成年人先天性肝纤维化1例报道  

A case report of adult congenital hepatic fibrosis

作　　者：乙国兴[1] 崔梅花[1] 傅怡[1] 杨桂彬[1] 

机构地区：[1]北京大学航天中心医院消化科,北京100049

出　　处：《胃肠病学和肝病学杂志》2010年第8期761-761,765,共2页Chinese Journal of Gastroenterology and Hepatology

摘　　要：先天性肝纤维化是一种常染色体隐性遗传病,绝大部分在儿童时期发病,成年发病少见。临床表现为门脉高压症,而肝功能基本正常,多伴有肾脏病变及Caroli’s病,明确诊断通常需要典型的组织病理学表现。本文报道1例成年发病的先天性肝纤维化病例。Congenital hepatic fibrosis（CHF） is an autosomal recessive disease,presenting principally in children with portal hypertension and normal liver function.It is associated with renal malformation and Caroli＇s disease.The diagnosis of CHF is usually confirmed by its typical histological features.A 26-year-old woman with congenital hepatic fibrosis was reported in this paper.

关 键 词：先天性肝纤维化 成年 

分 类 号：R575[医药卫生—消化系统]