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)

Rima Fawaz, Ulrich Baumann, Udeme Ekong, Björn Fischler, Nedim Hadzic, Cara L L Mack, Valérie A. McLin, Jean Molleston, Ezequiel Neimark, Vicky Lee Ng, Saul J. Karpen

Research output: Contribution to journalArticle

58 Citations (Scopus)

Abstract

ABSTRACT: 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 (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.

Original languageEnglish (US)
JournalJournal of Pediatric Gastroenterology and Nutrition
DOIs
StateAccepted/In press - Jul 16 2016

Fingerprint

Obstructive Jaundice
Gastroenterology
Cholestasis
Joints
Bilirubin
Guidelines
Pediatrics
Jaundice
Referral and Consultation
Infant Care
Biliary Atresia
Parenteral Nutrition
Primary Care Physicians
Serum
DNA Sequence Analysis
Practice Guidelines
Liver Diseases
Technology
Gastroenterologists
Therapeutics

ASJC Scopus subject areas

  • Gastroenterology
  • Pediatrics, Perinatology, and Child Health

Cite this

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). / Fawaz, Rima; Baumann, Ulrich; Ekong, Udeme; Fischler, Björn; Hadzic, Nedim; Mack, Cara L L; McLin, Valérie A.; Molleston, Jean; Neimark, Ezequiel; Ng, Vicky Lee; Karpen, Saul J.

In: Journal of Pediatric Gastroenterology and Nutrition, 16.07.2016.

Research output: Contribution to journalArticle

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abstract = "ABSTRACT: 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 (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.",
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AU - Fawaz, Rima

AU - Baumann, Ulrich

AU - Ekong, Udeme

AU - Fischler, Björn

AU - Hadzic, Nedim

AU - Mack, Cara L L

AU - McLin, Valérie A.

AU - Molleston, Jean

AU - Neimark, Ezequiel

AU - Ng, Vicky Lee

AU - Karpen, Saul J.

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N2 - ABSTRACT: 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 (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.

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