Last reviewed: November 2017
Last updated: November  2017

Summary

Definition

History and exam

Key diagnostic factors

  • neonatal jaundice
  • acholic stool

Other diagnostic factors

  • dark urine
  • bruising
  • hepatomegaly
  • ascites

Risk factors

  • genetic predisposition
  • immune dysregulation
  • viral infection
  • environmental or behavioural exposures
  • maternal DM

Diagnostic investigations

1st investigations to order

  • serum total and conjugated bilirubin
  • newborn screen (includes tests for galactosaemia, thyroid dysfunction, cystic fibrosis, and a variety of metabolic diseases)
  • prothrombin time (PT), INR
  • FBC with differential
  • serum AST, ALT, ALP, and gamma-GT
  • abdominal ultrasound
Full details

Investigations to consider

  • hepatobiliary scintigraphy (technetium Tc 99m-di-isopropyl-acetanilido-imino-diacetic acid scan)
  • liver biopsy
  • cholangiogram
  • ERCP
  • CXR
  • infection screen: blood and urine cultures +/- spinal fluid testing
  • urine PCR for CMV
  • plasma or serum amino acids
  • alpha-1 level and protease inhibitor (Pi) type
  • random serum cortisol
  • urinary organic acids
  • urinary succinyl acetone
  • urinary bile acids
  • serum lactate/pyruvate ratio
Full details

Treatment algorithm

Contributors

Authors VIEW ALL

Coordinator

Biliary Atresia Research Program

The Children's Hospital of Philadelphia

Philadelphia

PA

Disclosures

JE receives payment for contributions to UpToDate.

Professor of Pediatrics

Department of Pediatrics

The Children's Hospital of Philadelphia

Philadelphia

PA

Disclosures

JAF declares that he has no competing interests.

Associate Professor of Pediatrics

Department of Pediatrics

The Children's Hospital of Philadelphia

Philadelphia

PA

Disclosures

KL receives payment for contributions to UpToDate and has received an NIH grant to study biliary atresia.

Dr Jessi Erlichman, Dr Jonathan A. Flick, and Dr Kathleen Loomes would like to gratefully acknowledge Dr Barbara A. Haber, a previous contributor to this monograph. This monograph was first commissioned and written in 2008 when Dr Haber was Professor of Pediatrics at The Children's Hospital of Philadelphia. Dr Haber moved to a position at Merck Sharp & Dohme in 2012.

Peer reviewers VIEW ALL

Director

Pediatric Hepatology Center

Visiting Professor of Pediatrics

University of Pittsburgh

Pediatric Hepatology Center

Pittsburgh

PA

Disclosures

BLS has received research funding from, and been a consultant to, several pharmaceutical companies that make antibiotic agents that might be used for treating bacterial prostatitis.

Former Professor of Paediatric Surgery

University of Otago

Dunedin

New Zealand

Disclosures

MDS declares that he has no competing interests.

Use of this content is subject to our disclaimer