先天性肝纤维化7例临床病理分析

Congenital hepatic fibrosis: a clinicopathologic study of 7 cases

目的 探讨先天性肝纤维化 (CHF)的临床病理特征。方法 对 7例CHF病例进行临床资料分析及病理组织学观察。结果 7例CHF患者的平均年龄为 9 5岁 ,男女之比为 2∶5 ,7例均有门脉高压症状 ,4例肝功能轻度异常。肝组织学特征为粗细不等的纤维条索或纤维间隔穿插、包绕排列尚有序大致正常的肝实质 ,纤维间隔内胶原较致密 ;3例伴有明显的小胆管增生、扩张和畸形 ,其中 1例呈典型的Caroli病改变。结论 CHF的诊断要点为有门脉高压症状的年幼患者 ;较致密的纤维间隔穿插、包绕大致正常的肝实质 ,但不形成典型的假小叶 ;可伴有小胆管增生。

Objective To explore the clinical and pathological features of congenital hepatic fibrosis (CHF) Methods The clinical data and needle biopsied liver tissues were analyzed and observed in 7 cases of CHF Results The average age of 7 patients with CHF was 9 5 years , and the ratio of male to female was 2 to 5 All the patients with CHF presented the symptoms of portal hypertension The main histopathologic features of CHF showed that hepatocytes were encompassed or intruded by the fibrotic strips or septa, and most of the hepatic plates were still arrayed by one line hepatocytes The fibrotic septa were mainly composed of compact collogens. Meanwhile, aberrant, dilated and proliferated bile ducts were observed in 3 cases of CHF, and 1 case appeared typical changes of Caroli's syndrome Conclusions The features of clinicopathology of CHF include:symptoms of portal hypertension were presented during childhood;the inherent hepatocytes were encompassed or intruded by the fibrotic strips or densely collagenized septa, but no pseudolobule being formed;CHF may be associated with proliferation, dilation and malformation of bile ducts