Abstract
ObjectiveBy measuring the associations between the presence of sickle cell and β-thalassemia genes, we assessed the extent to which these hemoglobinopathies contribute to the high prevalence of anemia observed in preschool-aged children and women of reproductive age in Sierra Leone.ResultsThe prevalence of anemia was statistically significantly higher in children with homozygous sickle cell genes (HbSS) than in children with normal hemoglobin genes (HbAA or HbAC), but there was no difference in anemia prevalence in those with heterozygous sickle cell trait (HbAS or HbSC) compared with those with normal hemoglobin genes. In women, there was no difference in anemia prevalence by sickle cell status. In both children and women, there was no difference in the anemia prevalence for individuals with or without the β-thalassemia gene. For both sickle cell and β-thalassemia, there was no significant difference in hemoglobin concentrations by sickle cell or β-thalassemia status. Anemia prevalence was higher in children and women with homozygous sickle cell (HbSS). However, as the prevalence of HbSS children (5.4%) and women (1.6%) was quite small, it is unlikely that these hemoglobinopathies substantially contributed to the high anemia prevalence found in the 2013 national micronutrient survey.
Highlights
Sierra Leone’s Micronutrient Survey (SLMS) was conducted in late 2013
Children Results of sickle cell and β-thalassemia testing, along with the individual and household data required to calculate measures of precision, were available for 388 (59%) of the total sample of 654 children included in the SLMS who had contributed samples with sufficient volume for analysis of hemoglobinopathies
21 (5.4%; 95% confidence interval (CI) 3.7, 8.0) of 388 children tested for hemoglobinopathies had HbSS, 61 (15.7%; 95% CI 12.6, 19.5) had heterozygous sickle cell trait, and 306 (78.9%; 95% CI 74.8, 82.4) had either HbAA or HbAC. β-thalassemia was found in 64 (16.5%; 95% CI 12.7, 21.2) of 388 children
Summary
Children Results of sickle cell and β-thalassemia testing, along with the individual and household data required to calculate measures of precision, were available for 388 (59%) of the total sample of 654 children included in the SLMS who had contributed samples with sufficient volume for analysis of hemoglobinopathies. The mean hemoglobin concentrations in children with HbSS, heterozygous sickle cell trait, and normal hemoglobin are not statistically significantly different (see Table 2). No statistically significant difference in anemia prevalence was found between children with heterozygous sickle cell trait and those with either HbAA or HbAC. There was minimal difference in the mean hemoglobin concentration and the prevalence of anemia in children with versus without the β-thalassemia gene. The mean hemoglobin concentrations among women with heterozygous sickle cell trait or HbSS were 2.1 and 4.1 g/L, respectively, lower than concentrations in. There was no statistical significance in the differences in mean hemoglobin concentrations between women with β-thalassemia and those without this mutation. Women with HbSS had almost twice the risk of being anemic than women with HbAA or HbAC; this difference was not statistically significant. The prevalence of anemia in women without β-thalassemia was higher than in women with β-thalassemia, but with no statistical significance
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