Clinical and Pathological Features Analysis of 47 Cases of Congenital Hepatic Fibrosis

Objective: To analyze the clinical and pathological features of congenital hepatic fibrosis(CHF).Methods: Clinical and pathological features of 47 cases of congenital hepatic fibrosis in the 302 hospital of PLA from January 2008 to August 2012 were retrospectively studied.Results: There were 25 male and 22 female patients with mean age of 17.85±12.49 years old.The main clinical manifestations of CHF was portal hypertension and liver function test had no obvious decline.The main complications were ascites and hemorrhage of upper digestive tract.The main pathologic features of 45 cases of CHF showed that normally arranged hepatocytes were encompassed or intruded by the densely and non-inflammation collagenized septa,or hepatic plates were arrayed by fiber bundles with broaded periportal zone.No typical pseudolobule structure were found.Some cases showed small bile duct proliferation,expansion and deformity,35.6% of them with Calori disease.Conclusions:Congenital hepatic fibrosis usually presents in adolescent or young adulthood without significant gender difference.Portal hypertension and liver function damage are inconsistent in CHF patients,some of them with Calori disease.Liver biopsy is required for diagnosis.