解放军医学杂志2011，Vol.36Issue(1)：86-88,3.
先天性肝纤维化伴Caroli病临床病理分析
Congenital hepatic fibrosis with Caroli's disease: a iclmicopathological analysis of 6 cases
熊璐 1赵景民 1周光德 1郭晓东 1梁丽 1赵雨来 1李文淑 2刘树红 1韦立新3
作者信息
- 1. 100039,北京,解放军302医院病理诊断与研究中心
- 2. 100039,北京,解放军302医院新药临床实验中心
- 3. 100853,北京,解放军总医院病理科
- 折叠
摘要
Abstract
Objective To explore the clinical and pathological features of congenital hepatic fibrosis with Carolis disease. Methods The clinical characteristics of 6 patients with congenital hepatic fibrosis and Carolis disease were analyzed, and the histolngicsl features of hepatic tissue were observed by using hematoxylin and eosin staining and histochemical staining. Results The mean age of the 6 patients was 7. 8 years, and the sex ratio (male to female) was 2 : 4. The main clinical manifestations were hematemeais (5/6), hypodynamia (5/6) and hepatosplenormegaly (5/6). Three of the 6 patients were complicated with portal hypertension, 2 cases complicated with diffuse parenchyma lesion in the double kidneys, and 1 case showed abnormal hepatic function. The main histological features were fibrosis of periportal area, epithelial tissue proliferation of interfibrous bile ducts, capsular ectssis of intrahepatic bile ducts, cholestssis and inflammatory infiltration. Pseudolobule formation in hepatic tissue may observed, but most of the hepatic plates arranged still in order.Conclusions Congenital hepatic fibrosis with Carolis disease is presented in adolescent. The diagnosis of the disease is mainly based on periportal fibrosis and capsular ectssia of intrahepatic bile ducts combined with clinical data.关键词
肝硬化/Caroli病/病理学Key words
liver cirrhosis/ Carolis disease/ pathology分类
医药卫生引用本文复制引用
熊璐,赵景民,周光德,郭晓东,梁丽,赵雨来,李文淑,刘树红,韦立新..先天性肝纤维化伴Caroli病临床病理分析[J].解放军医学杂志,2011,36(1):86-88,3.
