Neonatal hemochromatosis.
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.
Knisely
A S
AS
Institute of Liver Studies, King's College Hospital, London, UK.
Mieli-Vergani
Giorgina
G
Whitington
Peter F
PF
D005260:Female; D005808:Genes, Recessive; D005817:Genetic Counseling; D006432:Hemochromatosis / Q000175:diagnosis / Q000209:etiology / Q000473:pathology / Q000628:therapy; D006801:Humans; D007231:Infant, Newborn; D007501:Iron / Q000378:metabolism; D008099:Liver / Q000378:metabolism / Q000473:pathology; D017093:Liver Failure / Q000209:etiology*; D008431:Maternal-Fetal Exchange; D010641:Phenotype; D011247:Pregnancy; D011296:Prenatal Diagnosis; D011379:Prognosis
D007501:Iron