Recent advances of the etiology and mechanism of biliary atresia / 中华实用儿科临床杂志

ABSTRACT

Biliary atresia ( BA) is a pediatric liver disease characterized by progressive inflammation and fi-brosis of both the extrahepatic and intrahepatic bile ducts. Even though Kasai portoenterostomy increases the survival of children with BA, 80% of patients with BA will eventually require liver transplantation, making this condition the leading indication for pediatric liver transplantation worldwide. Currently,the causes of this disease are largely unde-fined and theories of pathogenesis include viral infection,autoimmune-mediated bile duct destruction,and abnormalities in bile duct development.