Biliary Atresia

Biliary atresia, also known as extrahepatic ductopenia and progressive obliterative cholangiopathy, is a childhood disease of the liver in which one or more bile ducts are abnormally narrow, blocked, or absent, and it is the most common reason for pediatric liver transplantation in the United States. It can be congenital or acquired, usually presents in infancy with jaundice, pale stool, and dark urine, and can lead to cirrhosis, portal hypertension, and liver failure if untreated. The condition occurs in about 1 in 10,000 to 15,000 live births in the United States and is markedly more common in East Asia, at roughly 1 in 5,000 births; surgery and liver transplantation are the only effective treatments.