苏丹西部的镰状细胞病：遗传流行病学及知识、态度和行为的预测因素
Sickle cell disease in western Sudan: genetic epidemiology and predictors of knowledge attitude and practices.
作者信息
Daak Ahmed A, Elsamani Elfatih, Ali Eltigani H, Mohamed Fatma A, Abdel-Rahman Manar E, Elderdery Abozer Y, Talbot Octavious, Kraft Peter, Ghebremeskel Kebreab, Elbashir Mustafa I, Fawzi Wafaie
机构信息
Department of Global Health and Population, Harvard University, Boston, MA, USA.
Department of Medical Biochemistry, Faculty of Medicine, University of Khartoum, Khartoum, Sudan.
出版信息
Trop Med Int Health. 2016 May;21(5):642-53. doi: 10.1111/tmi.12689. Epub 2016 Mar 29.
OBJECTIVE
To investigate the epidemiology of sickle cell disease (SCD) and determinants of knowledge, attitudes and practices (KAP) towards SCD in western Kordofan State, Sudan.
METHODS
A community-based, descriptive, cross-sectional study was conducted in three towns. Three hundred and seventy-two households were polled, and blood samples for haemoglobin phenotyping were collected from 1116 individuals. Sociodemographic, socio-economic and KAP data were collected using investigator-administered questionnaires. Descriptive, frequency distribution and multiple regression analyses were performed.
RESULTS
About 50.9% of the study population were Misseriya tribes. Consanguineous marriages were reported by 67.5% of the households. The highest percentage of homozygous SCD was 2.8% among children under 5 years of age. About 24.9% were carriers of HbS allele (HbAS). HbS allele frequency was highest in children aged 5-11 years (18.3%, CI: 13.7-22.9%) and lowest in males >15 years old (12.0%, CI: 6.1-17.9%). The average HbS frequency across all age groups was 14.5% (95% CI: 12.2-16.8%). The most frequent β-globin gene cluster haplotype was the Cameroon (30.8%), followed by the Benin (21.8%), the Senegal (12.8%) and the Bantu (2.2%) haplotypes. About 17.0% of all-cause child deaths were due to SCD. The estimated change in log odds of having the SS genotype per year increase in age was (-) 0.0058 (95% CI -0.0359, 0.0242). This represents a non-statistically significant 2.9% increase in 5-year mortality for individuals with the SS genotype relative to those with AS and AA genotypes. About 46.9% of the households had poor knowledge, 26.1% had satisfactory knowledge, and 26.9% had good knowledge about sickle cell disease. Mothers' and fathers' educational levels were significant predictors of good knowledge about SCD (P < 0.05). About 48.0% had a satisfactory attitude towards sickle cell disease while 30.7% had poor attitude and only 21.3 showed good attitudes. Poor knowledge about SCD and low socio-economic status were the strongest positive predictors of poor attitude and practices towards SCD (P < 0.01).
CONCLUSIONS
Sickle cell disease is a major health problem in West Kordofan, Sudan. Knowledge, attitude and practices towards the disease are not satisfactory. The development of public health programs is highly recommended to control and manage SCD in western parts of Sudan.
目的
调查苏丹科尔多凡州西部镰状细胞病（SCD）的流行病学情况以及对SCD的知识、态度和行为（KAP）的决定因素。
方法
在三个城镇开展了一项基于社区的描述性横断面研究。对372户家庭进行了调查，并从1116名个体中采集了用于血红蛋白表型分析的血样。使用调查员管理的问卷收集社会人口统计学、社会经济和KAP数据。进行了描述性、频率分布和多元回归分析。
结果
约50.9%的研究人群为米塞里亚部落。67.5%的家庭报告有近亲结婚情况。5岁以下儿童中纯合子SCD的最高比例为2.8%。约24.9%为HbS等位基因（HbAS）携带者。HbS等位基因频率在5-11岁儿童中最高（18.3%，置信区间：13.7-22.9%），在15岁以上男性中最低（12.0%，置信区间：6.1-17.9%）。所有年龄组的平均HbS频率为14.5%（95%置信区间：12.2-16.8%）。最常见的β-珠蛋白基因簇单倍型是喀麦隆型（30.8%），其次是贝宁型（21.8%）、塞内加尔型（12.8%）和班图型（2.2%）单倍型。所有原因导致的儿童死亡中约17.0%归因于SCD。年龄每增加一岁，患SS基因型的对数优势估计变化为（-）0.0058（95%置信区间-0.0359，0.0242）。这表明与AS和AA基因型个体相比，SS基因型个体的5年死亡率非统计学显著增加2.9%。约46.9%的家庭对镰状细胞病知识了解不足，26.1%知识掌握情况令人满意，26.9%知识掌握良好。母亲和父亲的教育水平是对SCD知识掌握良好的显著预测因素（P<0.05）。约48.0%的人对镰状细胞病态度令人满意，而30.7%态度不佳，只有21.3%态度良好。对SCD知识了解不足和社会经济地位低是对SCD态度和行为不佳的最强正向预测因素（P<0.01）。
结论
镰状细胞病是苏丹科尔多凡州西部的一个主要健康问题。对该疾病的知识、态度和行为情况并不令人满意。强烈建议制定公共卫生项目以控制和管理苏丹西部的SCD。
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