Status of iron metabolism and erythropoietic proliferation in children with various genotypes of thalassemia
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R725

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    Abstract:

    OBJECTIVE: To study the status of iron metabolism and erythropoietic proliferation in children with various genotypes of thalassemia. METHODS: Serum concentrations of ferritin (SF), transferrin receptor (sTfR) and erythropoietin (EPO) were measured in 158 children with thalassemia. The differences in the concentrations of the three indices among children with different genotypes of thalassemia were compared. The correlations of the hemoglobin level with sereum SF, sTfR and EPO levels were assessed. RESULTS: Among the 158 children with thalassemia, 52(32.9%) were diagnosed with alpha-thalassemia minor, 27(17.1%) with HbH disease, 59(37.4%) with beta-thalassemia minor, 13(8.2%) with beta-thalassemia major, and 7(4.4%) with combining alpha beta thalassemia. The SF levels in children with HbH disease or beta-thalassemia major were significantly higher than those in the other thalassemia groups (P<0.01). The sTfR levels in children with beta-thalassemia major were the highest when compared with those in the other thalassemia groups (P<0.05). The EPO levels in children with beta-thalassemia major were also the highest when compared with those in the other thalassemia groups (P<0.01). There was a negative correlation between hemoglobin and EPO levels in children with HbH disease (r=-0.656, P<0.01) and beta-thalassemia major (r=-0.641; P<0.05). CONCLUSIONS: The status of iron metabolism and erythropoietic proliferation is different in children with different genotypes of thalassemia. A combined measurement of SF, sTfR and EPO may reflect the status of erythropoietic proliferation.[Chin J Contemp Pediatr, 2010, 12 (8):602-604]

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麦惠容, 李长钢, 王缨, 石红松, 赵维玲, 陈运生, 陈小文.不同基因型地中海贫血患儿铁代谢和红细胞系造血状况研究[J].中国当代儿科杂志英文版,2010,12(08):602-604

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  • Online: August 15,2010
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