FAMILIAL HEPATOPORTAL SCLEROSIS IN A CHIL D

Background. - Hepatoportal sclerosis is uncommon in European countries and its diagnosis is difficult. The etiology remains unknown; its fam ilial origin is probably exceptional. Case report. - An 18-month-old g irl born to a mother with hepatoportal sclerosis had hepatomegaly. She also had a moderate splenomegaly and mild increase transaminase and g amma GT activities. Ultrasound examination failed to show portal hyper tension. Histological study of liver showed changes quite similar to t hose seen in her mother. Two members of the mother's family had portal hypertension. Discussion. - Familial forms of portal hypertension hav e been reported: a familial form of incomplete septal cirrhosis and a familial occurrence of cavernous transformation of the portal vein. Ob struction of the portal vessels was not found in our patient, the youn gest in whom hepatoportal sclerosis is documented.