成年人先天性肝纤维化1例报道
Chinese Journal of Gastroenterology and Hepatology(2010)
Abstract
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。
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Key words
Congenital hepatic fibrosis,Adult
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