先天性肝纤维化24例临床分析并文献复习

目的分析先天性肝纤维化的临床特征。方法对24例先天性肝纤维化患者临床症状、体征、实验室化验检查及病理学特点进行回顾性分析。结果 24例患者均有门脉高压表现,8例曾有消化道出血。化验肝功能正常或轻度异常。影像学检查提示7例存在肝肾囊肿。22例病理结果均提示先天性肝纤维化,其中9例合并先天性肝内胆管扩张（Caroli’s）病。结论对于病因不明门脉高压,尤其门脉高压与肝功损害程度不一致的患者应尽量行肝组织活检病理检查以协助诊断。先天性肝纤维化与肝肾囊肿、Caroli’s病常常伴发。

Clinical analysis of 24 cases of congenital hepatic fibrosis

Objective To analysis the clinical features of congenital hepatic fibrosis（CHF）.Methods The clinical features,physical examination,laboratory data and pathological features were retrospectively reviewed in 24 cases of CHF patients.Results All 24 patients showed the symptoms of portal hypertension 8 patients have had variceal source of upper gastrointestinal bleeding.Laboratory workup reveal normal or mild elevation in liver enzymes.7 patients have liver or kidney cysts.22 patients were diagnosed CHF through pathological examination.Conclusion The patients with portal hypertension of unknown reasons should exclude CHF by liver pathological examination,especially in patients which degree of portal hypertension and liver damage is inconsistent.CHF is often associated with kidney cysts and Caroli＇s disease.