Incidence of Sickle Cell Disease and Other Hemoglobinopathies in Burkina Faso: Results of a Five-Year Systematic Neonatal Screening (2015-2019) in Four Urban Hospitals
Hemoglobinopathies,
mainly Sickle cell disease (SCD), are the most common monogenic disorders in
Africa. In Burkina Faso, data on these diseases are scarce, mainly hospital-based in Ouagadougou and its
surroundings. In order to assess the incidence and allelic frequencies of the
main hemoglobinopathies in newborns in Burkina Faso, we conducted a
cross-sectional study from 2015 to 2019 in four hospitals. The study included
babies of both sexes, regardless of ethnic group and parents’ hemoglobin
status. It was a newborn screening and hemoglobin variants were detected using
isoelectric focusing on cord blood samples and confirmed using hemoglobin
electrophoresis by high-performance liquid chromatography. The proportions
and cumulative incidences of the different hemoglobinopathies were computed.
Hardy-Weinberg equilibrium law was applied to calculate
genotypic and allelic frequencies. The significant level was p < 0.05. Out
of 11,337 newborns included, 47.8% were males and 60.2% were from Bobo-Dioulasso. Abnormal
hemoglobin was found in 27.1%, representing a cumulative incidence of 1:4
newborns. The incidence of SCD was 1.9% (1:53 newborns) with 27.9% of
homozygous SS. Homozygous CC and compound heterozygous Cβ-Thalassemia accounted for 1.1%. SCD cases were 1.51 times higher
in Bobo-Dioulasso (OR= 1.51;
95% CI [1.09 - 2.10]: p = 0.013). The
observed genotype frequencies were significantly different from the expected
ones (p<0.001). The βS and βC alleles represented 5.1 and 9.9%, respectively. This study showed
a high incidence of hemoglobinopathies. Such results raise the question of
control strategies for these hemoglobinopathies in our country.
References
[1]
Weatherall, D.J. and Clegg, J.B. (2001) Inherited Haemoglobin Disorders: An Increasing Global Health Problem. Bulletin of the World Health Organization, 79, 704-712.
[2]
World Health Organization (2006) Sickle-Cell Anaemia. WHO 59th World Health Assembly, New York, Report No. A59/9, 5 p.
[3]
Piel, F.B. (2015) Global Epidemiology of Sickle Cell Disease and the Thalassemias: New Management Challenges. Hematology Education, 9, 147-153.
[4]
Brousse, V., Allaf, B. and Benkerrou, M. (2021) Dépistage néonatal de la drépanocytose en France. Medical Sciences (Paris), 37, 482-490. https://doi.org/10.1051/medsci/2021056
[5]
Oron, A.P., Chao, D.L., Ezeanolue, E.E., Ezenwa, L.N., Piel, F.B., Ojogun, O.T., et al. (2020) Caring for Africa’s Sickle Cell Children: Will We Rise to the Challenge? BMC Medicine, 18, Article No. 92. https://doi.org/10.1186/s12916-020-01557-2
[6]
Simporé, J., Nikiema, J.B., Sawadogo, L., Pignatelli, S., Blot, I., Yara, J., et al. (2003) Prévalence des hémoglobinopathies HbS et HbC au Burkina Faso. Burkina Médical, 6, 99-107.
[7]
Traore, M., Zohoncon, T., Ouedraogo, P., Ouattara, A., Obiri-Yeboah, D., Tao, I., et al. (2020) Hemoglobin AE, AO-Arab and SO-Arab Genotypes in Burkina Faso: Hematological Parameters, Genotypic and Allelic Frequencies of Hemoglobinopathies. Journal of Human and Clinical Genetics, 2, 23-30. https://doi.org/10.29245/2690-0009/2020/1.1109
[8]
Nacoulma, E.W. (2007) Place de la drépanocytose dans le service de médecine interne du CHU Yalgado Ouedraogo de Ouagadougou (CHU-YO). Revue International des Sciences Médicales, 9, 54-58.
[9]
Kafando, E., Sawadogo, M., Cotton, F., Vertongen, F. and Gulbis, B. (2005) Newborn Screening for Sickle Cell Disorders in Ouagadougou, Burkina Faso: A Pilot Study. Journal of Medical Screening, 12, 112-114. https://doi.org/10.1258/0969141054855300
[10]
Modiano, D., Bancone, G., Ciminelli, B.M., Pompei, F., Blot, I., Simporé, J., et al. (2007) Haemoglobin S and Haemoglobin C: “Quick but Costly” versus “Slow but Gratis” Genetic Adaptations to Plasmodium falciparum Malaria. Human Molecular Genetics, 17, 789-799. https://doi.org/10.1093/hmg/ddm350
[11]
Modiano, D., Luoni, G., Sirima, B.S., Simporé, J., Verra, F., Konaté, A., et al. (2001) Haemoglobin C Protects against Clinical Plasmodium falciparum Malaria. Nature, 414, 305-308. https://doi.org/10.1038/35104556
[12]
Piel, F.B., Adamkiewicz, T.V., Amendah, D., Williams, T.N., Gupta, S. and Grosse, S.D. (2016) Observed and Expected Frequencies of Structural Hemoglobin Variants in Newborn Screening Surveys in Africa and the Middle East: Deviations from Hardy-Weinberg Equilibrium. Genetics in Medicine, 18, 265-274. https://doi.org/10.1038/gim.2015.143
[13]
Makalou, D., Doupa, D., Seck, N., Thiam, O., Guèye, M.W., Coly, M.N., et al. (2020) Dépistage néonatal de la drépanocytose et autres hémoglobinopathies à Saint Louis: Résultats préliminaires. Revue Africaine de Biologie Médicale, 5, 807-813.
[14]
Diallo, D.A., Guindo, A., Touré, B.A., Sarro, Y.S., Sima, M., Tessougué, O., et al. (2018) Dépistage néonatal ciblé de la drépanocytose: Limites du test de falciformation (test d’Emmel) dans le bilan prénatal en zone ouest africaine. Revue d’épidémiologie et de Santé Publique, 66, 181-185. https://doi.org/10.1016/j.respe.2018.02.007
[15]
Tshilolo, L., Aissi, L.M., Lukusa, D., Kinsiama, C., Wembonyama, S., Gulbis, B., et al. (2009) Newborn Screening for Sickle Cell Anaemia in the Democratic Republic of the Congo: Experience from a Pioneer Project on 31204 Newborns. Journal of Clinical Pathology, 62, 35-38. https://doi.org/10.1136/jcp.2008.058958
[16]
Odunvbun, M.E., Okolo, A.A. and Rahimy, C.M. (2008) Newborn Screening for Sickle Cell Disease in a Nigerian Hospital. Public Health, 122, 1111-1116. https://doi.org/10.1016/j.puhe.2008.01.008
[17]
Vierin Nzamz, Y., Boussougou Bu, B.I., Koko, J., Blot, P. and Moussavou, A. (2012) Dépistage néonatal de la drépanocytose au Gabon. Médecine d’Afrique Noire, 59, 95-99.
[18]
Shongo, M.Y.-P. and Mukuku, O. (2018) Dépistage néonatal de la drépanocytose à Lubumbashi, République Démocratique du Congo. Revue de l’Infirmier Congolais, 2, 62-63.
[19]
Ayéroué, J., Kafando, E., Kam, L., Gué, E., Vertongen, F., Ferster, A., et al. (2009) Le syndrome drépanocytaire de type hémoglobine SC: Expérience du CHU Yalgado Ouédraogo de Ouagadougou (Burkina Faso). Archives de Pédiatrie, 16, 316-321. https://doi.org/10.1016/j.arcped.2009.01.005
[20]
Souza, R.C., Miranda Neto, P.A.D., Santos, J.R.N., Monteiro, S.G., Gonçalves, M.C., Silva, F.B., et al. (2019) Sickle Cell Anaemia Prevalence among Newborns in the Brazilian Amazon-Savanna Transition Region. International Journal of Environmental Research and Public Health, 16, Article No. 1638. https://doi.org/10.3390/ijerph16091638
[21]
Nnodu, O.E., Adegoke, S.A., Ezenwosu, O.U., Emodi, I.I., Ugwu, N.I., Ohiaeri, C.N., et al. (2018) A Multi-Centre Survey of Acceptability of Newborn Screening for Sickle Cell Disease in Nigeria. Cureus, 10, e2354. https://doi.org/10.7759/cureus.2354
[22]
Rahimy, M.C., Gangbo, A., Ahouignan, G. and Alihonou, E. (2009) Newborn Screening for Sickle Cell Disease in the Republic of Benin. Journal of Clinical Pathology, 62, 46-48. https://doi.org/10.1136/jcp.2008.059113
[23]
Nnodu, O.E., Sopekan, A., Nnebe-Agumadu, U., Ohiaeri, C., Adeniran, A., Shedul, G., et al. (2020) Implementing Newborn Screening for Sickle Cell Disease as Part of Immunisation Programmes in Nigeria: A Feasibility Study. The Lancet Haematology, 7, e534-e540. https://doi.org/10.1016/S2352-3026(20)30143-5
