biliary atresia
A progressive inflammatory condition causing obliteration of extrahepatic bile ducts in infants, leading to cholestasis and liver dysfunction.
Full Definition
Biliary atresia is a rare pediatric condition characterized by progressive fibrotic obliteration of the extrahepatic biliary tree, resulting in conjugated hyperbilirubinemia and eventual cirrhosis. The condition typically presents in the first few months of life with jaundice, acholic stools, and hepatomegaly. Early diagnosis is crucial as the Kasai portoenterostomy procedure is most effective when performed before 60-90 days of age. Without intervention, the condition uniformly progresses to end-stage liver disease requiring transplantation.
Usage
Usage note: Critical to distinguish from other causes of neonatal cholestasis for timely intervention.
In Context
- "The infant's persistent jaundice and acholic stools prompted evaluation for biliary atresia." — Pediatric gastroenterology consultation
- "HIDA scan showed no excretion into the small bowel, supporting the diagnosis of biliary atresia." — Nuclear medicine report