摘要
胆道闭锁是危及婴幼儿生命的肝胆系统疾病，以肝内外胆管进行性炎症和纤维化为特征，导致胆汁淤积以及进行性肝纤维化和肝硬化，最终进展为肝功能衰竭，是婴幼儿时期肝移植的主要指征。Kasai手术的广泛开展，使胆道闭锁患儿获得更多的生存机会，但术后60％的患儿1年内需行肝移植，只有25％的患儿自体肝存活至10岁。证据表明上皮间质转化在胆管纤维化中扮演着非常重要的角色，并且TGF-β1表达增强与胆道闭锁发生具有一定的相关性；而肝细胞生长因子（HGF）可明显抑制纤维化促进因子TGF-β1，的表达，从而抑制组织纤维化。本文就胆道闭锁可能的发病机制、上皮间质转化在组织纤维化中的作用、针对上皮间质转化的抗纤维化治疗以及胆道闭锁治疗方法的展望作一综述。
As a life-threatening hepatobiliary disease in infants, biliary atresia （BA） is characterized by intrahepatic and extrahepatic bile duct inflammation and fibrosis leading to cholestasis, hepatic fibrosis, cirrhosis and even hepatic failure. It is a major indication for hepatic transplantation. With extensive refining of Kasai surgery, BA children have obtained more chances of survival. However, 60% of them required hepatic transplantation after 1 year and only 25% could survive for 10 years. Epithelial mesenchymal transition （EMT） is probably involved in the development of bile duct fibrosis and transforming growth factor-131 （TGF-beta 1） has been recognized as an inducer of EMT. And hepatocyte growth factor （HGF） is able to antagonize TGF-beta 1- mediated fibrosis. This review examined the possible pathogenesis of BA, the role of EMT in the development of fibrosis, anti-fibrotic treatment of EMT and therapeutic prospects of BA.
出处
《中华小儿外科杂志》
CSCD
2017年第8期631-635,共5页
Chinese Journal of Pediatric Surgery
基金
黑龙江省博士后科研启动基金（LBH-Q16154）
大学生创新创业训练计划项目（201610226004）
关键词
胆道闭锁
上皮间质转化
转化生长因子Β
肝细胞生长因子
Biliary atresia
Epithelia mesenchyrnal transition
Transforming growth factorbeta
Hepatocyte growth factor
