先天性肝纤维化——附12例临床分析

目的分析先天性肝纤维化的临床特征。方法对12例先天性肝纤维化患者临床表现、实验室检查及病理组织学特点进行回顾性分析。结果12例患者均有明显临床症状，10例有典型门脉高压症状，11例就诊时肝功能异常，肝脏病理主要表现为：肝组织内呈现宽大致密且炎症不明显的胶原纤维间隔，或纤维束弥漫穿插于固有的肝小叶内，无典型的假小叶结构。结论对于不明原因肝功能异常的门脉高压症患者，应尽可能进行肝组织活检病理检查以协助诊断及指导治疗。

Clinical analysis of 12 cases of congenital hepatic fibrosis and literature review

Objective To analyze the clinical features of congenital hepatic fibrosis（CHF）. Methods The clinical feature, laboratory data and pathological feature were retrospectively reviewed in 12 cases of CHF patients. Results All twelve CHF patients showed obvious clinical symptoms, ten patients presented the symptoms of portal hypertension and liver function test were abnormal in eleven patients. The main histopathologic features of CHF showed that hepatocytes were encompassed or intruded by the densely and non inflammation collagenized septa, or hepatic plates were arrayed by fiber bundles. No typical pseudolobule structure was found. Conclusion The patients with unknown causes portal hypertension and abnormal liver function should exclude congenital hepatic fibrosis, liver biopsy with pathologic exanfination should be carried out.