Zohoun Alban, Baglo Agbodande Tatiana, Zohoun Lutécia, Anani Ludovic
Faculté des Sciences de la Santé, Centre National Hospitalier Universitaire-Hubert Koutoukou Maga, Cotonou, Bénin; Hôpital d'Instruction des Armées de Cotonou, Centre Hospitalier Universitaire Cotonou, Bénin.
Faculté des Sciences de la Santé, Centre National Hospitalier Universitaire-Hubert Koutoukou Maga, Cotonou, Bénin.
Hematol Transfus Cell Ther. 2020 Apr-Jun;42(2):145-149. doi: 10.1016/j.htct.2019.06.005. Epub 2019 Sep 7.
Sickle cell disease is the most common monogenic disorder in humans and is a major public health concern in sub-Saharan Africa. In Benin, the prevalence of sickle cell disease is estimated to be 4.8%. Our study aimed to describe the prevalence of hemoglobin abnormalities in an apparently healthy Benin population.
One thousand four hundred and eighty-three men and women, apparently in good health after medical screening, were tested for hemoglobin abnormalities by hemoglobin electrophoresis and the Emmel test. Subjects who were found to have homozygous or double heterozygous hemoglobin abnormalities, were re-sampled and a confirmation hemogram and hemoglobin electrophoresis test by capillary electrophoresis was performed.
Our study population was predominantly male (97.7%) with an average age of 21.3 years. 1390 subjects reported that they did not know their hemoglobin electrophoresis status. Hemoglobin electrophoresis profiles found were as follows: 1077 (72.6%) AA (normal), 238 (16.1%) AS, 161 (10.9%) AC, 3 (0.2%) SC, 4 (0.2%) CC and 0 (0%) SS. The 406 subjects with abnormal hemoglobin had balanced somatic growth, with general physical examination results showing no abnormalities. In the seven subjects with major sickle cell syndrome or hemoglobinosis (SC and CC), their values of various hemogram parameters were normal apart from the discreet presence of microcytic anemia.
Our study highlights the need for increased routine testing of hemoglobin abnormalities and newborn screening for sickle cell disease in order to enhance early disease detection, prevention and comprehensive care.
镰状细胞病是人类最常见的单基因疾病，也是撒哈拉以南非洲地区主要的公共卫生问题。在贝宁，镰状细胞病的患病率估计为4.8%。我们的研究旨在描述贝宁一个看似健康的人群中血红蛋白异常的患病率。
对1483名经医学筛查后看似健康的男性和女性进行血红蛋白电泳和埃默尔试验，以检测血红蛋白异常情况。对发现有纯合或双重杂合血红蛋白异常的受试者重新采样，并通过毛细管电泳进行确认血常规和血红蛋白电泳试验。
我们的研究人群以男性为主（97.7%），平均年龄为21.3岁。1390名受试者表示他们不知道自己的血红蛋白电泳状态。发现的血红蛋白电泳谱如下：1077例（72.6%）AA（正常），238例（16.1%）AS，161例（10.9%）AC，3例（0.2%）SC，4例（0.2%）CC，0例（0%）SS。406名血红蛋白异常的受试者身体生长平衡，一般体格检查结果无异常。在7例患有主要镰状细胞综合征或血红蛋白病（SC和CC）的受试者中，除了存在轻微的小细胞贫血外，他们的各项血常规参数值均正常。
我们的研究强调需要加强血红蛋白异常的常规检测和镰状细胞病的新生儿筛查，以加强疾病的早期检测、预防和综合护理。
