Clinical Characteristics and Misdiagnosis Analysis of 47 Patients with Congenital Hepatic Fibrosis

Objective To explore clinical characteristics and misdiagnosis causes of congenital hepatic fibrosis(CHF),in order to improve the diagnosis rate.Methods Clinical data of 47 patients with congenital hepatic fibrosis in our hospital during January 2006 and December 2012 were retrospectively analyzed.Results In this group 44 patients were misdiagnosed,and the misdiagnosis rate was 93.6%.The main clinical manifestations were upper gastrointestinal bleeding,hepatosplenomegaly,mild jaundice,thrombocytopenia,fever and bellyache.A total of 34 patients(72.47%) suffered dysfunction of liver,and 12 patients(25.53%) suffered incorporated renal cysts.The 46 patients were confirmed as having CHF by hepatic puncture biopsy.The one patient did not undergo hepatic puncture biopsy.Among the patient's other two brothers or sisters,one died for alimentary tract hemorrhage inducted by hepatic cirrhosis,another was definitely diagnosed as having CHF by pathological result.In patients with dysfunction of liver,17 patients(36.17%) were misdiagnosed as having viral hepatitis,11 patients(23.40%) were misdiagnosed as having nonhepatotropic viral hepatitis;8 patients(17.02%) were misdiagnosed as having drug hepatitis;8 patients(17.02%) were misdiagnosed as having blood diseases because of thrombocytopenia;5 patients were still misdiagnosed as having hepatitis by liver biopsy in other hospitals.Conclusion Children with varicose bleeding,dysfunction of liver and thrombocytopenia should be suspected of having CHF in order to avoid misdiagnosis as hepatitis and blood diseases.