Highlights & Basics
- Biliary atresia is a serious disease requiring prompt early diagnosis preferably before age 6-8 weeks.
- 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 persisting beyond 14 days of age, or if the direct or conjugated bilirubin is >1 mg/dL.
- Hepatoportoenterostomy is warranted in infants aged <100 days of age and without evidence of end-stage liver disease, but many go on to require liver transplantation within a few years.
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Definition
Epidemiology
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Citations
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;64:154-68.[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.[Abstract][Full Text]
Martin P, DiMartini A, Feng S, et al; AASLD. Evaluation for liver transplantation in adults: 2013 practice guideline by the American Association for the Study of Liver Diseases and the American Society of Transplantation. Hepatology. 2014;59:1144-65.[Abstract][Full Text]
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