先天性肝纤维化1例

Congenital hepatic fibrosis: one case report

先天性肝纤维化(congenital hepatic fibrosis,CHF)是较为罕见的常染色体隐性遗传病,儿童多见,病程后期一般会导致门静脉高压,临床上常与一般门脉性肝硬化鉴别不清,常需要组织病理学明确诊断。现将第四军医大学第一附属医院消化科收治的1例CHF患者报道如下。

Congenital hepatic fibrosis （CHF） is a rare autosomal invisible hereditary disease, more common in children, late course will generally lead to portal hypertension. The identification with general portal cirrhosis in clinically is not clear, the diagnosis of CHF is usually confirmed by histopathology. In this paper, one case of CHF was reported in the Department of Gastroenterology of Xijing Hospital.