Iron storage disease in parents and sibs of infants with neonatal hemochromatosis: 30-year follow-up.
Am J Med Genet
; 37(3): 342-5, 1990 Nov.
Article
in En
| MEDLINE
| ID: mdl-2260562
ABSTRACT
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.
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Collection:
01-internacional
Database:
MEDLINE
Main subject:
Hemochromatosis
/
Iron
/
Metal Metabolism, Inborn Errors
Type of study:
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
Limits:
Female
/
Humans
/
Male
Language:
En
Journal:
Am J Med Genet
Year:
1990
Document type:
Article
Affiliation country:
Denmark