Summary
Definition
History and exam
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
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
Treatment algorithm
Contributors
Authors
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
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.
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