestinal Biopsy (46)
Capecomorin S. Pitchumoni, MDGastroenterology and Digestive DiseasesPrivate PracticeHowell, New JerseyTropical Calcific Pancreatitis (42.2)
55
Stephen R. Porter, MD, PhDProfessor of Oral MedicineDepartment of Oral MedicineEastman Dental Institute for Oral Health Care SciencesLondon, EnglandDisorders of the Oral Cavity (2)
John Puntis, BM, DM, MRCPConsultant in Pediatrics Gastroenterology and NutritionLeeds Teaching Hospitals NHS TrustLeeds, United KingdomShort Bowel Syndrome (21.1)
Riad Rahhal, MDClinical Associate Professor of PediatricsVice-chair for EducationCarver College of MedicineUniversity of Iowa HealthcareIowa City, IowaFunctional Constipation (23.2a)
Grant A. Ramm, PhDProfessor, School of Medicine, The University of QueenslandHead, Hepatic Fibrosis LaboratoryCoordinator, Department of Cell and Molecular BiologyNHMRC Senior Research FellowGroup Leader, Hepatic FibrosisQIMR Berghofer Medical Research InstituteHerston Queensland, AustraliaFibrogenesis and Cirrhosis (27.3)
Robert A. Rastall, PhDDepartment of Food and Nutritional SciencesUniversity of ReadingWhiteknights, Reading, United KingdomPrebiotics: Composition and Potential Functions (19.1b)
56
Gerald V. Raymond, MDProfessor, Department of NeurologyUniversity of MinnesotaMinneapolis, MinnesotaZellweger Syndrome and Other Disorders of Proximal Metabolism (34.6)
Robert H. Riddell, MBBSProfessor of Laboratory Medicine and PathobiologyUniversity of TorontoHead, Section of Gastrointestinal PathologyMount Sinai Hospital/Research InstituteToronto, Ontario, CanadaEsophageal and Gastric Polyps and Neoplasms (10)
Eve A. Roberts, MDProfessor, Departments of Pediatrics and PharmacologyUniversity of TorontoDivision of Gastroenterology, Hepatology and NutritionThe Hospital for Sick ChildrenAdjunct Scientist, Genetics and Genome Biology ProgramChildren’s Research InstituteToronto, Ontario, CanadaDrug-Induced Hepatotoxicity (32)
Sanne L. Rosekrans, MDDepartment of Gastroenterology and HepatologyTytgat Institute for Liver and Intestinal ResearchAcademic Medical CenterAmsterdam, the NetherlandsAnatomy and Embryology and Congenital Anomalies (7)
Marc E. Rothenberg, MD, PhDProfessor of Pediatrics, University of Cincinnati College of MedicineDirector, Division of Allergy and ImmunologyDirector, Cincinnati Center for Eosinophilic DisordersCincinnati Children’s HospitalCincinnati, Ohio
57
Eosinophilic Gastrointestinal Diseases (16.2a)
Philip Rosenthal, MDProfessor of Pediatrics and SurgeryUniversity of California School of MedicineDirector, Pediatric HepatologyIntestinal Rehabilitation and Transplantation ProgramSan Francisco, CaliforniaCholedocal Cysts and Other Biliary Disorders (29.4)
Ori D. Rotstein, MDProfessor of SurgeryUniversity of TorontoSt. Michael’s HospitalToronto, Ontario, CanadaPeritonitis and Intra-abdominal Abscesses (20.5)
Marion Rowland, MPH, PhDLecturer in Clinical Research and EpidemiologyCatherine McAuley Research and Education CentreUniversity College Dublin School of MedicineDublin, IrelandHelicobacter pylori and Peptic Ulcer Disease (9.1)
Colin D. Rudolph, MD, PhDProfessor of PediatricsVice-President of Global Medical AffairsChief Medical OfficerMead Johnson NutritionChicago, IllinoisGastroesophageal Reflux (4.2)
Frank M. Ruemmele, MD, PhDNational Institute of Health and Medical Research (INSERM)Necker-Enfants Malades HospitalUniversity of Paris DescartesParis, France
58
Autoimmune Enteropathy and IPEX Syndrome (16.3)
Julie Salomon, MD, PhDDepartment of Pediatric Gastroenterology, Hepatology, and NutritionNecker-Enfants Malades HospitalUniversity of Paris DescartesParis, FranceCongenital Enteropathies (15.3c)
Silvia Salvatore, MD, PhDAssistant Professor of PediatricsGastroenterologyPediatric Clinic of VareseUniversity of Dell’ InsubriaVarese, ItalyEsophageal pH and Impedance Measurement (48)
Miguel Saps, MDProfessor of PediatricsThe Ohio State University College of MedicineDivision of Gastroenterology, Hepatology and NutritionNationwide Children’s HospitalDirector of Research, The Motility CenterColumbus, OhioNormal Motility and Development of the Gastric Neuroenteric System
(11.1)Motility Disorders (11.2)
David F. Schaeffer, MDAssistant Professor, University of British ColumbiaHead, Division of Anatomical Pathology and Laboratory MedicineVancouver General HospitalVancouver, British Columbia, CanadaEsophageal and Gastric Polyps and Neoplasms (10)
Richard A. Schreiber, MDClinical Professor, Department of Pediatrics
59
University of British ColumbiaDivision of Gastroenterology, Hepatology and NutritionClinical InvestigatorBritish Columbia Children’s Hospital Research InstituteVancouver, British Columbia, CanadaBacterial, Parasitic, and Other Infections (30.2)
Kenneth D. R. Satchel, PhDProfessor of PediatricsUniversity of Cincinnati College of MedicineDirector, Clinical Mass Spectrometry LabCincinnati Children’s Hospital Medical CenterCincinnati, OhioBile Acid Synthesis and Metabolism (34.11)
Eyal Shemesh, MDAssociate Professor of Pediatrics and PsychiatryIcahn School of Medicine at Mount SinaiChief, Division of Behavioral and Developmental HealthDepartment of Pediatrics, Mount Sinai Medical CenterNew York, New YorkAdherence to Medical Regimens (52)
Ross W. Shepherd, MDProfessor of Pediatrics Baylor College of MedicineDirector, Texas Children’s Liver CenterDepartment of Pediatric Gastroenterology, Hepatology and NutritionHouston, TexasFibrogenesis and Cirrhosis (27.3)
Delane Shingadia, MPH, FRCPCHConsultant, Department of Pediatric Infectious Diseases and Specialty
LeadGreat Ormond Street Hospital for ChildrenLondon, United KingdomGastrointestinal Manifestations of Immunodeficiency Disorders (20.2)
60
Steven C. Shlozman, MDAssistant Professor of PsychiatryHarvard Medical SchoolAssociate Director, The Clay Center for Young Healthy MindsMassachusetts General Hospital for ChildrenBoston, MassachusettsPsychological Aspects of Chronic Disease (51)
Ian F. Slack, MDPost-graduate ResidentDepartment of PediatricsThe Children’s Hospital of PhiladelphiaPhiladelphia, PennsylvaniaGenetic Testing (34.1)
Virpi V. Smith, PhDPediatric SurgeryUCL Great Ormond Institute of Child HealthUniversity College,London, United KingdomIntestinal Biopsy (46)
Etienne M. Sokal, MD, PhDProfessor of MedicineDivision of Gastroenterology and HepatologyCatholic University of Louvain and Clinic of Saint LukeFounder, Promethera BiosciencesBrussels, BelgiumDiagnostic Approaches to Common Pediatric Liver Problems (28)
Nancy B. Spinner, PhDProfessor of Genetics, Pediatrics, and Pathology and Laboratory MedicinePerelman School of Medicine at University of PennsylvaniaChief, Division of Genomic DiagnosticsThe Children’s Hospital of PhiladelphiaPhiladelphia, PennsylvaniaGenetic Testing (34.1)
61
Annamaria Staiano, MDDepartment of PediatricsUniversity Federico IINaples, ItalyNormal Motility and Development of the Intestinal Neurenteric System
(23.1)
Steven J. Steinberg, PhDMedical Director, Molecular GeneticsMcKusick-Nathans Institute of Genetic MedicineJohns Hopkins University School of MedicineBaltimore, MarylandZellweger Syndrome and Other Disorders of Peroxisomal Metabolism
(34.6)
Jennifer P. Stevens, MDAssistant Professor, Harvard Clinical and Translational Science CenterCenter for Healthcare Delivery ScienceDivision of Pulmonary and Critical CareBeth Israel Deaconess Medical CenterBoston, MassachusettsEthics/Regulatory Issues (55)
Ekkehard Sturm, MD, PhDDepartment of Pediatric Gastroenterology and HepatologyUniversity Children’s HospitalUniversity Hospital TüebingenTuebingen, GermanyDisorders of the Intrahepatic Ducts (29.1)
Stuart Tanner, FRCP, FRCPHEmeritus Professor of PediatricsUniversity of Sheffield and Academic Unit of Child HealthSheffield Children’s HospitalSheffield, United KingdomWilson Disease (34.8)
62
Glenn P. Taylor, MDAssociate Professor, University of TorontoLaboratory Medicine and PathobiologyMount Sinai HospitalToronto, Ontario, CanadaEsophageal and Gastric Polyps and Neoplasms (10)
Jonathan E. Teitelbaum, MDAssistant Professor of PediatricsDrexel University School of MedicineDirector, Pediatric Gastroenterology and NutritionThe Children’s Hospital at Monmouth Medical CenterLong Branch, New JerseyPhiladelphia, PennsylvaniaAnatomy, Embryology, and Congenital Anomalies (1)
Mike Thomson, MDConsultant Pediatric Gastroenterology and Honorary Reader in Pediatric
GastroenterologyDirector, International Academy of Pediatric Endoscopy TrainingSheffield Children’s Hospital NHS Foundation TrustSheffield, United KingdomEsophagitis (5)Ileocolonoscopy and Enteroscopy (44.3a)Interventional Endoscopy: Recent Innovations (44.6)
Franco Torrente, MDClinical AssistantDepartment of Pediatric GastroenterologyG. Gaslini InstituteGenoa, ItalyFood Allergic Enteropathy (16.2b)
Khoa Tran, MDAssistant Professor of Pediatric GastroenterologyTufts University School of MedicineTufts Medical Center
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Boston, MassachusettsOther Dysmotilities Including Chronic Intestinal Pseudo-obstruction
Syndrome (23.2c)
David N. Tuchman, MDPediatric GastroenterologySinai HospitalLifebridge HealthBaltimore, MarylandDisorders of Deglutition (3)
Dominique Turck, MDProfessor of PediatricsUniversity of LilleLille, FranceLower Gastrointestinal Bleeding (44.3b)
Dimitrios Tzimorotas, PhDDepartment of Food and Nutritional SciencesUniversity of ReadingWhiteknights, Reading, United KingdomPrebiotics: Composition and Potential Functions (19.1b)
Aliye Uc, MDProfessor of PediatricsDivision of Pediatric GastroenterologyChildren’s Hospital of IowaUniversity of Iowa Carver College of MedicineIowa City, Iowa, USAFunctional Constipation (23.2a)
Reza Vali, MDAssistant Professor, Nuclear MedicineDepartment of Diagnostic ImagingHospital for Sick ChildrenUniversity of TorontoToronto, Canada
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Radionucleotide Diagnosis (49.4)
Kristin Whitfield Van Buren, MDAssistant Professor of PediatricsBaylor College of MedicineDivision of Gastroenterology, Hepatology and NutritionFellowship Program DirectorTexas Children’s HospitalHouston, TexasUpper Gastrointestinal Bleeding (44.2b)
Gijs R. van den Brink, MD, PhDTytgat Institute for Liver and Intestinal ResearchAcademic Medical Center,Amsterdam, the NetherlandsAnatomy, Embryology, and Congenital Anomalies (7)
Yvan Vandenplas, MD, PhDProfessor of Pediatrics,Department of Pediatrics, Gastroenterology, Nutrition and DieteticsVrije University BrusselsBrussels, BelgiumEsophageal pH and Impedance Measurement (48)
Roshni Vara, MBBSDepartment of Inherited Metabolic DiseaseEvelina Children’s HospitalSt Thomas’ HospitalLondon, United KingdomDisorders of Mitochondrial and Fatty Acid Metabolism (34.4)
Diego Vergani, MD, PhDProfessor of Liver ImmunopathologyInstitute of Liver StudiesKing’s College HospitalLondon, United KingdomAutoimmune Liver Disease (31)
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Hector Vilca-Melendez, MD, PHDConsultant Transplant SurgeonInstitute of Liver StudiesKing’s College HospitalLondon, United KingdomLiver Transplantation (39)
Pierre-Yves von der Weid, PhDAssociate Professor, Department of Physiology and PharmacologyCumming School of MedicineInflammation Research Network & Smooth Muscle Research GroupSnyder Institute for Chronic DiseasesUniversity of CalgaryCalgary, Alberta, CanadaLymphatic Disorders (14)
Brad W. Warner, MDJessie L. Ternberg, MD, PhD Distinguished Professor of Pediatric SurgeryWashington University School of MedicineSurgeon-in-chief, St. Louis Children’s HospitalSt. Louis, MissouriIntestinal Obstructions (17.1)
Paul A. Watkins, MD, PhDProfessor of NeurologyJohns Hopkins University School of MedicineResearch Scientist, The Kennedy Krieger Institute Baltimore, MarylandZellweger Syndrome and Other Disorders of Peroxisomal Metabolism
(34.6)
David C. Whitcomb, MD, PhDGiant Eagle Foundation Professor of Cancer GeneticsProfessor of Medicine, Cell Biology and Physiology and Human GeneticsUniversity of Pittsburgh Medical CenterDivision of Gastroenterology, Hepatology and NutritionDirector, UPMC Precision Medicine ServicePittsburgh Pennsylvania
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Pancreatitis: Acute and Chronic (42.1)
Peter F. Whitington, MDProfessor Emeritus of Pediatrics, Feinberg Medical School of
Northwestern UniversityDivision Head, Gastroenterology, Hepatology, and NutritionDirector, Organ Transplantation and Siragusa Transplantation CenterAnn and Robert Lurie Children’s Hospital of ChicagoChicago, IllinoisHemochromatosis (34.9)
David John Wilkinson, BMedSci, MBChBDepartment of Pediatric SurgeryUniversity of Liverpool Institute of Translational MedicineAlder Hey Children’s Hospital NHS Foundation TrustLiverpool, United KingdomCongenital Anomalies Including Hernias (13)
Mark Wilks, PhDClinical Scientist, Department of Microbiology and VirologyVisiting and Honorary Academic, Blizard Institue of Cell and Molecular
ScienceBarts and the London National Health Service TrustLondon, United KingdomAntimicrobials (19.1c)
Michael Wilschanski, MBBSProfessor of PediatricsThe Hebrew University-Hadassah School of MedicineDirector, Pediatric Gastroenterology UnitJerusalem, IsraelCystic Fibrosis (43.1)Shwachman-Diamond Syndrome (43.2)Other Hereditary and Acquired Pancreatic Disorders (43.3)
Eytan Wine, MD, PhDAssociate Professor
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Departments of Pediatrics and PhysiologyUniversity of AlbertaEdmonton, Alberta, CanadaMicrobial Interactions with Gut Epithelium (19.1)
Ernest M. Wright, PhD, DScProfessor of PhysiologyDavid Geffen School of MedicineUniversity of CaliforniaSan Francisco, CaliforniaCongenital Intestinal Transport Defects (15.3b)
Nada A. Yazigi, MDPediatric GastroenterologyMedStar Georgetown Transplant InstituteWashington, DCIntestinal Transplantation (21.3)
Sona Y. Young, MDNorthShore University Health SystemEvanston HospitalEvanston, IllinoisAcute Diarrhea (15.2a)
Klaus-Peter Zimmer, MDProfessor of PediatricsDepartment of General Pediatrics and NeonatologyUniversity of GiessenGiessen, GermanyGenetically Determined Disaccharides Deficiency (15.3a)
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PREFACE
As I look back on the five previous editions of this textbook, I recognizehow much the field has expanded and the importance of our understandinggastrointestinal diseases within the context of ongoing development ofinfants and children. This edition of the textbook will be published laterthan previous editions, which have been linked to the PediatricGastroenterology World Congresses occurring every four years since2000. This is due to a change in publishers. People’s Medical PublishingHouse—USA will now be publishing this textbook as well as itscompanion text, entitled Nutrition in Pediatrics (5th edition). In addition,new sections have been added to reflect the expanding complexity of thefield, particularly new techniques now used to accurately establishcomplex diagnoses.
It is now apparent that pathophysiologic occurrences during infancy,and even during the intrauterine period, may have a profound effect on theexpression of disease in later life. Two hypotheses, the Barker hypothesis(fetal programming) and the hygiene hypothesis, which have now beengenerally accepted, strongly suggest the importance of fetal nutrition andneonatal colonization on the enhanced incidence of metabolic andimmunologic diseases increasingly prevalent in developed countries. Thisis strongly illustrated by the pediatric complications of maternal obesityduring pregnancy and the increased incidence of Cesarean section deliveryof the newborn for the convenience of the obstetrician and parents at theexpense of adequate initial colonization for the newborn infant.Accordingly, this edition of Pediatric Gastrointestinal Disease and itscompanion Pediatric Nutrition devote new chapters to the adequatenutrition beginning in utero and the colonization of the newborn intestineand its disease consequences.
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As with previous editions, the size and content of each section hasbeen modified and new chapters added to reflect an increasing evolution ofsubspecialty programs within gastroenterology and a better understandingof subpecialty conditions (motility, inflammation, and autoimmune boweldisease) that have emerged in pediatric gastroenterology. As theimportance of genetics and molecular biology in the pathophysiology anddiagnosis of complex pediatric conditions of the gut, pancreas, and liverhave become appreciated, expanded chapters cover their importance in thefield. Furthermore, a more comprehensive representation of world leadersin pediatric gastroenterology among the editors of this edition haveexpanded the authorship to reflect experts in specific diseases withinvarious continents rather than principally in North America. In otherwords, the edition is dedicated to a comprehensive, worldwide approach tothe practice of pediatric gastroenterology.
Finally, as occurs in many rapidly expanding medical fields, difficultdecisions are required before treatment of complex gastrointestinalconditions can be recommended that are based on clinical evidence. Thesetherapeutic approaches require large, multi-centered, randomly controlled,single-protocol studies. Accordingly, many pediatric gastroenterologistsare now resorting to clinical investigation to answer their therapeuticneeds. In this edition, two comprehensive chapters on principles of clinicalinvestigation are provided, and then, in an endeavor to be inclusive,another chapter on ethics and regulatory considerations is included. Inaddition, psychosocial aspects of gastrointestinal disease are provided inthree chapters that consider the physiologic components of intestinaldisease.
The editors have provided an excellent background for an approach toevaluating children with gastrointestinal disease in the 21st century. I havebeen honored to be part of this approach.
—Allan Walker
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I Mouth andEsophagus
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1 Anatomy, Embryology,Congenital Anomalies ofthe Mouth and Esophagus
Jonathan E. Teitelbaum, MD
Congenital anomalies of the mouth and esophagus are relatively common.The majority of these anomalies are readily apparent at birth or, in manycases, can be appreciated on prenatal ultrasonography. Our understandingof the embryology of the mouth and esophagus and the associated genesand gene products has increased rapidly over the past decade. With that,there has been a rapid advancement in identifying various geneticmutations that result in abnormalities of development and their phenotypicexpression. Whereas some malformations are clinically silent, others cancause difficulties in feeding, articulation, or even life-threateningrespiratory difficulties. More complex malformations often require supportfrom multidisciplinary teams, including surgeons (general,otolaryngologic, orthodontic), gastroenterologists, speech pathologists, andgeneticists.
FACIAL CLEFTS (CLEFT LIP AND CLEFTPALATE)Oral clefts are among the most common of all birth defects, second only toclubfoot. Cleft lip with or without cleft palate [CL(P)] occurs with anincidence of 1 in 500 to 1 in 2,500 in different populations based on ethnicgroup, geographic location, and socioeconomic conditions.1 The highestincidence is among Native Americans (3.6 in 1,000 live births), whereas
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among blacks, it is less (0.3 in 1,000 live births). Whites have an incidenceof 1 in 1,000 live births. Defects are unilateral in 80%.2
Isolated cleft palate (CP) occurs in approximately 1 in 2,000 livebirths, and there is little to no racial preponderance.2 CL(P) is morecommon in boys, whereas CP is seen more commonly in girls. The causeis likely multifactorial disruption of embryologic morphogenesis.2 Higherbirth order may also be a risk factor for CL(P) and CP. However, studiesare not conclusive and may be confounded by other factors, such asadvanced maternal or paternal age or increased exposure to teratogens,which, in themselves, may be risk factors.3 While nonsyndromic clefts aregenerally believed to be relatively benign conditions, a study shows thatthe perinatal mortality rate for babies with isolated facial clefts wassignificantly higher than the background population (odds ratio 3.3).4
The risk of having subsequent children with clefts is different for thosewith CL(P) from those with CP. When both parents are unaffected andhave an affected child, the risk of recurrence is 4.4% for CL(P) and 2.5%for CP. If one parent is affected, the risk is increased to 15.8% forCL(P)and 14.9% for CP. If two children are affected and the parents areunaffected, the risk for a third child is 9% for CL(P) and 1% for CP.2Concordance among monozygotic twins ranges between 40% and 60%,whereas it is 5% among dizygotic twins. The lack of 100% concordancerates among monozygotic twins argues against genetic events alone beingresponsible for the clefting phenotype.5
Cleft lip is a unilateral or bilateral gap in the upper lip and jaw, whichform during the third to seventh week of embryologic development.1 Theincisive foramen divides the hard palate into a primary and secondarypalate. The primary palate lies anterior to the incisive foramen andincludes the bony premaxilla, mucoperiosteal covering, and incisor teeth.The secondary palate is posterior to the incisive foramen and is composedof horizontal plates of the maxilla and palatine bone. The remainingdentition arrives from the secondary palate. Primary palate formationbegins at four to five weeks gestation with the fusion of the paired mediannasal prominences. This marks the separation of the oral and nasalcavities. Ultimately, the median nasal prominences give rise to the dentalarch, incisor teeth, and philtrum of the upper lip. Formation of thesecondary palate (hard and soft) begins at approximately the seventh weekof gestation. The posterior maxillary prominences form palatal shelves,which rotate inferiorly and medially to fuse with the vomer in the midline.Anterior to posterior palatal closure occurs in a zipper-like fashion. At nine
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weeks gestation, the hard palate fuses with the septum to complete theseparation of the oral and nasal cavities. The soft palate is composed offive paired muscles: tensor veli palatini, levator veli palatini,palatoglossus, palatopharyngeus, and musculus uvulae. Midlineapproximation of the soft palatal musculature marks the completion ofpalatogenesis at approximately 12 weeks gestation.2
The multifactorial inheritance model is currently the most widelyaccepted theory of nonsyndromic clefts. In this model, the risk ofdeveloping a given anomaly is determined by the presence of eithergenetic or environmental liabilities. Each liability occurs in a normaldistribution within the population. The accumulation of multiple smallliabilities eventually reaches a threshold, beyond which a defect occurs.Variable penetrance of the phenotype for many genes results in non-Mendelian inheritance patterns. Ongoing research is investigating the rolethat associated features play in the familial transmission patterns ofnonsyndromic clefts. Associated features include fluctuating anddirectional asymmetry, left handedness, dermatoglyphic patterns,craniofacial morphology, orbicularis oris muscle defects, structural brainand vertebral anomalies, minor physical anomalies, and velopharyngealincompetence.6
An estimated 300 syndromes include CL(P) in their phenotype;however, syndromic clefts account for only 30% of CL(P).1 Theproportion of patients with CP who are syndromic versus nonsyndromicremains unresolved, with estimates varying widely, between 15% and80%.1 Approximately 25% of syndromic clefts are associated with Sticklersyndrome, whereas another 15% are associated with velocardiofacialsyndrome.2 The most common malformations in association with clefts arefound in the central nervous system and the skeletal system, followed bythe urogenital and cardiovascular systems.7 Various syndromes associatedwith CP (Table 1-1) and CL(P) (Table 1-2) have been described.
Defects in the PVRL1 gene (chromosome 11q23) result in abnormalformation of nectin 1, a cell–cell adhesion molecule expressed in thedeveloping face and palate that is essential for fusion of the medial edgeepithelia. A 50% reduction in the amount of nectin 1 appears to be a riskfactor for nonsyndromic CL(P) in patients in Margarita Island andVenezuela.1 A similar gene, OFC3 (19q13, MIM#600757), has also beenimplicated in nonsyndromic CL(P) based on genetic linkage studies. Othercandidate genes include OFC1 (6p24.3, MIM #119530), OFC2 (2p13,MIM #602966), OFC4 (4q, MIM#608371), OFC5 (MSX1, 4p16.1, MIM
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#608874), OFC6 (MIM#608864), TP63 (3q27), TGFA (2p13), TBX22,1PGD1 (1p36), 6 methylenetetrahydrofolate reductase (1q36),8,9
transcobalamin 2,10 and TGFalpha (2p13).8 A defect in the CDH1/E-cadherin gene (MIM#192090) has been associated with CL(P) andhereditary diffuse gastric cancer.11
The causative role of teratogens in the formation of clefts has beensupported by studies of maternal exposures to corticosteroids, phenytoin,valproic acid,5 thalidomide,5 alcohol,5 cigarettes,5 dioxin,5 or retinoicacid. In addition, maternal diabetes mellitus, hormone imbalance, andvarious vitamin and trace mineral deficiencies have been associated withclefts in the offspring.12 Although there is no consensus that any particularteratogen or environmental factor is implicated in most clefts,1 somestudies suggest that the risk may be increased with exposure to oxygenated(odds ratio 1.8), chlorinated (odds ratio 9.4), and petroleum (odds ratio3.6) solvents.13 Folic acid may have a protective effect to reduce the riskof clefting, although this is controversial.8,14 Studies suggest that maternalzinc deficiency may also be associated with nonsyndromic clefting.15,16
Prenatal diagnosis allows for early parental counseling. Currenttechnology can detect CL(P) at 15 weeks gestation because the soft tissuesof the fetal face become distinct to transabdominal ultrasonography.7During the second trimester, ultrasonography detects less than 20% ofcases of isolated CL(P) and far fewer cases of isolated CP.7 However,syndromic CL(P) is detected in more than 30%, perhaps because a moredetailed scan is undertaken given the associated anomalies, or becausethese clefts are larger and more readily visualized. Optimum timing fordiagnosis is between 20 and 22 weeks gestation. The ability to see thedefect is influenced by the position of the fetus, position of an overlyinghand or umbilical cord, maternal obesity, multiple pregnancies,oligohydramnios, and the experience of the technician. The use oftransvaginal ultrasonography and three-dimensional ultrasonography alsoincreases the sensitivity and specificity of the test.2 Real-time magneticresonance imaging (MRI) has also been proven useful in prenataldiagnosis of CP.17 A delay of greater than 24 hours in diagnosis ofnonsyndromic CP without cleft lip in the newborn can occur in as much as37%.18 Those with a delay in diagnosis often had feeding problems ornasal regurgitation. The authors suggest that all newborns should undergovisual inspection of the palate as palpation alone is inadequate.
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TABLE 1-1 Cleft Palate or Bifid Uvula Without Cleft Lip
Syndrome OMIM Numbera
Catel-Manzke 302380
Cerebrocostomandibular 117650
Deletion 4q
Dubowitz 223370
Duplication 3q
Duplication 10q
Escobar 265000
Femoral hypoplasia–unusual facies 134780
Fibrochondrogenesis 228520
Hay-Wells syndrome of ectodermal dysplasia 106260
Hydrolethalus 236680
Kabuki make-up 147920
Kniest dysplasia 156550
Marden–Walker 248700
Meckel–Gruber 249000
Nager 154400
Orofaciodigital 311200
Otopalatodigital, type I 311300
Otopalatodigital, type II 304120
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Popliteal pterygium 119500
Retinoic acid embryopathy 243440
Short-rib polydactyly, type II 263520
Velocardiofacial 192430
Spondyloepiphyseal dysplasia congenita 183900
Stickler 1 08300
Treacher Collins 154500
Van der Woude 119300
aSearching the OMIM (Online Mendelian Inheritance in Man) can be done athttp://www3.ncbi.nlm.nih.gov/omim/.
Source: Adapted from Jones.120
TABLE 1-2 Syndromes With Cleft Lip With or WithoutCleft Palate
Syndrome OMIM Numbera
Deletion 4p
Ectrodactyly–ectodermal dysplasia–clefting 604292
Fryns 229850
Hay-Wells syndrome of ectodermal dysplasia 106260
Holoprosencephaly sequence 157170
Miller 247200
Mohr 252100
Orofaciodigital 311200
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Popliteal pterygium 119500
Rapp-Hodgkin ectodermal dysplasia 129400
Roberts 268300
Short-rib polydactyly, type II 263520
Trisomy 13
Van der Woude 119300
aSearching the OMIM (Online Mendelian Inheritance in Man) can be done athttp://www3.ncbi.nlm.nih.gov/omim/.
Source: Adapted from Jones.120
Initial evaluation of a patient with CP should include prenatal care,birth history, teratogen exposure, and a family history of clefting orsyndromes. A multidisciplinary team is often helpful in assessing thefamily’s medical and psychosocial needs. The cleft team should consist ofa maxillofacial or plastic surgeon, otolaryngologist, audiologist, dentist(orthodontist or oral surgeon), social worker, geneticist, pediatrician,nutritionist, and speech pathologist. Breastfeeding is possible in somepatients with a short or narrow cleft.19 Infants with larger clefts can rarelygenerate adequate suction for traditional breastfeeding or bottle feeding.Various specialized nipples have been created to facilitate feeding.Feeding typically takes longer, and frequent burping may be required inthese infants because they often swallow large amounts of air. Infantsshould be weighed on a weekly basis initially to ensure adequate intake.2Evidence suggests that children with CL have a lesser degree of weightand length impairment in the first few years of life compared to thosechildren with cleft lip and palate or CP.20
Palatal clefting disrupts all layers of the normal palate architecture,including mucosa, muscle, and bone. The muscles of the soft palate mustwrap anteriorly and insert on the cleft margin or the posterior palate.Aberrant tensor veli palatini insertion results in eustachian tubedysfunction, so nearly all CP patients will have chronic otitis mediarequiring myringotomy tube placement. Abnormal insertion of the levatorveli palatini results in loss of normal velopharyngeal competence.2
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CP may be classified as primary or secondary, complete or incomplete,unilateral or bilateral, or submucous. Primary CP results in incompleteclosure of the hard palate anterior to the incisive foramen, whereassecondary CP results in a midline defect posterior to the incisive foramen.Secondary clefts appear to be distinct genetic entities, unrelated to cleft lipbut often associated with Pierre Robin sequence (PRS). Complete CPinvolves the primary secondary and soft palate and is usually associatedwith cleft lip. Submucous CP results from inadequate development of themuscles of the soft palate without disruption of the mucosa. They cancharacteristically include a bifid uvula, dehiscence of the central palatalmusculature (may be palpable or result in bluish discoloration in themidline, termed a zona pellucida), and loss of the posterior nasal spine.2
Presurgical orthopedic techniques are used to modify the shape of thecleft deformity before definitive cleft repair. These increase the ease of theprimary repair, normalize facial growth, and prevent alveolar collapse.Active techniques include finger massage, lip taping and strapping, andoral prosthetics. Passive techniques are aimed at inhibiting tongueprotrusion between the palatal shelves by using oral obturators. Althoughthese techniques have been shown to effectively narrow the distancebetween alveolar segments, no differences in esthetic outcome, need forrevision surgery, or improvement in feeding have been prospectivelydemonstrated.2
Palatoplasty aims to separate the oral and nasal cavities and restorevelopharyngeal competence. An aggressive approach must be balancedwith the risk of maxillary growth disturbance.2
Although 90% of patients with a cleft lip have repair between 3 and 6months of age,21 the timing of CP repair is controversial. Proponents ofearly CP repair (3–6 months) believe that early velopharyngealcompetence is critical to normal speech development. Proponents of latepalatal repair (2–15 years) believe that the risk of iatrogenic disruption ofpalatal growth and midfacial hypoplasia outweighs the risk of speechabnormalities. Clefts delayed for more than two years generally requireobturation to overcome velopharyngeal incompetence and allow normalspeech development. Oral obturators placed prior to two years are oftenpoorly tolerated. The lack of clear evidence supporting early versus laterepair has led to a compromise in which most surgeons perform repairfrom 12 to 24 months.2 Experience with neonatal cleft lip and palate repairhas been described as safe, although long-term follow-up is not yetavailable.22 Fetal surgery for CL(P) has also been described.23 The risks
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