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Familial Aspects of Idiopathic Hemochromatosis

Familial Aspects of Idiopathic Hemochromatosis WHETHER or not idiopathic hemochromatosis is a genetically determined disease is still controversial. Because of the clinically proved fact1-4 that phlebotomy therapy certainly ameliorates and possibly permanently reverses the natural course of idiopathic hemochromatosis, we have undertaken a study of 1 family with 2 biopsy-proved instances of hemochromatosis. It is often difficult to ascertain from the literature the actual size of those families purported to have more than one member afflicted with hemochromatosis. Hence, in this family, we expanded our study to include virtually every living relative over the age of 5 years. In his excellent monograph Sheldon5 (p. 339) stated, "The view advanced as the most reasonable explanation of hemochromatosis is that it should be classed as an inborn error of metabolism, which has an overwhelming incidence in males, and which at times actually has a familial incidence." He cited in his review 7 instances of hemochromatosis http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png JAMA American Medical Association

Familial Aspects of Idiopathic Hemochromatosis

Johnson, George B.; Frey, Walter G.
JAMA , Volume 179 (10) – Mar 10, 1962
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References (13)

Publisher
American Medical Association
Copyright
Copyright © 1962 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.
ISSN
0098-7484
eISSN
1538-3598
DOI
10.1001/jama.1962.03050100001001
Publisher site
See Article on Publisher Site

Abstract

WHETHER or not idiopathic hemochromatosis is a genetically determined disease is still controversial. Because of the clinically proved fact1-4 that phlebotomy therapy certainly ameliorates and possibly permanently reverses the natural course of idiopathic hemochromatosis, we have undertaken a study of 1 family with 2 biopsy-proved instances of hemochromatosis. It is often difficult to ascertain from the literature the actual size of those families purported to have more than one member afflicted with hemochromatosis. Hence, in this family, we expanded our study to include virtually every living relative over the age of 5 years. In his excellent monograph Sheldon5 (p. 339) stated, "The view advanced as the most reasonable explanation of hemochromatosis is that it should be classed as an inborn error of metabolism, which has an overwhelming incidence in males, and which at times actually has a familial incidence." He cited in his review 7 instances of hemochromatosis

Journal

JAMA – American Medical Association

Published: Mar 10, 1962

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