NEONATAL HEMOCHROMATOSIS - REPORT OF SUCCESSFUL ORTHOTOPIC LIVER-TRANSPLANTATION

Neonatal hemochromatosis is clinically characterized by profound hepatic insufficiency and death in the newborn period. Hemosiderosis develops in utero with iron deposition in a particular organ distribution with sparing of the reticuloendothelial system. Neonatal hemochromatosis has been established as a distinct form of giant cell hepatitis for more than three decades, but no consensus has yet been reached as to whether the iron deposition represents a primary disease or a secondary response (1,2). Neonatal hemochromatosis has pathologic similarities to hereditary hemochromatosis as seen in adults, but the human leukocyte antigen associations are absent (3-7). Many of the reported cases of neonatal hemochromatosis are in sibling pairs, but the mechanism of transmission is unknown (3, 4,7). © 1992 Raven Press, Ltd., New York.