Nutrition Branch, Centers for Disease Control and Prevention (CDC), Atlanta, Georgia, USA; Department of Pediatrics and Global Health, Emory University, Atlanta, Georgia, USA.
Matern Child Nutr. 2014 Jan;10(1):135-44. doi: 10.1111/j.1740-8709.2012.00454.x. Epub 2012 Sep 13.
Although inherited blood disorders are common among children in many parts of Africa, limited data are available about their prevalence or contribution to childhood anaemia. We conducted a cross-sectional survey of 858 children aged 6-35 months who were randomly selected from 60 villages in western Kenya. Haemoglobin (Hb), ferritin, malaria, C-reactive protein (CRP) and retinol binding protein (RBP) were measured from capillary blood. Using polymerase chain reaction (PCR), Hb type, -3.7 kb alpha-globin chain deletion, glucose-6-phosphate dehydrogenase (G6PD) genotype and haptoglobin (Hp) genotype were determined. More than 2 out of 3 children had at least one measured blood disorder. Sickle cell trait (HbAS) and disease (HbSS) were found in 17.1% and 1.6% of children, respectively; 38.5% were heterozygotes and 9.6% were homozygotes for α(+) -thalassaemia. The Hp 2-2 genotype was found in 20.4% of children, whereas 8.2% of males and 6.8% of children overall had G6PD deficiency. There were no significant differences in the distribution of malaria by the measured blood disorders, except among males with G6PD deficiency who had a lower prevalence of clinical malaria than males of normal G6PD genotype (P = 0.005). After excluding children with malaria parasitaemia, inflammation (CRP > 5 mg L(-1) ), iron deficiency (ferritin < 12 μg L(-1) ) or vitamin A deficiency (RBP < 0.7 μg L(-1) ), the prevalence of anaemia among those without α(+) -thalassaemia (43.0%) remained significantly lower than that among children who were either heterozygotes (53.5%) or homozygotes (67.7%, P = 0.03). Inherited blood disorders are common among pre-school children in western Kenya and are important contributors to anaemia.
尽管遗传性血液疾病在非洲许多地区的儿童中很常见，但关于其流行程度或对儿童贫血的贡献的资料有限。我们对来自肯尼亚西部 60 个村庄的 858 名 6-35 个月大的儿童进行了横断面调查。从毛细血管血中测量血红蛋白（Hb）、铁蛋白、疟疾、C 反应蛋白（CRP）和视黄醇结合蛋白（RBP）。使用聚合酶链反应（PCR）确定 Hb 类型、-3.7kb 阿尔法珠蛋白链缺失、葡萄糖-6-磷酸脱氢酶（G6PD）基因型和结合珠蛋白（Hp）基因型。超过 2/3 的儿童至少有一种已测量的血液疾病。镰状细胞特征（HbAS）和疾病（HbSS）分别在 17.1%和 1.6%的儿童中发现；38.5%为杂合子，9.6%为α(+) -地中海贫血纯合子。Hp 2-2 基因型在 20.4%的儿童中发现，而 8.2%的男性和总体 6.8%的儿童存在 G6PD 缺乏症。除了 G6PD 缺乏症男性的临床疟疾患病率低于正常 G6PD 基因型的男性（P=0.005）外，所测量的血液疾病的疟疾分布没有显著差异。在排除疟疾寄生虫血症的儿童后，炎症（CRP>5mg·L(-1)）、铁缺乏（铁蛋白<12μg·L(-1)）或维生素 A 缺乏（RBP<0.7μg·L(-1)），无α(+) -地中海贫血的儿童（43.0%）贫血的患病率仍明显低于杂合子（53.5%）或纯合子（67.7%，P=0.03）。遗传性血液疾病在肯尼亚西部的学龄前儿童中很常见，是贫血的重要原因。
