Biliary atresia

Biliary atresia, also known as "extrahepatic ductopenia" and "progressive obliterative cholangiopathy" is a congenital or acquired disease of the liver and one of the principal forms of chronic rejection of a transplanted liver allograft. As a birth defect in newborn infants, it has an occurrence of 1/10,000 to 1/15,000 cases in live births in the United States. In the congenital form, the common bile duct between the liver and the small intestine is blocked or absent. The acquired type most often occurs in the setting of autoimmune disease, and is one of the principal forms of chronic rejection of a transplanted liver allograft. Infants and children with biliary atresia have progressive cholestasis with all the usual concomitant...