Glycosylated hemoglobin levels in a benign form of sickle cell anemia in Saudi Arabia.
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.
Alayash, AI; Dafallah, A; Al-Husayni, H; Al-Ali, AK; Al-Quorain, A; Omer, AH; Wilson, MT; Bonaventura, J; Cashon, R
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