title: [Congenital hepatic fibrosis. Study of 26 cases].
abstract: We studied 26 children with congenital hepatic fibrosis during the period 1971-1993. About half of the children were about the 6 years old. Only two had brothers with same disease. The chief clinical manifestation was hematemesis associated or not with liver enlargement, predominantly of left lobe. Only one case showed fever and cholangitis. Liver function tests were usually normal. Twenty-two children had portal hypertension. Liver biopsy was of definitive for diagnosis. Seven children died.
authors.affiliation: Servicio de Gastroenterología, Instituto Nacional de Pediatría, Distrito Federal, México.
authors.forename: J A
authors.initials: JA
authors.lastname: Ramírez Mayans
authors.forename: N
authors.initials: N
authors.lastname: Mata Rivera
authors.forename: M A
authors.initials: MA
authors.lastname: Mora Tiscareño
authors.forename: R
authors.initials: R
authors.lastname: Cervantes Bustamante
authors.forename: M A
authors.initials: MA
authors.lastname: Vargas Gómez
authors.forename: V
authors.initials: V
authors.lastname: Aguinaga
authors.forename: G
authors.initials: G
authors.lastname: Rocío
mesh_terms: D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008103:Liver Cirrhosis / Q000151:congenital* / Q000175:diagnosis; D008297:Male
vernacular_title: Fibrosis hepática congénita. Estudio de 26 casos.