Congenital hepatic fibrosis combined with protein-losing enteropathy and recurrent thrombosis

Two siblings, suffering from recurrent thrombosis and protein-losing enteropathy are presented. Histopathological examination of the liver in one of the patients, who died of multiple thrombosis, showed changes characteristic of Congenital Hepatic Fibrosis. Coagulation studies in the second child revealed decreased antithrombin-III in plasma, which might have had pathogenetic implications for the thrombosis tendency seen in both patients.