Risk of disease in siblings of patients with hereditary hemochromatosis.
BACKGROUND
Parents of hereditary hemochromatosis (HH) homozygote patients, who are predominantly HH heterozygotes, have been found to have an increased risk of colonic neoplasia, diabetes, stroke death, stomach cancer and leukemia. The health histories of siblings of HH patients are reported.


METHODS
Individuals homozygous for HH were mailed questionnaires concerning the health histories of their siblings. Spouses of the HH homozygotes were asked to complete accompanying questionnaires concerning their siblings. The frequencies of serious illness and, when specified, each reported disease were determined by zygosity, odds ratios and 95% confidence intervals estimated.


RESULTS
Data were available for 279 siblings known to be HH homozygotes (HHs), and 1,265 other siblings of HH patients, who are predominantly hemochromatosis heterozygotes (HHhet). Controls consisted of 1,338 spouse siblings in whom only the general population prevalence of HH homozygosity and heterozygosity existed. Odds ratios comparing HH strata to controls were elevated for serious illness (1.12 in HHhet, 1.71 in HHs), diabetes (0.78, 2.45), arthritis (2.10, 1.69), and hepatoma (1.06, 11.96).


CONCLUSIONS
There was a significant trend for increased risk of serious illness among siblings of HH patients with increasing exposure to the HH gene, especially for diabetes, arthritis and hepatoma.
Nelson
R L
RL
Department of Surgery, College of Medicine, Chicago, Ill 60612, USA. altohorn@uic.edu
Persky
V
V
Davis
F
F
Becker
E
E
D001168:Arthritis / Q000235:genetics; D002318:Cardiovascular Diseases / Q000235:genetics; D002908:Chronic Disease; D015331:Cohort Studies; D003920:Diabetes Mellitus / Q000235:genetics; D005192:Family Health; D005260:Female; D006432:Hemochromatosis / Q000235:genetics*; D006579:Heterozygote; D006720:Homozygote; D006801:Humans; D008297:Male; D009369:Neoplasms / Q000235:genetics; D009679:Nuclear Family; D016017:Odds Ratio; D018570:Risk Assessment; D011795:Surveys and Questionnaires