Highlights & Basics
- Biliary atresia is a serious disease requiring prompt early diagnosis to optimize treatment outcomes. Treatment should ideally occur before 30-45 days of life.
- A fibro-obliterative obstruction of the extrahepatic biliary tree progressing to intrahepatic ducts, which can develop in utero or during the neonatal period.
- Presentation may include jaundice, pale stools, or hepatomegaly.
- Biliary atresia should be suspected in any baby with jaundice and pale stools, jaundice or pale stools persisting beyond 14 days of age, or if the direct or conjugated bilirubin is ≥1 mg/dL.
- Hepatoportoenterostomy is warranted in infants without evidence of end-stage liver disease, but many go on to require liver transplantation within a few years.
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Harpavat S, Aucott SW, Karpen SJ, et al. Guidance for the primary care provider in identifying infants with biliary atresia by 2-4 weeks of life: clinical report. 2025 Feb 18:e2024070077.[Abstract][Full Text]
Fawaz R, Baumann U, Ekong U, et al. Guideline for the evaluation of cholestatic jaundice in infants: joint recommendations of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition. J Pediatr Gastroenterol Nutr. 2017 Jan;64(1):154-68.[Abstract][Full Text]
Squires RH, Ng V, Romero R, et al. Evaluation of the pediatric patient for liver transplantation: 2014 practice guideline by the American Association for the Study of Liver Diseases, American Society of Transplantation and the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. Hepatology. 2014;60:362-98.[Full Text]
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