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Published on: June 5, 2014
Neonatal hemochromatosis
A S Knisely1, Giorgina Mieli-Vergani, Peter F Whitington
1Institute of Liver Studies, King's College Hospital, London, UK.
Neonatal hemochromatosis is a rare gestational condition in which iron accumulates in the fetal tissues in a distribution like that seen in hereditary hemochromatosis. Extensive liver damage is the dominant clinical feature, with late fetal loss or early neonatal death. NH recurs within sibships at a rate higher than that predicted for simple Mendelian autosomal-recessive inheritance, possibly suggesting the role of a maternal factor. Immunomodulation during pregnancy at risk appears to lessen the severity of disease.
Neonatal hemochromatosis is a rare gestational condition in which iron accumulates in the fetal tissues in a distribution like that seen in hereditary hemochromatosis. Extensive liver damage is the dominant clinical feature, with late fetal loss or early neonatal death. NH recurs within sibships at a rate higher than that predicted for simple Mendelian autosomal-recessive inheritance, possibly suggesting the role of a maternal factor. Immunomodulation during pregnancy at risk appears to lessen the severity of disease.
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