Coincidental nontransfusional iron overload and thalassemia minor: association with HLA-linked hemochromatosis
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چکیده
منابع مشابه
Coincidental nontransfusional iron overload and thalassemia minor: association with HLA-linked hemochromatosis.
A pedigree was studied in which five individuals with beta-thalassemia minor were found to have nontransfusional hemochromatosis. Three were children under the age of 10 and two were young male adults, ages 28 and 33. A 5-yr-old child without evidence of thalassemia also had hemochromatosis. Since hemochromatosis is transmitted as an HLA-linked autosomal recessive disorder, HLA haplotypes serve...
متن کاملIron Overload and Thalassemia Minor: Association With HLA-Linked Hemochromatosis
A pedigree was studied in which five individuals with fl-thalassemia minor were found to have nontransfusional hemochromatosis. Three were children under the age of 10 and two were young male adults. ages 28 and 33. A 5-yr-old child without evidence of thalassemia also had hemochromatosis. Since hemochromatosis is transmitted as an HIA-linked autosomal recessive disorder. HLA haplotypes serve a...
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genes. Hemochromatosis is inherited as an autosomal recessive trait and The Cys282Tyr mutation was detected in only 1 of the 81 individis manifested by excessive absorption of dietary iron in homozygotes uals with thalassemia intermedia. The proband presented in 1975 at leading to complications of iron overload and premature death. In the age of 42 years with joint pains. Hepatomegaly was noted...
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Five of seven patients with idiopathic refractory sideroblastic anemia carried an HLA-A3 alloantigen (relative risk, 7.3; P = 0.02). The significance of this association was strengthened by study of two pedigrees. An abnormality in iron metabolism was found in two siblings who had an HLA-A3,B14 haplotype in common with the first proband. A second proband with idiopathic refractory sideroblastic...
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Volume 41, January 2001 TRANSFUSION 123 Hemochromatosis occurs in approximately 1 in 200 white persons of Western European descent, and it increases the propensity to absorb excess iron. Iron overload associated with hemochromatosis can cause hepatic cirrhosis, primary liver cancer, arthropathy, diabetes mellitus, other endocrinopathic disorders, and a reduction in lifespan.1,2 These complicati...
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ژورنال
عنوان ژورنال: Blood
سال: 1981
ISSN: 0006-4971,1528-0020
DOI: 10.1182/blood.v58.4.844.bloodjournal584844