Caroli病的临床病理特征及文献复习

Clinicopathological characteristics of Caroli’s disease and a brief review of the literature

目的:通过5例Caroli病的观察结果探讨和回顾该疾病的临床病理特征。方法:收集5例Caroli病患者临床资料,并通过2位消化病理医生对肝组织进行病理学观察。结果:5例Caroli病患者发病平均年龄为21岁,男女比例为2∶3。临床主要表现为乏力、肝脾大。3例患者伴有门脉高压及先天性多囊肾,3例伴肝功能稍异常。肝组织学特征为汇管区周围纤维化,大量胆管上皮增生,管腔囊状扩张,胆管内胆汁淤积,炎细胞浸润,肝组织内可形成假小叶结构,但多数肝细胞排列基本正常。结论:Caroli病以青少年多见,组织学上以汇管区纤维化、胆管增生扩张为主要特点,结合临床资料是诊断的主要依据。

Objective:To review five cases of Caroli’s disease and to generalize the clinical characteristics of the disease.Methods:The clinicopathological characteristics of five patients with Caroli’s disease was analyzed,and the histological features of hepatic tissue were observed by two gastrointestinal pathologists.Results:The mean age of the five patients was 21 years,and the ratio of male to female was 2∶3.The main clinical symptoms and signs were malaise and hepatosplenomegaly.Three of the five patients were complicated with portal hypertension,two cases complicated with renal cyst and three cases showed abnormal liver function.The main histological features were portal fibrosis,ductular proliferation,dilatation of intrahepatic bile ducts,cholestasis and inflammatory infiltration.Pseudolobule formation may be observed,but the array of the hepatic plates appears normal.Conclusion:Caroli’s disease mainly occurs in adolescent.The diagnosis of the disease is mainly based on periportal fibrosis,dilatation of intrahepatic bile ducts,and clinical information.