Idiopathic Hemochromatosis

Abstract

Hemochromatosis is a disease of iron storage characterized by excessive iron deposition in certain body organs. Depending on the manner in which the excessive deposition results, the disease may be classified as dietary, secondary to excessive blood transfusions, or idiopathic. In 1935 Sheldon1suggested that the idiopathic type resulted from a gastrointestinal mucosal defect which in view of the familial incidence of the disease was secondary to an inborn error of metabolism. This is the presently accepted concept of the disease, although recent studies have suggested that this is not always the case. Two features of the disease are the age incidence—90% over 35 years of age, and the sex incidence—90% or more in males. Finch and Finch2in their review of 787 cases found only 8 in women under 40 years of age, and in at least 4 of these cases there was a history of decreased