HEMOCHROMATOSIS: REPORT OF A CASE IN A NEGRO; DISCUSSION OF IRON METABOLISM

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Abstract
A case of hemochromatosis in a Negro is reported and the question is raised of the mechanism of entrance of the vast amt. of stored iron in this disease. The ferritin-apoferritin mechanism, mucosal block and physiological saturation, if operative in patients with hemochromatosis, should exclude excess iron absorption. A disturbance in the mechanism of iron absorption is postulated in hemochromatosis, and it is suggested that this disease is due to an inborn error of metabolism with a biologic basis in enzyme system defects occurring somewhere along the chain of nucleo-protein synthesis, as is shown in analogous situations for Neuro-spora by Beadle. The 95:5 sex distribution in favor of the male may be due to sex linkage of this defect or to special channels of iron excretion in the female such as menstruation, pregnancy and lactation. Hemochromatosis is a disease whose clinical picture is manifested only after many yrs. of excess iron absorption. An increase in the number of patients with this disease is therefore to be expected in an aging population.