Burden of sickle cell trait and disease in the Uganda Sickle Surveillance Study (US3): a cross-sectional study
Burden of sickle cell trait and disease in the Uganda Sickle Surveillance Study (US3): a cross-sectional study
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DOI：
10.1016/s2214-109x(15)00288-0
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发表时间：
2016-03-01
影响因子：
34.3
通讯作者：
Aceng, Jane R.
中科院分区：
文献类型：
--
作者：
Ndeezi, Grace;Kiyaga, Charles;Aceng, Jane R.
镰状细胞病是撒哈拉以南非洲5岁以下儿童死亡率的主要原因。在乌干达，每年有2万名婴儿被认为患有镰状细胞病，但没有准确的数据。我们做了横断面乌干达镰状细胞监测研究，以评估疾病的负担。方法该研究的主要目的是计算镰状细胞性状和疾病的患病率。我们从乌干达10个地区和112个地区的艾滋病毒暴露婴儿中常规收集的干血斑中获得了打孔样本，用于国家早期婴儿诊断计划。通过等电聚焦对所有样本进行血红蛋白电泳，以识别患有镰状性状或疾病的婴儿。结果在2014年2月至2015年3月期间，分析了99 243个干血斑，并提供了97 631个结果。患有镰状细胞性状的儿童总数为12 979人（13.3%），患病儿童总数为716人（0.7%）。镰状细胞数量从西南部地区的13649例中的631例（4.6%）和23例（0.2%）到东部中部地区的6581例中的1306例（19.8%）和96例（1.5%）。镰状细胞性状在所有地区都有发现。最低的两个区的患病率低于3.0%。有8个区的患病率大于20.0%，最高为23.9%。镰状细胞病是不常见的儿童年龄超过12个月或谁是HIV阳性，这是一致的合并症和早期mortality.Interpretation镰状细胞性状和疾病的患病率高，在乌干达，地区和地区之间的显着变化。这些数据将有助于为包括新生儿筛查在内的镰状细胞病国家战略提供信息。(C)Ndeezi et al.根据CC BY-NC-ND条款分发的开放获取文章。
Background Sickle cell disease contributes substantially to mortality in children younger than 5 years in sub-Saharan Africa. In Uganda, 20 000 babies per year are thought to be born with sickle cell disease, but accurate data are not available. We did the cross-sectional Uganda Sickle Surveillance Study to assess the burden of disease.Methods The primary objective of the study was to calculate prevalence of sickle cell trait and disease. We obtained punch samples from dried blood spots routinely collected from HIV-exposed infants in ten regions and 112 districts across Uganda for the national Early Infant Diagnosis programme. Haemoglobin electrophoresis by isoelectric focusing was done on all samples to identify those from babies with sickle trait or disease.Findings Between February, 2014, and March, 2015, 99 243 dried blood spots were analysed and results were available for 97 631. The overall number of children with sickle cell trait was 12 979 (13.3%) and with disease was 716 (0.7%). Sickle cell numbers ranged from 631 (4.6%) for trait and 23 (0.2%) for disease of 13 649 in the South Western region to 1306 (19.8%) for trait and 96 (1.5%) for disease of 6581 in the East Central region. Sickle cell trait was seen in all districts. The lowest prevalence was less than 3.0% in two districts. Eight districts had prevalence greater than 20.0%, with the highest being 23.9%. Sickle cell disease was less common in children older than 12 months or who were HIV positive, which is consistent with comorbidity and early mortality.Interpretation Prevalence of sickle cell trait and disease were high in Uganda, with notable variation between regions and districts. The data will help to inform national strategies for sickle cell disease, including neonatal screening. (C) Ndeezi et al. Open Access article distributed under the terms of CC BY-NC-ND.