Clinical analysis of 24 cases of congenital hepatic fibrosis

Objective To analysis the clinical features of congenital hepatic fibrosis (CHF) .Methods The clinical features, physical examination, laboratory data and pathological features were retrospectively reviewed in 24 cases of CHF patients.Results All 24 patients showed the symptoms of portal hypertension 8 patients have had variceal source of upper gastrointestinal bleeding.Laboratory workup reveal normal or mild elevation in liver enzymes.7 patients have liver or kidney cysts.22 patients were diagnosed CHF through pathological examination.Conclusion The patients with portal hypertension of unknown reasons should exclude CHF by liver pathological examination, especially in patients which degree of portal hypertension and liver damage is inconsistent.CHF is often associated with kidney cysts and Caroli's disease.