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Congenital hepatic fibrosis with Caroli’s disease:a clinicopathological analysis of 6 cases

Author(s): Lu XIONG | Jing-min ZHAO | Guang-de ZHOU | Xiao-dong GUO | Li LIANG | Yu-lai ZHAO | Wen-shu LI | Shu-hong LIU | Li-xin WEI

Journal: Medical Journal of Chinese People's Liberation Army
ISSN 0577-7402

Volume: 36;
Issue: 1;
Start page: 86;
Date: 2011;
Original page

Keywords: liver cirrhosis; Caroli’s disease; pathology

Objective To explore the clinical and pathological features of congenital hepatic fibrosis with Caroli’s disease.Methods The clinical characteristics of 6 patients with congenital hepatic fibrosis and Caroli’s disease were analyzed,and the histological 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 hematemesis(5/6),hypodynamia(5/6) and hepatosplenomegaly(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 ectasia of intrahepatic bile ducts,cholestasis 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 Caroli’s disease is presented in adolescent.The diagnosis of the disease is mainly based on periportal fibrosis and capsular ectasia of intrahepatic bile ducts combined with clinical data.
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