Alayash A I, Dafallah A, Al-Husayni H, Al-Ali A K, Al-Quorain A, Omer A H, Wilson M T, Bonaventura J, Cashon R
Acta Haematol. 1986;75(3):160-4. doi: 10.1159/000206111.
Glycosylated hemoglobin was determined by the thiobarbituric acid method in sickle cell anemia patients from the Eastern Province of Saudi Arabia. The level of glycosylated hemoglobin in a Saudi SS sample (4.36%, SD 0.83) is 90% of that of the sample of normals (4.85%, SD 0.51). This is in contrast with the reported value of glycosylated hemoglobin in an American Black SS sample (3.9%, SD 0.6), which is only 60% of that of the sample of normals (6.6%, SD 0.7). The fetal hemoglobin level in Saudi sickle cell patients was 12.03% (SD 4.84), which is significantly different from that of Americans of African origin at p = 0.001. There was no significant correlation (r = 0.236) between the percentages of glycosylated Hb and Hb F at the 10% confidence level. The reported positive relationship between the percentages of glycosylated Hb and Hb F in American Blacks seems to be valid in the Saudi population only up to the level of 10-12% of fetal hemoglobin. Above this threshold of Hb F no further alleviating effect is seen. The 2,3-diphosphoglycerate value in Saudi Hb SS adults was 21.7 mumol/g (SD 7.4) and accordingly only twice as high as that of normal individuals. The benign clinical course exhibited by Saudi sickle cell patients is reflected by the survival of the RBC as indexed by its content of glycosylated Hb and 2,3-diphosphoglycerate. Moreover 10-12% of fetal hemoglobin in the RBC seems sufficient to ameliorate the severity of this disease in patients from the Eastern Province of Saudi Arabia.
采用硫代巴比妥酸法测定了沙特阿拉伯东部省份镰状细胞贫血患者的糖化血红蛋白水平。沙特SS样本的糖化血红蛋白水平为4.36%(标准差0.83),是正常样本(4.85%,标准差0.51)的90%。这与美国黑人SS样本中糖化血红蛋白的报告值(3.9%,标准差0.6)形成对比,后者仅为正常样本(6.6%,标准差0.7)的60%。沙特镰状细胞患者的胎儿血红蛋白水平为12.03%(标准差4.84),与非洲裔美国人的水平相比有显著差异,p = 0.001。在10%置信水平下,糖化血红蛋白百分比与胎儿血红蛋白百分比之间无显著相关性(r = 0.236)。美国黑人中糖化血红蛋白百分比与胎儿血红蛋白百分比之间报告的正相关关系,似乎仅在沙特人群中胎儿血红蛋白水平达到10% - 12%时才成立。超过这个胎儿血红蛋白阈值,就看不到进一步的缓解效果。沙特Hb SS成年患者的2,3 - 二磷酸甘油酸值为21.7 μmol/g(标准差7.4),因此仅为正常个体的两倍。沙特镰状细胞患者所表现出的良性临床病程,通过红细胞的存活情况得以体现,其指标为糖化血红蛋白和2,3 - 二磷酸甘油酸的含量。此外,红细胞中10% - 12%的胎儿血红蛋白似乎足以改善沙特阿拉伯东部省份患者的这种疾病严重程度。