Abstract

Neonatal hemochromatosis is a very rare disease of gestation associated with fetal liver injury. The etiology of NH is not fully understood and alloimmune etiology has been suggested. We describe a case of NH in a term baby with a successful outcome. The early neonatal period was complicated with severe haemorrhagic syndrome, anaemia, thrombocytopenia, hepatosplenomegalia and hepatic insufficiency which culminated in remarcable  cholestasis and severe iron deposition. Severe NH was diagnosed at the age of two weeks on the basis of clinical, biochemical and liver biopsy findings. With iron reduction and immunoglobulin therapy laboratory findings gradually returned to normal at the age of 4–5 months. Considering successful recovery from severe NH, alloimmune neonatal haemochromatosis can be suggested.