胆道闭锁病因及其发病机制研究进展

Recent advances of the etiology and mechanism of biliary atresia

胆道闭锁是发生于出生3个月内婴幼儿的疾病，其特征为肝内外胆管纤维化闭锁。尽管肝门空肠吻合术能部分缓解症状，但仍有80%左右的患儿需行肝移植治疗。目前关于胆道闭锁的病因及发病机制并未完全阐明，现主要研究集中于分子生物学因素、胚胎学因素、病毒感染/免疫应答因素。

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.