Practical Algorithms in Pediatric Gastroenterology Editor Ron Shaoul, Haifa 51 graphs and 9 tables, 2014 BBaanseglk ·o Fkr ·e Bibeuijrign g· P· aSrhisa n· Lgohnadi ·o Tno ·k Nyoe w· K Yuoarlka ·L Cuhmepnunra i· ·S Ninegwa pDoerleh i· ·S ydney Downloaded by: Kainan University 203.64.11.45 - 1/25/2015 4:52:43 PM Contents IV Contributors Gastroesophageal refl ux and vomiting 44 Chronic intestinal II pseudo-obstruction 22 Neonatal vomiting VI Preface R. Shaoul; N.L. Jones T. Zangen; P.E. Hyman Z. Hochberg 24 Nausea and vomiting 46 Irritable bowel syndrome VII Introduction P.S. Lemos; R. Shaoul E. Chiou; S. Misra; S. Nurko R. Shaoul 26 Recurrent vomiting and/or regurgitation Stomach and intestine Y. Vandenplas; R. Shaoul Various gastrointestinal conditions 48 Protein-losing enteropathy 2 Abdominal pain 28 Typical and atypical refl ux syndrome B. Zeisler; F.A. Sylvester Y. Vandenplas; R. Shaoul R. Arnon; S. Misra 50 Celiac disease 4 Acute gastroenteritis 30 Recurrent vomiting and/or Y. Bujanover; R. Shaoul regurgitation and poor weight gain A. Lo Vecchio; D. Turck; A. Guarino 52 Helicobacter pylori Y. Vandenplas; R. Shaoul 6 Food allergy N.L. Jones; B.D. Gold 32 Cyclic vomiting syndrome B. Wershil; F.A. Sylvester 54 Gastrointestinal polyps Y. Vandenplas; B.D. Gold 8 Failure to thrive B. Zeisler; F.A. Sylvester A. Lahad; S. Reif 56 Intestinal malabsorption – Part 1: Other motility disorders Pathogenesis and etiology 10 Chronic diarrhea A. Guarino; E. Ruberto; Y. Finkel Y. Finkel; A. Guarino 34 Achalasia 12 Upper gastrointestinal bleeding S. Nurko; Y. Vandenplas 58 Intestinal malabsorption – Part 2: First diagnostic steps C.G. Sauer; B.D. Gold 36 Constipation A. Guarino; E. Ruberto; Y. Finkel 14 Lower gastrointestinal bleeding S. Misra; H.M. Van de Vroot F.A. Sylvester; D. Turck 38 Dysphagia 16 Malnutrition P.E. Hyman; T. Zangen Infl ammatory bowel disease P.S. Lemos; B. Wershil 40 Fecal incontinence 60 Infl ammatory bowel disease 18 Perianal disease A. Siddiqui; O. Eshach Adiv; S. Nurko D. Turner; A.S. Day A.S. Day; N.L. Jones 42 Hirschsprung’s disease 62 Crohn’s disease 20 Gastrointestinal foreign bodies P.E. Hyman; T. Zangen D. Turner; A.S. Day I. Rosen; R. Shaoul 64 UD. lTcuernraert;i vAe.S .c Doalyitis Downloaded by: Kainan University 203.64.11.45 - 1/25/2015 4:52:43 PM Surgical conditions 80 Hepatitis C Pancreas E. Granot 66 Abdominal mass 100 Acute pancreatitis R. Udassin; A. Vromen; E. Gross 82 Elevated aminotransferases M. Wilschanski; R. Arnon J. Garah; R. Shaoul 68 Right lower quadrant abdominal pain 102 Chronic pancreatitis I. Sukhotnik; P.S. Lemos 84 Elevated alkaline phosphatase M. Wilschanski; R. Shaoul R. Shaoul; J. Garah 70 Peritonitis S. Peleg; R. Shaoul 86 Autoimmune liver disease 104 Index G. Mieli-Vergani; M. Samyn 72 Short bowel syndrome Y. Finkel; I. Sukhotnik 88 Sclerosing cholangitis 1 09 Abbreviations G. Mieli-Vergani; M. Samyn 90 Acute liver failure Liver G. Mieli-Vergani; M. Samyn 74 Neonatal jaundice 92 Hepatomegaly E. Fitzpatrick; A. Dhawan E. Fitzpatrick; A. Dhawan 76 Acute hepatitis 94 Gallstones Y. Bujanover; S. Reif E. Broide; S. Reif 78 Hepatitis B 96 Portal hypertension E. Granot R. Arnon; A. Dhawan 98 Ascites A. Ben Tov; S. Reif Library of Congress Cataloging-in-Publication Data Disclaimer. The statements, options and data contained in this publi- All rights reserved. No part of this publication may be translated into cation are solely those of the individual authors and contributors and other languages, reproduced or utilized in any form or by any means, Practical algorithms in pediatric gastroenterology / editor Ron Shaoul. not of the publisher and the editor(s). The appearance of advertise- electronic or mechanical, including photocopying, recording, micro- p. ; cm. -- (Practical algorithms in pediatrics) ments in the book is not a warranty, endorsement, or approval of the copying, or by any information storage and retrieval system, without Includes bibliographical references and index. products or services advertised or of their effectiveness, quality or permission in writing from the publisher. ISBN 978-3-318-02509-5 (alk. paper) -- ISBN 978-3-318-02510-1 safety. The publisher and the editor(s) disclaim responsibility for any (e-ISBN) injury to persons or property resulting from any ideas, methods, in- © Copyright 2014 by S. Karger AG, P.O. Box, CH–4009 Basel I. Shaoul, Ron, editor of compilation. II. Series: Practical algorithms in structions or products referred to in the content or advertisements. (Switzerland) pediatrics. Printed in Switzerland on acid-free and non-aging paper (ISO 9706) [DNLM: 1. Gastrointestinal Diseases--Handbooks. 2. Adolescent. 3. Drug Dosage. The authors and the publisher have exerted every effort by Werner Druck, Basel III Child. 4. Decision Trees--Handbooks. 5. Infant. 6. Liver to ensure that drug selection and dosage set forth in this text are in ISBN 978–3–318–02509–5 Diseases--Handbooks. 7. Pancreatic Diseases--Handbooks. WS 39] accord with current recommendations and practice at the time of pub- e-ISBN 978–3–318–02510–1 RJ446 lication. However, in view of ongoing research, changes in government 618.92’33--dc23 regulations, and the constant fl ow of information relating to drug ther- 2014004252 afwoopmary rme naainencndhgd sdde rarduun gagd g r fpeeoranrec tca tiainsouy ant cisnoh, entahwsne. g a Trenhe diains/d o iiesnrr dpi nisiacf rruateritqcigouuenledasn r taltoyln y cid mhe medpcoopksrl atothagyenee t pd awa ndchdrkue afgnog .rte ha iend srdeeecrd-t Downloaded by: Kainan University 203.64.11.45 - 1/25/2015 4:52:43 PM Contributors Orly Eshach Adiv, MD Emer Fitzpatrick Avishay Lahad, MD IV Pediatric Gastroenterology and Nutrition Unit Consultant Paediatric Hepatologist Pediatric Gastroenerology and Nutrition Unit Rambam Medical Center Paediatric Liver GI and Nutrition Center Pediatric B North Department Haifa, Israel King’s College Hospital Edmond and Lili Safra Children’s Hospital London, UK Sheba Medical Center Ronen Arnon, MD, MHA Tel Hashomer, Israel Associate Professor of Pediatrics and Surgery Jamal Garah, MD Medical Director of Pediatric Hepatology and Pediatric Gastroenterology and Nutrition Unit Piedade Sande Lemos, MD, PhD Liver Transplantation Rambam Medical Center Pediatric Gastroenterology Consultant Recanati-Miller Transplant Institute Haifa, Israel Hospital Fernando Fonseca Mount Sinai School of Medicine Director, Clinica CUF Cascais New York, NY, USA Benjamin D. Gold, MD, FACG Cascais, Portugal Pediatric Gastroenterology, Hepatology and Efrat Broide, MD Nutrition Andrea Lo Vecchio, MD Head of Pediatric Gastroenterology Children’s Center for Digestive Healthcare Section of Pediatrics Institute of Gastroenterology Atlanta, GA, USA Department of Translational Medical Science Assaf Harofeh Medical Center University of Naples Federico II Zerifin, Israel Esther Granot, MD Naples, Italy Kaplan Medical Center Prof. Yoram Bujanover Rehovot and Hebrew University-Hadassah Giorgina Mieli-Vergani Pediatric Gastroenterology Unit Medical School Professor of Paediatric Hepatology Edmond and Lili Safra Children’s Hospital Jerusalem, Israel Consultant Paediatric Hepatologist Sheba Medical Center Paediatric Liver, GI and Nutrition Centre Tel Hashomer, Israel Eitan Gross, MD King’s College London School of Medicine Pediatric Surgery Department King’s College Hospital Eric Chiou, MD The Hebrew University Medical School London, UK Pediatric Gastroenterology, Hepatology and Hadassah Nutrition Jerusalem, Israel Sudipta Misra, MBBS, MD, DM Texas Children’s Clinical Care Center Clinical Professor of Pediatrics and Chief Houston, TX, USA Alfredo Guarino, MD Division of Pediatric Gastroenterology, Section of Pediatrics Hepatology and Nutrition Prof. Andrew S. Day Department of Translational Medical Science Brody School of Medicine, East Carolina University Paediatric Gastroenterologist University of Naples Federico II Vidant Medical Center Department of Paediatrics Naples, Italy Greenville, NC, USA University of Otago Christchurch, New Zealand Paul E. Hyman, MD Samuel Nurko, MD Professor of Pediatrics, Louisiana State University Director Anil Dhawan Chief, Gastroenterology Center for Motility and Professor of Paediatric Hepatology Children’s Hospital Functional Gastrointestinal Disorders Director Paediatric Liver GI and Nutrition Centre New Orleans, LA, USA Boston Children’s’ Hospital King’s College Hospital Boston, MA, USA London, UK Nicola L. Jones, ND, FRCPC, PhD PDKSatreoorpcofak.lri hnYtmosilkgmeaan ,Iet nS los wFft ieCitnudlikteneenitcl al Science and Education DUCToeenrlpilov aBnerirttoomsli,ot eyOgn Noyts f,P ToCroofa rgPnoraanaedmtdaoi, a Htroicssp aitnadl f Pohr ySsiciokl oCghyildren SPHAeafaud’reliiaamt, t PreIiseckr laGMeegael s,d tMircoaDeln Cteernotelorgy Service Downloaded by: Kainan University 203.64.11.45 - 1/25/2015 4:52:43 PM Shimon Reif, MD Anees Siddiqui, MD Prof. Dr. Yvan Vandenplas Director Pediatric Gastroenterology Pediatric Gastroenterology Department of Pediatrics Specially for Children/Dell Children’s UZ Brussel, Vrije Universiteit Brussel Hadassah Medical Center Medical Center Brussels, Belgium Jerusalem, Israel Austin, TX, USA Holly M. Van de Voort, MD Irit Rosen, MD Igor Sukhotnik, MD Centennial Pediatrics Murfreesboro Pediatric Gastroenterology and Nutrition Unit Pediatric Surgery Unit Murfreesboro, TN, USA Rambam Medical Center Bnai Zion Medical Center Haifa, Israel Haifa, Israel Amos Vromen, MD Pediatric Surgery Department Eliana Ruberto, MD Francisco A. Sylvester, MD The Hebrew University Medical School Section of Pediatrics Professor of Pediatrics and Immunology Hadassah Department of Translational Medical Science University of Connecticut School of Medicine Jerusalem, Israel University of Naples Federico II Attending Pediatric Gastroenterologist Naples, Italy Connecticut Children’s Medical Center Barry Wershil, MD Hartford, CT, USA Professor of Pediatrics Marianne Samyn Feinberg School of Medicine at Paediatric Liver, GI and Nutrition Centre Amir Ben Tov, MD Northwestern University King’s College London School of Medicine Gastroenterology Unit Chief, Division of Pediatric Gastroenterology, King’s College Hospital Dana-Dwek Children’s Hospital Hepatology, and Nutrition London, UK Tel Aviv Sourasky Medical Center Ann and Robert H. Lurie Children’s Sackler Faculty of Medicine, Tel Aviv University Hospital of Chicago Cary G. Sauer, MD, MSc Tel Aviv, Israel Chicago, IL, USA Assistant Professor of Pediatrics Emory School of Medicine Dominique Turck, MD Prof. Michael Wilschanski Endoscopy Director Professor of Pediatrics Director, Pediatric Gastroenterology Children’s Healthcare of Atlanta University of Lille Hadassah University Hospital Training Program Director, Pediatric GI Fellowship Lille, France Jerusalem, Israel Emory Children’s Center Atlanta, GA, USA Dan Turner, MD, PhD Tsili Zangen, MD Head, Pediatric Gastroenterology and Pediatric Motility Service Ron Shaoul, MD Nutrition Unit Pediatric Gastroenterology and Nutrition Unit Associate Clinical Professor of Pediatrics Shaare Zedek Medical Center Wolfson Medical Center Director, Pediatric Gastroenterology and The Hebrew University of Jerusalem Holon, Israel Nutrition Unit Jerusalem, Israel Rambam Medical Center Bella Zeisler, MD Haifa, Israel Raphael Uddasin, MD Connecticut Children’s Medical Center Associate Professor of Pediatric Surgery Hartford, CT, USA Head of Pediatric Surgery Department The Hebrew University Medical School Hadassah Jerusalem, Israel V Contributors Downloaded by: Kainan University 203.64.11.45 - 1/25/2015 4:52:43 PM Preface VI This is the fourth volume of the se- troenterologists and nutritionists in given problem. In the process of ries Practical Algorithms in Pediat- the world and edited by my friend writing this book I served as the rics. The previous volumes – Practi- Dr. Ron Shaoul, it is obvious that non-specialist lay reader. Thirty- cal Algorithms in Pediatric Endocri- the spirit of the algorismus has five years after having completed nology (now in its 2nd edition), been utilized to its best. my pediatric residency, I discov- Practical Algorithms in Pediatric Practical Algorithms in Pediatric ered that pediatric gastroenterolo- Hematology-Oncology and Practi- Gastroenterology is meant as a gy has become a sophisticated spe- cal Algorithms in Pediatric Nephrol- pragmatic text to be used at the pa- cialty with a solid scientific back- ogy – have become working tools tient’s bedside. The experienced ground of which I know so little. I for many general pediatricians and practitioner applies step-by-step would still refer my patients to a trainees in the respective pediatric logical problem-solving techniques specialist with many of the diagno- subspecialties. for each patient individually. Deci- ses, symptoms and signs discussed The term ‘algorithm’ is derived sion trees prepared in advance here. But, with the help of this out- from the name of the ninth century have the disadvantage of unac- standing book, I would refer them Arabic mathematician Algawrismi, quaintedness with the individual after an educated initial workup, who also gave his name to ‘alge- patient. Yet, for the physician who and would be better equipped to bra’. His ‘algorismus’ indicated a is less experienced with a given follow the specialist’s manage- step-by-step logical approach to problem, a prepared algorithm pro- ment. mathematical problem-solving. In vides a logical, concise, cost-effec- reading the final product, written tive approach prepared by a spe- Ze’ev Hochberg, MD, PhD by some of the finest pediatric gas- cialist who is experienced with the Series Editor Downloaded by: Kainan University 203.64.11.45 - 1/25/2015 4:52:43 PM Introduction The field of pediatric gastroenterol- gists. There is an increasing need well as a very enriching and gratify- ogy is rapidly expanding, and the from both trainees in pediatric gas- ing experience to interact and work approach to diagnosis and man- troenterology and general pediatri- with everybody. I would like to agement of the various conditions cians for simple, bedside algo- thank the series editor Professor is continuously changing. A better rithms. Practical Algorithms in Pe- Hochberg for his guidance and sup- understanding of the pathogenesis diatric Gastroenterology is meant port and am especially grateful to of many gastrointestinal disorders to be a pragmatic text which classi- Freddy Brian from Karger Publish- has led to a more physiologic ap- fies common clinical symptoms, ers for his patience and his under- proach and the development of bet- signs, laboratory abnormalities and standing in this long process. ter diagnosis and treatment modal- issues of management as present- ities. Pediatric gastroenterology is ed in daily practice. I am honored Ron Shaoul, MD quite unique compared to adult that many of the algorithms in this gastroenterology. We are dealing book have been written by the lead- with developmental disorders, ing experts in the area of pediatric some of which start in utero. The gastroenterology and the surround- I would like to thank my family, issue of growth and development is ing fields. I would like to take this my wife Ety and my children unique for pediatrics, and therefore opportunity to thank all those who Dolev and Shaked for their the approach to the same disease agreed to take part in this book and continuous understanding and condition may be different between contributed their priceless experi- support. adult and pediatric gastroenterolo- ence. It has been my privilege as VII Downloaded by: Kainan University 203.64.11.45 - 1/25/2015 4:52:43 PM Various gastrointestinal conditions R. Arnon · S. Misra Abdominal pain Abdominal pain 2 History and complete physical examination (cid:3) – (cid:4) Presence of alarm symptoms and signs (cid:2) Yes No Directed laboratory studies and imaging (cid:5) Periumbilical abdominal pain Normal limited screening (cid:9) Referral and consultation as indicated (cid:8) No Yes Functional abdominal pain Specific directed treatment (cid:6) Biopsychological treatment (cid:7) Downloaded by: Kainan University 203.64.11.45 - 1/29/2015 7:20:12 PM Chronic abdominal pain, defined as long-lasting intermittent or (cid:10) (cid:5) — In case of alarming symptoms and suspected IBD, direct- Selected reading constant abdominal pain, is a common pediatric problem. It is ed imaging studies include upper GI series and small intestinal Chiou E, Nurko S: Management of functional abdominal pain usually functional, without objective evidence of an underlying follow-through, US or CT of the abdomen. and irritable bowel syndrome in children and adolescents. organic disorder. The exact prevalence of chronic abdominal pain in children is not known. It appears to account for 2–4% of (cid:10) (cid:9) — Clinical evaluation: in functional abdominal pain, a lim- Expert Rev Gastroenterol Hepatol 2010; 4: 293–304. Di Lorenzo C, Colletti RB, Lehmann HP, Boyle JT, Gerson WT, all pediatric office visits. ited and reasonable screening includes a complete blood cell Hyams JS, Squires RH Jr, Walker LS, Kanda PT: Chronic ab- count, erythrocyte sedimentation rate or CRP measurement, (cid:10) (cid:3) — The required duration of symptoms to define chronicity is urinalysis and urine culture. Other biochemical profiles (liver dominal pain in children: a technical report of the American Academy of Pediatrics and the North American Society for 2 months, and the symptoms should occur at least once a week. and kidney) and diagnostic tests (stool culture and examination Pediatric Gastroenterology, Hepatology and Nutrition. J Pedi- for ova and parasites, serology for celiac disease and breath (cid:10) (cid:11) — Patients with chronic abdominal pain commonly com- hydrogen testing for sugar malabsorption) can be performed at atr Gastroenterol Nutr 2005; 40: 249–261. Di Lorenzo C, Colletti RB, Lehmann HP, Boyle JT, Gerson WT, plain of pain in the periumbilical area. the discretion of the clinician, based on the child’s predominant Hyams JS, Squires RH Jr, Walker LS, Kanda PT: Chronic ab- symptoms and degree of functional impairment and parental (cid:10) (cid:12) — Constipation can be a cause of chronic periumbilical ab- anxiety. dominal pain in children: a clinical report of the American Academy of Pediatrics and the North American Society for dominal pain. Functional constipation according to the Rome III committee describes all children with a developmental age of at (cid:10) (cid:8) — Referral to and consultation of a pediatric gastroenterolo- Pediatric Gastroenterology, Hepatology and Nutrition. J Pedi- atr Gastroenterol Nutr 2005; 40: 245–248. least 4 years in whom constipation does not have an organic gist for further evaluation may be indicated. Huertas-Ceballos A, Logan S, Bennett C, Macarthur C: Pharma- etiology and who have insufficient criteria for IBS. (cid:10) (cid:6) — Specific directed treatment for IBD, celiac disease or cological interventions for recurrent abdominal pain (RAP) and (cid:10) (cid:13) — IBS is abdominal discomfort or pain, which improves PUD may be indicated according to the laboratory, imaging or irritable bowel syndrome (IBS) in childhood. Cochrane Data- base Syst Rev 2008; 1:CD003017. with defecation and is associated with a change in the frequen- endoscopic and pathological findings. Rasquin A, Di Lorenzo C, Forbes D, Guiraldes E, Hyams JS, cy and form of the stool without evidence of an inflammatory, anatomic, metabolic or neoplastic process that could explain (cid:10) (cid:7) — A biopsychosocial approach to children with functional Staiano A, Walker LS: Childhood functional gastrointestinal disorders: child/adolescent. Gastroenterology 2006; 130: 1527– these symptoms. abdominal pain is recommended, including cognitive therapy 1537. and hypnosis. Reassurance and explanation of possible mecha- (cid:10) (cid:14) — The clinical history in children with chronic abdominal nisms involving the brain-gut interaction should be given to Shulman RJ, Eakin MN, Jarrett M, Czyzewski DI, Zeltzer LK: Characteristics of pain and stooling in children with recurrent pain should include: family history of abdominal pain, IBS or the child and parents. The possible role of psychosocial factors, abdominal pain. J Pediatr Gastroenterol Nutr 2007; 44: 203–208. IBD, dietary history (excessive sugar intake, lactose intolerance) including triggering events, should be explained. It is often Weydert JA, Ball TM, Davis MF: Systematic review of treat- of the patient and evidence of constipation, diarrhea or growth helpful to summarize the child’s symptoms and explain that, ments for recurrent abdominal pain. Pediatrics 2003; 111:e1– failure. although the pain is real, there is most likely no underlying e11. serious or chronic disease. (cid:10) (cid:4) — The physical examination, including rectal examination, should be complete. A recent Cochrane Database review of the pharmacological in- terventions for RAP and IBS in childhood revealed only weak (cid:10) (cid:2) — Alarm symptoms, signs and features in children and ado- evidence for the efficacy of any pharmacological agent in chil- lescents with nonfunctional abdominal pain include: persistent dren with RAP and recommended the use of drugs only in clini- right upper or right lower quadrant pain, pain that wakes the cal trials. child from sleep, dysphagia, persistent vomiting, arthritis, peri- rectal disease, GI blood loss, involuntary weight loss, nocturnal diarrhea, deceleration of linear growth, unexplained fever or family history of IBD or celiac disease. 3 Various gastrointestinal conditions R. Arnon · S. Misra Abdominal pain Downloaded by: Kainan University 203.64.11.45 - 1/29/2015 7:20:12 PM Various gastrointestinal conditions A. Lo Vecchio · D. Turck · A. Guarino Acute gastroenteritis Acute gastroenteritis(cid:2) 4 Initial assessment Neonates Exclude etiologies other than intestinal infections Food poisoning Surgical conditions Antibiotic toxicity Urinary infection Assessment of dehydration (cid:3) <5% 5–10% >10% None/minimal Mild/moderate Severe Offer ORS ad libitum; encourage normal feeding with no restriction (cid:5) Reassess dehydration after 3–4 h Improved/stable Impaired Hospitalization (cid:7) (cid:129) Weigh child and start fluid balance chart (cid:129) Assessment of electrolytes, bicarbonate, urea and creatinine Consider: blood count, CRP/ESR, stool culture, parasites, urinalysis and urine culture, blood gasses analysis (cid:4) CoPRSnramsoicdbeeecicortai tttdiehcoset rfiolllowing in addition to rehydration (cid:6) Ctoo cnasried efro rfa tmheil ych ailbdilities Strongly consider NG tube ORS (preferred) or i.v. fluids Downloaded by: Kainan University 203.64.11.45 - 1/29/2015 7:20:12 PM