Neonatal Hemochromatosis

Neonatal hemochromatosis is a rare disease clinically defined as severe neonatal liver disease in association with extrahepatic siderozis. The etiology of neonatal hemochromatosis is not understood exactly. However, according to a theory neonatal hemochromatosis is accepted to be an alloimmune disorder causing liver injury in fetus. After an effected one in the pregnancy the recurrence rate of neonatal hemochromatosis is ~80%. Hepatocellular failure which occurs in the first days of life with coagulopathy, hypoglycemia, hypoalbuminemia, hypofibrinogenemia, thrombocytopenia, anemia, and direct and indirect hyperbilirubinemia characterizes neonatal hemochromatosis. In order to diagnose neonatal hemochromatosis there are some certain criteria that sould be taken into account such as a positive family history, high serum ferritin levels, high serum alpha-fetoprotein levels and siderozis demonstrated with histology or with magnetic resonance. Since an affective medical treatment has not been found yet, liver transplantation is almost always required. The prognosis of neonatal hemochromatosis is generally poor. This review will discuss neonatal hemochromatosis that leads to liver failure in the fetus or newborn.