el-Hazmi M A, Warsy A S
Medical Biochemistry Department, College of Medicine, Riyadh, Saudi Arabia.
Trop Geogr Med. 1989 Jan;41(1):52-6.
This study was conducted on 81 children with sickle cell anaemia (SCA) from the south-western province of Saudi Arabia. Ten of these children had associated glucose-6-phosphate dehydrogenase (G-6-PD) deficiency due to the presence of the phenotype 'G-6-PD Mediterranean'. Slightly lower, statistically non significant, values of red blood cell count, total haemoglobin, packed cell volume and red cell indices were found in SCA patients with G-6-PD deficiency. However, certain clinical features were improved in these patients. Comparison was also made with results published from the eastern province of the country, where SCA is reported to be mild. It is suggested that G-6-PD deficiency due to G-6-PD 'Mediterranean' worsens certain manifestations and improves others in SCA patients with a severe form of SCA, while improvement in haematological parameters is observed in SCA patients with mild SCA.
本研究对沙特阿拉伯西南部省份的81名镰状细胞贫血（SCA）患儿进行。这些患儿中有10名因存在“地中海型葡萄糖-6-磷酸脱氢酶（G-6-PD）”表型而伴有G-6-PD缺乏症。在伴有G-6-PD缺乏症的SCA患者中，红细胞计数、总血红蛋白、红细胞压积和红细胞指数的值略低，但无统计学意义。然而，这些患者的某些临床特征有所改善。研究还与该国东部省份发表的结果进行了比较，据报道该地区的SCA病情较轻。研究表明，对于患有严重SCA的患者，“地中海型”G-6-PD缺乏会使SCA的某些表现恶化，而使另一些表现改善；而对于患有轻度SCA的患者，血液学参数则有所改善。
