Congenital hepatic fibrosis in children.
Twenty-seven children with congenital hepatic fibrosis were followed for three months to 12 years. Hepatosplenomegaly, normal liver function tests, and kidney abnormalities were present in most patients, indicating that a correct diagnosis of congenital hepatic fibrosis could be made using simple clinical, biologic, and radiologic criteria. Esophageal endoscopy showed varices in 21 patients. Sixteen children underwent portal-systemic shunt surgery. Follow-up examinations did not show any impairment of liver function or any sign of hepatic encephalopathy. Cholangitis was present in only three children.
D000293:Adolescent; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D002758:Cholangiography; D005260:Female; D006529:Hepatomegaly / Q000209:etiology; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology / Q000601:surgery; D008099:Liver / Q000473:pathology; D008107:Liver Diseases / Q000150:complications / Q000151:congenital*; D008297:Male; D007690:Polycystic Kidney Diseases / Q000209:etiology; D011170:Portasystemic Shunt, Surgical; D013163:Splenomegaly / Q000209:etiology