Iron storage disease in parents and sibs of infants with neonatal hemochromatosis: 30-year follow-up.
Neonatal hemochromatosis (NH), an uncommon and generally fatal disorder of infancy, is defined by hepatic disease of antenatal onset, generally manifest at birth, and by stainable iron in a tissue distribution like that seen in heritable adult-onset hemochromatosis (HH). It is not known if parents and sibs of infants with NH are at risk of iron storage disease in later life. We provide 30-year follow-up for iron overload of a family in which 6 of 9 children died in utero or early in the neonatal period. Four of the 6 came to autopsy, where severe liver disease was found; in 3 of the 4, autopsy material could be reviewed. These 3 children had NH. Studies 30 years later did not identify HH or other iron storage disease in the parents or surviving sibs. These findings suggest that first-degree relatives of persons with NH are not necessarily at increased risk of iron storage disease in later life.
Dalhøj
J
J
Arvepatologisk Institut, Odense Universitet, Denmark.
Kiaer
H
H
Wiggers
P
P
Grady
R W
RW
Jones
R L
RL
Knisely
A S
AS
D000375:Aging; D005260:Female; D005500:Follow-Up Studies; D006432:Hemochromatosis / Q000235:genetics*; D006801:Humans; D007501:Iron / Q000378:metabolism*; D008297:Male; D008664:Metal Metabolism, Inborn Errors / Q000235:genetics*; D010375:Pedigree
D007501:Iron