中华实用儿科临床杂志
中華實用兒科臨床雜誌
중화실용인과림상잡지
Journal of Applied Clinical Pediatrics
2015年
19期
1516-1518
,共3页
胆道闭锁%病因%发病机制
膽道閉鎖%病因%髮病機製
담도폐쇄%병인%발병궤제
Biliary atresia%Etiology%Mechanism
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。
膽道閉鎖是髮生于齣生3箇月內嬰幼兒的疾病,其特徵為肝內外膽管纖維化閉鎖。儘管肝門空腸吻閤術能部分緩解癥狀,但仍有80%左右的患兒需行肝移植治療。目前關于膽道閉鎖的病因及髮病機製併未完全闡明,現主要研究集中于分子生物學因素、胚胎學因素、病毒感染/免疫應答因素。
담도폐쇄시발생우출생3개월내영유인적질병,기특정위간내외담관섬유화폐쇄。진관간문공장문합술능부분완해증상,단잉유80%좌우적환인수행간이식치료。목전관우담도폐쇄적병인급발병궤제병미완전천명,현주요연구집중우분자생물학인소、배태학인소、병독감염/면역응답인소。
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.