Neonatal hemochromatosis: failure of deferoxamine therapy

We describe a premature neonate with severe hepatic dysfunction from birth which progressed to fatal hepatic failure at 3 months of age. The diagnosis of a familial disorder, neonatal hemochromatosis, was made, and therapy was attempted with deferoxamine. Features of iron metabolism are presented, and this infant's illness is contrasted with other liver diseases of infancy associated with hepatic iron overload.