Neonatal hemochromatosis (NH), although rare, is the most common cause of liver failure in the neonate. This disorder is associated with extrahepatic siderosis and has been called neonatal iron storage disease. The pathogene sis was a mystery until 2004, when Peter Whitington hypothesized that NH may be an immune-mediated disorder. Clinical and laboratory findings demonstrate liver failure but usually are nonspecific. Evidence of extrahepatic siderosis by magnetic resonance imaging with decreased intensity of T2-weighted sequences of specific organs confirms the diagnosis. Treatment of NH is challenging, and survival rates are dismal despite medical agents (ie, iron chelators and antioxidants) or liver transplantation. Recently, NH has been prevented in subsequent pregnancies by administering intrauterine immunoglobulin to pregnant women, changing NH from a lethal to a nonlethal disease.

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