Neonatal hemochromatosis: five cases.

Neonatal hemochromatosis is characterized by abnormal hepatic and extrahepa tic iron overload, which spares the reticuloendothelial system. In neonates , hemochromatosis results in an acute and frequently lethal liver failure. Case reports. - We report five cases of neonatal hemochromatosis which demo nstrate various aspects of this disorder and underline the complexity of bo th the diagnosis and treatment. Case 3 had an extremely low arterial pressu re, a presentation not yet described. Conclusion. - Neonatal hemochromatosis should be suspected in the presence of cholestasis with liver failure of perinatal onset and with high blood le vel ferritin. Abdominal nuclear magnetic resonance and/or liver biopsy can confirm neonatal hemochromatosis, For one of our patients, a medical treatm ent allowed us to perform a liver transplantation. (C) 2000 Editions scient ifiques et medicales Elsevier SAS.