Congenital hepatic fibrosis: a family study

One family with four of seven siblings with congenital hepatic fibrosis is reported. The proband, the only member of this family with symptoms referable to the disease, was hospitalized because of an upper gastrointestinal hemorrhage. He had a presinusoidal type of portal hypertension. The other three siblings had the latent form of congenital hepatic fibrosis. In the family studied, intravenous pyelography and kidney biopsies showed normal results. This condition is possibly an inherited recessive autosomal disease.