Author(s): Hamadou DJIBO ,Sina IDE, ÃaÄdaÅ Erkan AKYÃREK, Åükrü Anıl TOYGAR
The aim of this study is to determine the prevalence and gene frequency of Hemoglobin Constant Spring (HbS) and other forms of thalassemia among the users of biochemistry laboratory of the faculty of health sciences of Niamey/Niger. We realized, at the laboratory of biochemistry of the Faculty of Science and Health of Niamey/Niger from 1988 to 2010, a retrospective study which concerned in-patients, external patients and groups of population apparently healthy for which the electrophoresis of the hemoglobin is required were used. An eligible sample of 14.389 subjects was included in the analysis. Data analysis was done by descriptive statistics. The expanding subjects of a defect hemoglobin are among 58.8 %. The hemoglobin S was identified to 54, 96 % under the forms AS, SC, SS. Besides phenotypes, SC and SS which are the most spread, we identified other rare associations of the hemoglobin S, associations’ thalassotherapy-sickle cell diseases. The expanding subjects of it ß + thalassotherapy-sickle cell disease represents 1.1 % and occupies the third place among the major sickle cell diseases met in Niger. Although this hemoglobinopathies is not at present curable, complete programs of fight centered on the prevention, the treatment and the support can be set up, at every level, and allow to improve as well the quality of life as the life expectancy of the people suffering from these pathologies. Age and locality factor play a role on Hemoglobinopathies in Niger. The findings indicate that there are serious impacts on children and remain neglected pathologies. The Niger government should focus on funding and providing the treatment and support have to be set up at all the levels, to allow improvement in terms of life quality as well as the life expectancy of the people suffering from these pathologies.
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