摘要
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.
出处
《中华实用儿科临床杂志》
CAS
CSCD
北大核心
2015年第19期1516-1518,共3页
Chinese Journal of Applied Clinical Pediatrics
基金
国家自然科学基金(81070284,81401240)
关键词
胆道闭锁
病因
发病机制
Biliary atresia
Etiology
Mechanism