Guideline for the Evaluation of Cholestatic Jaundice in Infants: Joint Recommendations of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition (NASPGHAN) and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN)

ABSTRACTCholestatic jaundice in infancy affects approximately 1 in every 2500 term infants and is infrequently recognized by primary providers in the setting of physiologic jaundice. Cholestatic jaundice is always pathologic and indicates hepatobiliary dysfunction. Early detection by the primary car...

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Published inJournal of pediatric gastroenterology and nutrition Vol. 64; no. 1; pp. 154 - 168
Main Authors Fawaz, Rima, Baumann, Ulrich, Ekong, Udeme, Fischler, Björn, Hadzic, Nedim, Mack, Cara L, McLin, Valérie A, Molleston, Jean P, Neimark, Ezequiel, Ng, Vicky Lee, Karpen, Saul J
Format Journal Article
LanguageEnglish
Published United States by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology 01.01.2017
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Abstract ABSTRACTCholestatic jaundice in infancy affects approximately 1 in every 2500 term infants and is infrequently recognized by primary providers in the setting of physiologic jaundice. Cholestatic jaundice is always pathologic and indicates hepatobiliary dysfunction. Early detection by the primary care physician and timely referrals to the pediatric gastroenterologist/hepatologist are important contributors to optimal treatment and prognosis. The most common causes of cholestatic jaundice in the first months of life are biliary atresia (BA, 25–40%) followed by an expanding list of monogenic disorders (25%), plus many unknown or multifactorial (e.g., parenteral nutrition related) causes, each of which may have time-sensitive and distinct treatment plans. Thus, these Guidelines can have an essential role for the evaluation of neonatal cholestasis to optimize care. The recommendations from this clinical practice guideline are based upon review and analysis of published literature as well as the combined experience of the authors. The Committee recommends that any infant noted to be jaundiced after 2 weeks of age be evaluated for cholestasis with measurement of total and direct serum bilirubin, and that an elevated serum direct bilirubin level (direct bilirubin levels >1.0 mg/dl or >17 μmol/L) warrants timely consideration for evaluation and referral to a pediatric gastroenterologist or hepatologist. Of note, current differential diagnostic plans now incorporate consideration of modern broad-based next generation DNA sequencing technologies in the proper clinical context. These recommendations are a general guideline and are not intended as a substitute for clinical judgment or as a protocol for the care of all infants with cholestasis. Broad implementation of these recommendations is expected to reduce the time to the diagnosis of pediatric liver diseases, including BA, leading to improved outcomes.
AbstractList Cholestatic jaundice in infancy affects approximately 1 in every 2500 term infants and is infrequently recognized by primary providers in the setting of physiologic jaundice. Cholestatic jaundice is always pathologic and indicates hepatobiliary dysfunction. Early detection by the primary care physician and timely referrals to the pediatric gastroenterologist/hepatologist are important contributors to optimal treatment and prognosis. The most common causes of cholestatic jaundice in the first months of life are biliary atresia (25%-40%) followed by an expanding list of monogenic disorders (25%), along with many unknown or multifactorial (eg, parenteral nutrition-related) causes, each of which may have time-sensitive and distinct treatment plans. Thus, these guidelines can have an essential role for the evaluation of neonatal cholestasis to optimize care. The recommendations from this clinical practice guideline are based upon review and analysis of published literature and the combined experience of the authors. The committee recommends that any infant noted to be jaundiced after 2 weeks of age be evaluated for cholestasis with measurement of total and direct serum bilirubin, and that an elevated serum direct bilirubin level (direct bilirubin levels >1.0 mg/dL or >17 μmol/L) warrants timely consideration for evaluation and referral to a pediatric gastroenterologist or hepatologist. Of note, current differential diagnostic plans now incorporate consideration of modern broad-based next-generation DNA sequencing technologies in the proper clinical context. These recommendations are a general guideline and are not intended as a substitute for clinical judgment or as a protocol for the care of all infants with cholestasis. Broad implementation of these recommendations is expected to reduce the time to the diagnosis of pediatric liver diseases, including biliary atresia, leading to improved outcomes.
ABSTRACTCholestatic jaundice in infancy affects approximately 1 in every 2500 term infants and is infrequently recognized by primary providers in the setting of physiologic jaundice. Cholestatic jaundice is always pathologic and indicates hepatobiliary dysfunction. Early detection by the primary care physician and timely referrals to the pediatric gastroenterologist/hepatologist are important contributors to optimal treatment and prognosis. The most common causes of cholestatic jaundice in the first months of life are biliary atresia (BA, 25–40%) followed by an expanding list of monogenic disorders (25%), plus many unknown or multifactorial (e.g., parenteral nutrition related) causes, each of which may have time-sensitive and distinct treatment plans. Thus, these Guidelines can have an essential role for the evaluation of neonatal cholestasis to optimize care. The recommendations from this clinical practice guideline are based upon review and analysis of published literature as well as the combined experience of the authors. The Committee recommends that any infant noted to be jaundiced after 2 weeks of age be evaluated for cholestasis with measurement of total and direct serum bilirubin, and that an elevated serum direct bilirubin level (direct bilirubin levels >1.0 mg/dl or >17 μmol/L) warrants timely consideration for evaluation and referral to a pediatric gastroenterologist or hepatologist. Of note, current differential diagnostic plans now incorporate consideration of modern broad-based next generation DNA sequencing technologies in the proper clinical context. These recommendations are a general guideline and are not intended as a substitute for clinical judgment or as a protocol for the care of all infants with cholestasis. Broad implementation of these recommendations is expected to reduce the time to the diagnosis of pediatric liver diseases, including BA, leading to improved outcomes.
Cholestatic jaundice in infancy affects approximately 1 in every 2500 term infants and is infrequently recognized by primary providers in the setting of physiologic jaundice. Cholestatic jaundice is always pathologic and indicates hepatobiliary dysfunction. Early detection by the primary care physician and timely referrals to the pediatric gastroenterologist/hepatologist are important contributors to optimal treatment and prognosis. The most common causes of cholestatic jaundice in the first months of life are biliary atresia (25%-40%) followed by an expanding list of monogenic disorders (25%), along with many unknown or multifactorial (eg, parenteral nutrition-related) causes, each of which may have time-sensitive and distinct treatment plans. Thus, these guidelines can have an essential role for the evaluation of neonatal cholestasis to optimize care. The recommendations from this clinical practice guideline are based upon review and analysis of published literature and the combined experience of the authors. The committee recommends that any infant noted to be jaundiced after 2 weeks of age be evaluated for cholestasis with measurement of total and direct serum bilirubin, and that an elevated serum direct bilirubin level (direct bilirubin levels >1.0 mg/dL or >17 μmol/L) warrants timely consideration for evaluation and referral to a pediatric gastroenterologist or hepatologist. Of note, current differential diagnostic plans now incorporate consideration of modern broad-based next-generation DNA sequencing technologies in the proper clinical context. These recommendations are a general guideline and are not intended as a substitute for clinical judgment or as a protocol for the care of all infants with cholestasis. Broad implementation of these recommendations is expected to reduce the time to the diagnosis of pediatric liver diseases, including biliary atresia, leading to improved outcomes.
Author Fischler, Björn
Fawaz, Rima
Ekong, Udeme
Karpen, Saul J
Hadzic, Nedim
Molleston, Jean P
Mack, Cara L
McLin, Valérie A
Baumann, Ulrich
Neimark, Ezequiel
Ng, Vicky Lee
AuthorAffiliation Division of Gastroenterology, Hepatology and Nutrition, Boston Childrenʼs Hospital, Boston, MA 02115, Harvard Medical School †Division of Paediatric Gastroenterology and Hepatology, Department of Paediatric Kidney, Liver and Metabolic Diseases, Hannover Medical School, Hannover, Germany ‡Yale University School of Medicine, Yale New Haven Hospital Transplantation Center, 333 Cedar Street; LMP 4093, New Haven, CT 06520 §Department of Pediatrics, Karolinska University Hospital, CLINTEC, Karolinska Institute, SE-141 86 Stockholm, Sweden ||Paediatric Centre for Hepatology, Gastroenterology and Nutrition Kingʼs College Hospital, Denmark Hill, London SE5 9RS, UK ¶Section of Pediatric Gastroenterology, Hepatology and Nutrition, Childrenʼs Hospital Colorado, University of Colorado School of Medicine, 13123 E. 16th Ave., B290, Aurora, CO 80045 #Swiss Center for Liver Disease in Children, University Hospitals Geneva, Geneva, Switzerland Indiana University School of Medicine/Riley Hospital for Chi
AuthorAffiliation_xml – name: Division of Gastroenterology, Hepatology and Nutrition, Boston Childrenʼs Hospital, Boston, MA 02115, Harvard Medical School †Division of Paediatric Gastroenterology and Hepatology, Department of Paediatric Kidney, Liver and Metabolic Diseases, Hannover Medical School, Hannover, Germany ‡Yale University School of Medicine, Yale New Haven Hospital Transplantation Center, 333 Cedar Street; LMP 4093, New Haven, CT 06520 §Department of Pediatrics, Karolinska University Hospital, CLINTEC, Karolinska Institute, SE-141 86 Stockholm, Sweden ||Paediatric Centre for Hepatology, Gastroenterology and Nutrition Kingʼs College Hospital, Denmark Hill, London SE5 9RS, UK ¶Section of Pediatric Gastroenterology, Hepatology and Nutrition, Childrenʼs Hospital Colorado, University of Colorado School of Medicine, 13123 E. 16th Ave., B290, Aurora, CO 80045 #Swiss Center for Liver Disease in Children, University Hospitals Geneva, Geneva, Switzerland Indiana University School of Medicine/Riley Hospital for Children, 705 Riley Hospital Drive, ROC 4210, Indianapolis, IN 46202 ††Division of Pediatric Gastroenterology, Hepatology and Nutrition, Hasbro Childrenʼs Hospital, The Warren Alpert School of Medicine at Brown University, Providence, RI ‡‡Division of Pediatric Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children, University of Toronto, Toronto, Canada §§Department of Pediatrics, Emory University School of Medicine/Childrenʼs Healthcare of Atlanta, 1760 Haygood Dr. HSRB E204, Atlanta, Georgia 30322
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  fullname: McLin, Valérie A
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PublicationTitle Journal of pediatric gastroenterology and nutrition
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Snippet ABSTRACTCholestatic jaundice in infancy affects approximately 1 in every 2500 term infants and is infrequently recognized by primary providers in the setting...
Cholestatic jaundice in infancy affects approximately 1 in every 2500 term infants and is infrequently recognized by primary providers in the setting of...
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SubjectTerms Biliary Atresia - complications
Biliary Tract
Bilirubin - blood
Cholestasis - blood
Cholestasis - diagnosis
Cholestasis - etiology
Cholestasis - pathology
Diagnosis, Differential
Europe
Gastroenterology
Humans
Hyperbilirubinemia - blood
Hyperbilirubinemia - diagnosis
Hyperbilirubinemia - etiology
Infant
Infant, Newborn
Jaundice - diagnosis
Jaundice - etiology
Jaundice, Obstructive - blood
Jaundice, Obstructive - diagnosis
Jaundice, Obstructive - etiology
Liver
Liver Diseases - blood
Liver Diseases - diagnosis
Liver Diseases - pathology
Medicin och hälsovetenskap
North America
Pediatrics
Societies
Title Guideline for the Evaluation of Cholestatic Jaundice in Infants: Joint Recommendations of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition (NASPGHAN) and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN)
URI https://www.ncbi.nlm.nih.gov/pubmed/27429428
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