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Hemochromatosis

Hemochromatosis is a hereditary disease, a defect in the regulation of the absorption of iron from foodstuffs. It results in the progressive accumulation of iron in the body. By midlife sufficient iron may have been absorbed to damage the liver, pancreas and testes to cause failure of these organs. Cirrhosis, liver cancer, diabetes and impotence may result. A form of arthritis develops in many patients. There is also susceptibility to serious infectious diseases; one such infection may occur after eating raw oysters. Females are relatively protected against these abnormalities, at least until after menopause, because of the loss of iron with menstruation and pregnancy.

The disorder remains asymptomatic until the development of organ failure but the disease can readily be detected by laboratory studies to measure levels of iron in the blood. Early diagnosis is important because the late effects of iron accumulation can be wholly prevented by periodic phlebotomies (blood donations). Probably a one-time study of iron levels early in adult life would be sufficient to evaluate an individual.

The prevalence of hemochromatosis varies in different populations. In Northern Europeans it is of the order of one in 400 persons. Other populations probably have a lower prevalence of this disease. There exist other causes of excess iron accumulation as well. These include multiple blood transfusions, the presence of certain blood diseases, and the drinking of iron-rich beer by Bantus.

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