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沙特阿拉伯的镰状细胞-β0-地中海贫血

Sickle cell-beta 0-thalassaemia in Saudi Arabia.

作者信息

el-Hazmi M A, Al-Swailem A R

机构信息

College of Medicine, King Saud University, Riyadh, Saudi Arabia.

出版信息

Hum Hered. 1987;37(4):211-6. doi: 10.1159/000153705.

Abstract

During an extensive investigation to determine the frequency of sickle cell and thalassaemia genes in the Saudi population, 22 cases with S/beta 0-thalassaemia were identified and the haematological, biochemical and clinical findings were compared with those in patients with sickle cell anaemia. The values of mean cell volume, mean cell haemoglobin and packed cell volume were found to be lower while all other haematological parameters including Hb A2 were higher in the S/beta 0-thalassaemia group. No statistically significant difference in the Hb F level was found between the two groups. Biochemical parameters were grouped according to organ function tests. Only slight differences were seen in the values of some parameters. The clinical data showed that, in general, patients with sickle cell anaemia had a more severe condition than the S/beta 0-thalassaemia.

摘要

在一项旨在确定沙特人群中镰状细胞和地中海贫血基因频率的广泛调查中,识别出了22例S/β0-地中海贫血患者,并将其血液学、生化和临床检查结果与镰状细胞贫血患者的结果进行了比较。结果发现,S/β0-地中海贫血组的平均红细胞体积、平均红细胞血红蛋白含量和红细胞压积值较低,而包括血红蛋白A2在内的所有其他血液学参数均较高。两组之间的胎儿血红蛋白水平未发现统计学上的显著差异。生化参数根据器官功能测试进行分组。仅在某些参数值上发现了细微差异。临床数据表明,总体而言,镰状细胞贫血患者的病情比S/β0-地中海贫血患者更为严重。

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