先天性肝纤维化48例临床特点分析

The clinical features analysis of 48 cases of congenital hepatic fibrosis

目的了解先天性肝纤维化的临床病理特征。方法回顾性分析48例经病理明确诊断为先天性肝纤维化的患者临床资料,总结其临床症状、体征、实验室检查特点及肝活组织病理检查结果。结果 48例患者均有门静脉高压表现,10例曾有消化道出血,13例行脾脏切除术,40例肝功能正常或轻度异常。影像学检查提示10例存在肝肾囊肿。48例病理结果均提示先天性肝纤维化,其中11例合并先天性肝内胆管扩张(Calori’s)病。结论对于病因不明肝硬化、门静脉高压与肝功能损害程度不相符的患者,应考虑先天性肝纤维化的诊断。

Objective To analyze the clinical features of congenital hepatic fibrosis（CHF）. Methods The clinical features, including symptoms, signs, laboratory data and pathological features, were retrospectively reviewed in 48 patients with CHF. Results All of 48 patients showed the symptoms of portal hypertension, of which 10 cases had variceal source of upper gastrointestinal bleeding, 13 underwent splenectomy, 40 had normal or mild abnormal liver function, and 10 had kidney cyst. All of 48 cases were diagnosed as CHF by liver pathological examination, of which 11 had congenital intrahepatic bile ducts dilation （Calori＇ s） disease concomitantly. Conclusion CHF should be considered in the patients with unknown etiological cirrhosis, and portal hypertension being inconsistent with the degree of liver damage.