Stevens D W, Wainscoat J S, Ketley N, Timms P, Ayoub D, Shah R
J Trop Pediatr. 1989 Dec;35(6):301-5. doi: 10.1093/tropej/35.6.301.
The pathogenesis of hypochromic anaemia was studied in 138 Saudi bedouin infants aged 9 months. Approximately 25 per cent had hypochromic anaemia, but less than 10 per cent had serum ferritin levels indicative of iron deficiency. A few infants had heterozygous beta-thalassaemia, but many infants with hypochromic anaemia had normal haemoglobin A2 levels together with serum ferritin levels above 20 micrograms/l. DNA analysis of cord blood taken from the hospital where the infants were born showed that the frequency of the single alpha-globin gene deletion type (-alpha 3.7) of alpha-thalassaemia is 0.13 in the bedouin population of Western Saudi Arabia. alpha-Thalassaemia probably accounts for much of the anaemia previously thought to be due to iron deficiency in Saudi infants. Studies of iron status and estimation of the frequency of genetic causes of hypochromic anaemia are important when assessing the need for widespread nutritional programmes to prevent iron deficiency and in the interpretation of reference ranges of red cell indices in populations from malarial areas.
对138名9个月大的沙特贝都因婴儿的低色素性贫血发病机制进行了研究。约25%的婴儿患有低色素性贫血,但血清铁蛋白水平表明存在缺铁的婴儿不到10%。少数婴儿为杂合子β地中海贫血,但许多低色素性贫血婴儿的血红蛋白A2水平正常,且血清铁蛋白水平高于20微克/升。对婴儿出生医院采集的脐带血进行DNA分析显示,沙特阿拉伯西部贝都因人群中α地中海贫血的单α珠蛋白基因缺失型(-α3.7)频率为0.13。α地中海贫血可能是沙特婴儿以前认为由缺铁引起的贫血的主要原因。在评估广泛开展预防缺铁营养计划的必要性以及解释疟疾地区人群红细胞指数参考范围时,研究铁状态和估计低色素性贫血的遗传病因频率非常重要。