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Value of hepatic iron measurements in early hemochromatosis and determination of the critical iron level associated with fibrosis

✍ Scribed by Mark L. Bassett; June W. Halliday; Lawrie W. Powell


Publisher
John Wiley and Sons
Year
1986
Tongue
English
Weight
593 KB
Volume
6
Category
Article
ISSN
0270-9139

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✦ Synopsis


The role of the measurement of hepatic iron in the diagnosis of genetic hemochromatosis was studied, with particular reference to the differentiation of early hemochromatosis from alcoholic siderosis and the critical hepatic iron concentration associated with fibrosis in hemochromatosis. Hepatic iron was measured in 30 homozygous relatives of 17 hemochromatosis probands, 8 heterozygous relatives, 5 1 patients with alcoholic liver disease and 40 control subjects. Hepatic iron concentrations were greatly increased in the majority of homozygous hemochromatosis subjects, and there was little overlap with the other groups. In the absence of alcoholism, fibrosis or cirrhosis in hemochromatosis was present only with hepatic iron concentrations above a threshold of approximately 400 pmoles per gm (22.3 mg per gm) dry weight. In some heterozygous hemochromatosis subjects and in some alcoholic patients, hepatic iron concentrations were in the range seen in young homozygous subjects. However, an age-related rise in hepatic iron was Seen only in hemochromatosis homozygotes, and calculation of an hepatic iron index (hepatic ironlage) resulted in a clear distinction between homozygotes and the other three groups. It is concluded: (a) that chemical measurement of hepatic iron concentration, when corrected for the age of the subject, reli- ably distinguishes early hemochromatosis from alcoholic siderosis, and (b), that there appears to be a threshold level of hepatic iron above which there is a high risk of fibrosis.


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