Clinical manifestations and pathological features of congenital hepatic fibrosis: an analysis of 13 patients

Objective To summarize the clinical manifestations and pathological features of congenital hepatic fibrosis( CHF),and to improve the experience in the diagnosis and treatment of this disease. Methods A total of 13 patients with a confirmed diagnosis of CHF based on histopathological examinations who were hospitalized and treated in Xijing Hospital,Fourth Military Medical University,from January2011 to June 2015 were analyzed retrospectively. The clinical data including age,clinical manifestations,laboratory markers,and imaging findings were summarized and analyzed. Results Of all the patients,there were 8 cases of portal hypertension type,1 case of cholangitis type,1 case of mixed type,and 1 case of latent type. The imaging findings suggested that 8 patients had liver and kidney cysts,and 4 patients had cavernous transformation of the portal vein. Conclusion CHF patients often have portal hypertension and normal liver function as prominent manifestations,with concurrent liver and kidney cysts and Caroli disease. Liver biopsy should be performed for unexplained liver cirrhosis,especially for patients with inconsistency between reduction in liver function and portal hypertension.