biliary atresia
Pronunciation: BIL-ee-air-ee uh-TREE-zhuh
Congenital condition where bile ducts are absent or blocked, causing progressive liver damage in infants.
Full Definition
Biliary atresia is a serious neonatal condition characterized by the absence, destruction, or obstruction of the extrahepatic bile ducts, leading to cholestasis and progressive liver fibrosis. It typically presents in the first few weeks of life with jaundice, acholic stools, and hepatomegaly. Early diagnosis is crucial as the Kasai portoenterostomy procedure is most effective when performed before 60 days of age. Without treatment, the condition progresses to cirrhosis and liver failure.
Usage
Usage note: Always specify 'biliary atresia' rather than just 'atresia' to avoid confusion with other atretic conditions.
In Context
- "The persistent jaundice and acholic stools raised suspicion for biliary atresia requiring urgent hepatobiliary evaluation." — Pediatric gastroenterology consult
- "Early referral for Kasai procedure is essential in suspected biliary atresia cases." — Clinical guideline documentation