Introduction:In view of the number of sickle cell patients and due to a low production
of descriptive studies, we decided to determine the prevalence of genes S and C
of the disease in the Zinder region. The objective was to contribute to
improving the management of sickle cell disease in Zinder. Methodology: This
was a systematic screening by the “Sickle Scan” test of any blood donor
admitted to the Zinder Regional Blood Transfusion Center during the 6-month
study period, from January to June 2023. The Sickle Scan is a qualitative
lateral flow chromatography immunoassay using whole blood samples that aid in
the rapid diagnosis of sickle cell disease. Results: The study was
carried out on 613 samples during the period concerned. The frequency of sickle
cell genes was 26.9% (n = 165) in all samples collected, with 23.1% (n = 142)
and 3.8% (n = 23) for the S gene and the C gene, respectively. The 18-30 age group was the most represented with 64.4% (n
= 395) cases. The median age of blood donors was 26 years ± 10 years (min = 18
years/max = 60 years). The sex ratio was 2.5. Donors of Nigerien nationality
accounted for 84.1% (n = 516). There is a predominance of blood donors with an
average monthly income between 34,000 and 70,000 CFA
francs in 44.3% (n = 272), lived in permanent housing with drinking water
supply. Sickle cell trait (SMA) was found in 22.5% (n = 138). Conclusion: The analysis of these results highlights a high frequency of the S gene for
sickle cell disease. The population with an average monthly income is the most
affected, with a male predominance.
References
[1]
Diagne, I., Somda, P.K., N’Diaye, A., Alson, O.R., et al. (2019) Guide pour la prise en charge de la drépanocytose en Afrique, 2019 e-drépanocytose.
[2]
World Health Organization (Fifty-Ninth World Health Assembly (A59/9)) (2006) Provisional Agenda Item 11.4. Sickle-Cell Anaemia: Prevalence of Sickle-Cell Anaemia.
[3]
Evan, M. and Braunstein, M.D. (2022) Sickle Cell Disease, MSD Manual.
[4]
Thiam, L., et al. (2017) Epidemiological, Clinical and Hematological Profiles of SS Homozygous Sickle Cell Disease in the Inter-Critical Phase in Children in Ziguinchor, Senegal. Pan African Medical Journal, 28, 208.
https://doi.org/10.11604/pamj.2017.28.208.14006
[5]
Streetly, A., Latinovic, R., Hall, K. and Henthorn, J. (2009) Implementation of Universal Newborn Bloodspot Screening for Sickle Cell Disease and Other Clinically Significant Haemoglobinopathies in England: Screening Results for 2005-7. Journal of Clinical Pathology, 62, 26-30. https://doi.org/10.1136/jcp.2008.058859
[6]
Piel, F.B., Patil, A.P., Howes, R.E., Nyangiri, O.A., Gething, P.W., Dewi, M., et al. (2013) Global Epidemiology of Sickle Haemoglobin in Neonates: A Contemporary Geostatistical Model-Based Map and Population Estimates. Lancet, 381, 142-151.
https://doi.org/10.1016/S0140-6736(12)61229-X
[7]
Honsel, V., Khimoud, D., Ranque, B., Offredo, L., Joseph, L., Pouchot, J., et al. (2019) Comparison between Adult Patients with Sickle Cell Disease of Sub-Saharan African Origin Born in Metropolitan France and in Sub-Saharan Africa. Journal of Clinical Medicine, 8, 2173. https://doi.org/10.3390/jcm8122173
[8]
Arlet, J.-B. (2023) Epidemiology of Sickle Cell Disease in France and Worldwide.
[9]
Piel, F.B., Tatem, A.J., Huang, Z., Gupta, S., Williams, T.N., Weatherall, D.J., et al. (2014) Global Migration and the Changing Distribution of Sickle Haemoglobin: A Quantitative Study of Temporal Trends between 1960 and 2000. Lancet Glob Health, 2, e80-89. https://doi.org/10.1016/S2214-109X(13)70150-5
[10]
Uyoga, S., Macharia, A.W., Mochamah, G., Ndila, C.M., Nyutu, G., Makale, J., et al. (2019) The Epidemiology of Sickle Cell Disease in Children Recruited in Infancy in Kilifi, Kenya: A Prospective Cohort Study. The Lancet Global Health, 7, e1458-e1466.
https://doi.org/10.1016/S2214-109X(19)30328-6
[11]
Ranque, B., Kitenge, R., Ndiaye, D.D., Ba, M.D., Adjoumani, L., Traore, H., et al. (2022) Estimating the Risk of Child Mortality Attributable to Sickle Cell Anaemia in Sub-Saharan Africa: A Retrospective, Multicentre, Case-Control Study. The Lancet Haematology, 9, e208-e216. https://doi.org/10.1016/S2352-3026(22)00004-7
[12]
Ataga, K.I., Kutlar, A., Kante,r J., et al. (2017) Crizanlizumab for the Prevention of Pain Crises in Sickle Cell Disease. The New England Journal of Medicine, 376, 429-439.
https://doi.org/10.1056/NEJMoa1611770
[13]
Niihara, Y., Miller, S.T., Kanter, J., et al. (2018) A Phase 3 Trial of L-Glutamine in Sickle Cell Disease. The New England Journal of Medicine, 379, 226-235.
https://doi.org/10.1056/NEJMoa1715971
[14]
Vichinsky, E., Hoppe, C.C., Ataga, K.I., et al. (2019) A Phase 3 Randomized Trial of Voxelotor in Sickle Cell Disease. The New England Journal of Medicine, 381, 509-519.
https://doi.org/10.1056/NEJMoa1903212
[15]
Boiro, D., Gueye, M., Thiongane, A, et al. (2016) Sickle Cell Disease in Children: Clinical and Evolutionary Profiles of 138 Cases Followed at the Pediatric Department of Abass Ndao Hospital in Dakar. Médecine d’Afrique Noire, 63, 326-332.
[16]
Samira, O. (2015) Homozygous Sickle Cell Disease in Children at the Provincial Hospital in Tangier: A Study of 10 Cases. Ph.D. Thesis, Mohammed V University of Rabat, Rabat.
[17]
Nacoulma, E.W.C., Sakande, J., Kafando, E., Kpowbié, E.D. and Guissou, I.P. (2006) Hematological and Biochemical Profiles of SS and SC Sickle Cell Patients in a Steady-State at the Yalgado Ouedraogo National Hospital Center in Ouagadougou. Mali Medical, 21, 8-11.
[18]
Thuilliez, V. and Vierin, Y. (1997) The Burden of Sickle Cell Disease in Pediatric Settings in Gabon. Public Health, 1, 45-60.
[19]
Dreux, O. (2012) Therapeutic Education for Sickle Cell Disease in Children: Rationale for Implementation and Initiation of This Project at the University Hospital of Grenoble. Ph.D. Thesis, Joseph Fournier University, Grenoble.
[20]
Nacoulma, E.W.C., Dembelele, B.P., Ye, D. and Kam, L. (2006) Major Sickle Cell Diseases in the Pediatric Department of Sourou Sanon University Hospital Center in Bobo Dioulasso. Médecine d’Afrique Noire, 53, 694-698.
[21]
Tolo, A., Touré, A., N’dhatz, E., et al. (2006) Evolutionary Profile of Followed Homozygous Sickle Cell Disease: Experience from the Clinical Hematology Department of the University Hospital Center in Yopougon. Médecine d’Afrique Noire, 53, 5-10.