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沙特阿拉伯血红蛋白S的血液学、生化及临床表现(一)。血液学及临床表达。

The haematological, biochemical and clinical--presentation of haemoglobin S in Saudi Arabia (i). Haematological & clinical expression.

作者信息

El-Hazmi M A, Jabbar F A, Al-Faleh F Z, Al-Swailem A R, Warsy A S

出版信息

Trop Geogr Med. 1987 Apr;39(2):157-62.

PMID:3629709
Abstract

The haematological and clinical expression of haemoglobin S (Hb S) was investigated in a group of male and female Saudis of haemoglobin genotype AS and SS, and the results compared with those from normal individuals (AA). The influence of alpha thalassaemia in each genotype was also investigated. The total haemoglobin concentration (Hb), red blood cell count (RBC) and packed cell volume (PCV) were significantly lower in the SS group without thalassaemia. In the thalassaemic group Hb, RBC and PCV levels were higher, while MCV and MCH were considerably lower. The clinical investigations reveal that almost 30% of the SS cases had not required blood transfusion, and none of the patients had suffered from leg ulceration or hand and foot syndrome. Comparison of the results obtained for Saudi sickle cell patients with those reported for Jamaicans show several differences, indicating a milder disease in the Saudis.

摘要

对一组血红蛋白基因型为AS和SS的沙特男女的血红蛋白S(Hb S)的血液学和临床表达进行了研究,并将结果与正常个体(AA)的结果进行了比较。还研究了每种基因型中α地中海贫血的影响。无地中海贫血的SS组中,总血红蛋白浓度(Hb)、红细胞计数(RBC)和血细胞比容(PCV)显著较低。在地中海贫血组中,Hb、RBC和PCV水平较高,而平均红细胞体积(MCV)和平均红细胞血红蛋白含量(MCH)则显著较低。临床研究表明,几乎30%的SS病例不需要输血,且没有患者出现腿部溃疡或手足综合征。将沙特镰状细胞病患者的结果与牙买加人报告的结果进行比较,发现了一些差异,表明沙特人的病情较轻。

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