Management of hemochromatosis

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Abstract
IntroductionThe complications of iron overload in the hemochromatosis can be avoided by early diagnosis and appropriate management. Therapeutic phlebotomy is used to remove excess iron and maintain low normal body iron stores, and it should be initiated in men with serum ferritin levels of 300 g/l or more and in women with serum ferritin levels of 200 μg/l or more, regardless of the presence or absence of symptoms. Typically, therapeutic phlebotomy consist of (i) removal of 1 unit (450 to 500 ml) of blood weekly untilthe serum ferritin level is 10 to 20 μg/l and (ii) maintenance of the serum ferritin level at 50 μg/l or less the reafter by periodic removal of blood. Hyperferritinemia attributable to iron overload is resolved by therapeutic phlebotomy. When applied before iron overload becomes severe, this treatment also prevents complications of iron overload, including hepatic cirrhosis, primary liver cancer, diabetes mellitus, hypogonadotrophic hypogonadism, joint disease, and cardiomyopathy. In patients with established iron overload disease, weakness, fatigue, increased hepaticenzyme concentrations, right upper quadrant pain, and hyperpigmentation are often substantially alleviated by therapeutic phlebotomy. Patients with liver disease, joint disease, diabetes mellitus and other endocrinopathic abnormalities, and cardiac abnormalities of ten require additional, specific management. Dietary management of hemochromatosis includes avoidance of medicinal iron, mineral supplements, excess vitamin C, and uncooked sea foods. This can reduce the rate of iron reaccumulation; reduce retention of non-ferrous metals; and help reduce complications of liver disease, diabetes mellitus, and Vibrio infection. This comprehensive approach to the management of hemochromatosis can decrease the frequency and severity of iron overload, improve quality of life, and increase longevity.

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