Abstract

Sickle cell disease is a hereditary disease that predominantly affects black people. It is very widespread in sub-Saharan Africa, particularly at the Lehmann “sickle belt” level, where the prevalence of the hemoglobin S involves at least 10% of the population in West Africa and can reach 40% in Central Africa. In Côte d'Ivoire, the prevalence of the hemoglobin S is about 12–14% in the general population and about 11.71% in the child population in Abidjan. On the other hand, its coexistence with other hemoglobin phenotypes such as AC (6.2%) and β-thalassemia (2.7%) traits may also cause composite heterogeneous sickle cell disease, e.g., SC or S/β-thalassemia in this study. Since 2009, sickle cell disease has been recognized as a public health problem; however, much still remains to be performed despite the progress achieved. The objective of this study is thus to promote a rapid screening for the struggling against sickle cell disease in Côte d'Ivoire. This study was carried out over 6 months (April–September 2019) and has included 336 children, of which 236 all-comers, recruited in the municipality of Treichville in Abidjan and 100 other children with already known hemoglobin phenotype followed up in the Hematology Department of the University Hospital of Treichville. Two tests were used: the HemoTypeSC™ for rapid screening and the hemoglobin electrophoresis which is the reference method used for confirming the diagnosis in the laboratory. The findings confirmed the reliability of the HemoTypeSC™ with a sensitivity and specificity at 100% for the detection of hemoglobin A, S, and C. On the other hand, this sensitivity and specificity drop to 98.2% and 99.7%, respectively, when we analyze all the 336 children together, including the cases with HbF detected by hemoglobin electrophoresis. Hence, the importance of performing certainty tests following the HemoTypeSC™ screening test in order to determine the accurate phenotypes and proportions of the types of hemoglobin. The prevalence of hemoglobin S in subgroup 1 of 236 children of all-comers was 15%. The HemoTypeSC™ is therefore reliable, inexpensive, and disposable for rapid screening and early detection of sickle cell disease in Côte d'Ivoire. The HemoTypeSC™ provides rapid detection of hemoglobin phenotypes HbAA, HbSS, HbSC, HbCC, HbAS, and HbAC.

Highlights

  • Sickle cell disease is a genetic condition that affects 3% of the world’s population

  • In Cote d’Ivoire, studies have shown a prevalence of hemoglobin S between 12% and 14% in the general population according to Cabannes et al [3] in 1967 and about 12% in the infant population in Abidjan with a coexistence of AC (6.2%) and AFA2 or β-thalassemia (2.7%) traits according to Danho Bassimbieet al. in 1988 [4]

  • A total of 336 children were included in the study. e study population was divided into two subgroups: Subgroup 1 included 236 apparently healthy children all-comers, recruited in 2 schools and 1 maternal and child protection health center in the municipality of Treichville Subgroup 2 included 100 outpatients followed up in the hematology wards of the University Hospital of Treichville, in whom the hemoglobin phenotype was already known at the hemoglobin electrophoresis

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Summary

Introduction

Sickle cell disease is a genetic condition that affects 3% of the world’s population. About 75% of them live in sub-Saharan Africa, and more than 50% of children with this condition die before the age of 5 years [1, 2]. Sickle disease was recognized as a public health problem by the WHO in 2009. Ese hemoglobin defects are most often at the origin of major sickle cell syndromes commonly found in the country. Sickle cell disease has been recognized as a public health problem since 2009 (WHO). Despite notable progress in the treatment of major sickle cell syndromes, screening remains insufficient in Cote d’Ivoire. Hemoglobin electrophoresis, the reference test for the diagnosis of sickle cell anemia in the laboratory, remains generally

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