HEMOCHROMATOSIS is an uncommon disease of uncertain etiology occurring predominantly in males and characterized by extensive deposition of iron throughout the body. Although the liver, pancreas, and skin are chiefly involved, significant iron accumulation may be present in lymph nodes, testes, heart, and adrenals. Of all the organs, the brain alone is said to be spared.1 Clinically, hemochromatosis is characterized by cirrhosis of the liver usually accompanied by diabetes and pigmentation of the skin. Asthenia, loss of weight, and hypogonadism are frequently present.
It seems fairly well established, particularly by radioiron absorption studies, that the underlying defect in hemochromatosis is a disturbance in the normal intestinal mucosal iron-blocking mechanism, resulting in excessive iron absorption regardless of the saturation of the body stores.* Since there is no active mechanism, except for hemorrhage, by which iron can be excreted,† a gradual increase in tissue iron saturation occurs. As a result, the
MYERSON RM, CARROLL IN. TREATMENT OF HEMOCHROMATOSIS BY MASSIVE VENESECTION: Report of a Case Treated by Removal of Forty Liters of Blood in Twenty-Eight Months. AMA Arch Intern Med. 1955;95(2):349–353. doi:10.1001/archinte.1955.00250080171018
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