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首页> 外文期刊>Molecular biology reports >Plasma lipids and lipoproteins in children and young adults with major beta-thalassemia from western Iran: influence of genotype
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Plasma lipids and lipoproteins in children and young adults with major beta-thalassemia from western Iran: influence of genotype

机译:伊朗西部患有严重β-地中海贫血的儿童和年轻人的血浆脂质和脂蛋白:基因型的影响

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摘要

To determine the plasma lipid and lipoprotein profiles and their possible association with the type of beta-thalassemia mutation we studied 103 major beta-thalassemia patients including 71 children and 32 young adults compared to 102 healthy subjects consisted of 90 children and 12 young healthy adults. The plasma lipid and lipoprotein levels were measured by conventional methods. Considering all of the patients the levels of total cholesterol (TC), LDL-cholesterol (LDL-C), and HDL-cholesterol (HDL-C) were significantly lower compared to controls. However, the level of TG was significantly higher in cases than controls. Comparing thalassemic patients homozygous for a beta(0) type of mutation with those homozygous for a beta(+) type of mutation (IVSI.110 G:A) indicated that the levels of LDL-C, TC were significantly increased and TG concentration tended to be higher in the latter patients. In conclusion, our study indicates that hemolytic stress results in hypocholesterolemia in major beta-thalassemia patients and the presence of more severe genotype in patients is correlated with more reduction in TG, TC, and LDL-C levels.
机译:为了确定血浆脂质和脂蛋白谱及其与β地中海贫血突变类型的可能关系,我们研究了103名主要β地中海贫血患者,包括71名儿童和32名年轻成年人,而102名健康受试者包括90名儿童和12名年轻健康成年人。血浆脂质和脂蛋白水平通过常规方法测量。考虑到所有患者的总胆固醇(TC),LDL-胆固醇(LDL-C)和HDL-胆固醇(HDL-C)水平均显着低于对照组。但是,在某些情况下,TG的水平明显高于对照组。将纯合β(0)型突变的地中海贫血患者与纯合β(+)型突变(IVSI.110 G:A)的地中海贫血患者进行比较,结果表明LDL-C,TC的水平显着增加,TG浓度升高在后者的患者中更高。总之,我们的研究表明,溶血应激可导致主要的β地中海贫血患者发生低胆固醇血症,并且患者中存在更严重的基因型与TG,TC和LDL-C水平的降低更多有关。

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