Epidemiologic profile of hemoglobinopathies in Benin.

Hematology, transfusion and cell therapy Pub Date : 2024-10-08 DOI:10.1016/j.htct.2024.07.008

Selma Gomez, Adjile Edjide Roukiyath Amoussa, Edwige Dedjinou, Manasse Kakpo, Pélagie Gbédji, Nouhoum Amossou Soulé, Bernice Quenum

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Abstract

Background: Sickle cell disease is the most common inherited blood disorder in the world with the birth of approximately 300,000 newborns screened each year. In 2009, the World Health Organization ranked the fight against sickle cell disease among the priorities for the Africa regions. The best way to prevent this incurable disease remains, on one hand systematic screening at birth, and on the other the proscription of risky union between heterozygous subjects.

Aim: The aim of this study was to analyze the epidemiological profile of sickle cell disease and other hemoglobinopathies in Benin and determine more up-to-date prevalence rates of the disease within the population.

Methods: The hemoglobin profiles of 2910 study participants were determined by quantitative electrophoresis. Samples with abnormal hemoglobin results were subjected to a complete blood count.

Results: Our study population was balanced between males (1528) and females (1382) with a sex ratio of 1.1. The mean age ranged from eight years in the pediatric group to 26 years in adults. The hemoglobin electrophoresis profiles found were as follows: 59.7 % Hb AA (normal), 21.7 % Hb AS, 10.2 % Hb AC, 3.1 % Hb SS, 3.7 % Hb SC, and 1.6 % of the rare phenotypes (Hb AD, Hb AE, Hb AF, Hb A/β-thal, Hb SD, Hb SF, Hb CC and Hb C/β-thal). Participants with abnormal hemoglobin presented a normochromic normocytic anemia. A total of 356 (12 %) people knew their profile compared to 2554 (88 %) who did not.

Conclusion: The high prevalence of hemoglobinopathies found in this study highlights in importance of screening in the Benin population.

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贝宁血红蛋白病的流行病学概况。

背景：镰状细胞病是世界上最常见的遗传性血液疾病，每年约有 30 万新生儿接受筛查。2009 年，世界卫生组织将防治镰状细胞病列为非洲地区的优先事项之一。预防这种不治之症的最佳方法，一方面是在新生儿出生时进行系统筛查，另一方面是禁止杂合子之间的危险结合。目的：本研究旨在分析贝宁镰状细胞病和其他血红蛋白病的流行病学概况，并确定该疾病在人口中的最新患病率：采用定量电泳法测定了 2910 名研究参与者的血红蛋白状况。对血红蛋白结果异常的样本进行了全血细胞计数：我们的研究对象中男性（1528 人）和女性（1382 人）比例均衡，性别比为 1.1。平均年龄从儿童组的 8 岁到成人组的 26 岁不等。血红蛋白电泳图谱如下：59.7% 为血红蛋白 AA（正常），21.7% 为血红蛋白 AS，10.2% 为血红蛋白 AC，3.1% 为血红蛋白 SS，3.7% 为血红蛋白 SC，1.6% 为罕见表型（血红蛋白 AD、血红蛋白 AE、血红蛋白 AF、血红蛋白 A/β-thal、血红蛋白 SD、血红蛋白 SF、血红蛋白 CC 和血红蛋白 C/β-thal）。血红蛋白异常的参与者表现为正常色素性正常细胞性贫血。共有 356 人（12%）知道自己的情况，而不知道的有 2554 人（88%）：本研究发现血红蛋白病的发病率很高，这凸显了在贝宁人口中进行筛查的重要性。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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Hematology, transfusion and cell therapy

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