Abstract
Neonatal hemochromatosis (NH) is a clinical syndrome consisting of liver disease and
pathologic siderosis of various extrahepatic tissues. NH is a form of secondary hemochromatosis
in which severe fetal liver injury causes iron overload due to poor regulation of
maternofetal iron flux. Gestational alloimmune liver disease (GALD) has been established
as the cause of fetal liver injury resulting in nearly all cases of NH. In GALD, sensitization
of some women to a fetal liver antigen results in development of specific antifetal
liver IgG antibodies. When delivered to the fetal circulation these antibodies bind
to the antigen and activate the terminal complement cascade resulting in hepatocyte
injury and death. GALD may produce subacute and chronic fetal liver injury (congenital
cirrhosis) typical of NH. It may also produce acute injury and acute liver failure
of the fetus and newborn, often with no iron overload or siderosis.
Keywords
hemochromatosis - alloimmune - fetus
