Sickle-cell disease epidemiology and demographics
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shyam Patel [2]
Overview
Sickle cell disease affects millions of patients throughout the world.[1] Sickle cell disease is known to have a high prevalence in Africa (specifically the sub-Saharan region), India, and the Middle East.[2] Worldwide, the current prevalence of sickle cell disease is 20 million, and nearly 50% of affected people are in Africa. The prevalence of sickle cell disease in the United States is relatively low; sickle-cell disease affects approximately 100,000 people.[3]
Epidemiology and Demographics
Prevalence
- Sickle cell disease affects millions of patients throughout the world.[1] Sickle cell disease is known to have a high prevalence in Africa (specifically the sub-Saharan region), India, and the Middle East.[2]
- Worldwide, the current prevalence of sickle cell disease is 20 million, and nearly 50% of affected people are in Africa. India comprises 5-10 million people with sickle cell disease. Regarding allele frequencies, the S (glutamic acid to valine) and C (glutamic acid to lysine) alleles are highest in Africa.[4]
- The prevalence of sickle cell disease in the United States is relatively low; sickle-cell disease affects approximately 100,000 people.[3] Sickle-cell disease affects 1 in 400 African Americans.[5]
Incidence
- The incidence of sickle cell disease is 312,000 per year worldwide. Among these, 76% (236,000 people) are born in sub-Saharan Africa.[3]
Developed Countries
- HbS-beta-thalassemia is commonly found in Greece.[3]
Developing Countries
- People from India have higher frequencies of sickle cell alleles compared to others. The HbS gene is sometimes associated with the Arab-India beta-globin cluster phenotype.[2] Compared to people from Africa, those from India have a lower frequency of the HbSS genotype (30-70%) and a higher frequency of sickle cell-beta-thalassemia (HbSB) genotype.[3]
- The heterozygote prevalence (HbAS) in India of sickle disease varies from 1-40%. Certain tribal populations in India have high prevalences of sickle cell trait and/or disease. For more information on the frequency of sickle-cell trait in India, please click here.[6]
- The frequency of sickle cell alleles are highest in regions of the country where malaria is endemic.[3]
- Besides Africa and India, other geographic regions are affected by particular sickle cell phenotypes. The nomenclature of certain sickle cell hemoglobinopathies and haplotypes is based on the region in which the prevalence is high.
- The HbSC phenotype, for example, is prevalent in geographic areas such as Burkina Faso.[3]
- Hemoglobin Senegal has been found in Senegal. Others include hemoglobin Benin and hemoglobin Bantu.[4]
References
- ↑ 1.0 1.1 Alapan Y, Kim C, Adhikari A, Gray KE, Gurkan-Cavusoglu E, Little JA; et al. (2016). "Sickle cell disease biochip: a functional red blood cell adhesion assay for monitoring sickle cell disease". Transl Res. 173: 74–91.e8. doi:10.1016/j.trsl.2016.03.008. PMC 4959913. PMID 27063958.
- ↑ 2.0 2.1 2.2 Ngo D, Bae H, Steinberg MH, Sebastiani P, Solovieff N, Baldwin CT; et al. (2013). "Fetal hemoglobin in sickle cell anemia: genetic studies of the Arab-Indian haplotype". Blood Cells Mol Dis. 51 (1): 22–6. doi:10.1016/j.bcmd.2012.12.005. PMC 3647015. PMID 23465615.
- ↑ 3.0 3.1 3.2 3.3 3.4 3.5 3.6 Saraf SL, Molokie RE, Nouraie M, Sable CA, Luchtman-Jones L, Ensing GJ; et al. (2014). "Differences in the clinical and genotypic presentation of sickle cell disease around the world". Paediatr Respir Rev. 15 (1): 4–12. doi:10.1016/j.prrv.2013.11.003. PMC 3944316. PMID 24361300.
- ↑ 4.0 4.1 Makani J, Ofori-Acquah SF, Nnodu O, Wonkam A, Ohene-Frempong K (2013). "Sickle cell disease: new opportunities and challenges in Africa". ScientificWorldJournal. 2013: 193252. doi:10.1155/2013/193252. PMC 3988892. PMID 25143960.
- ↑ Brandow AM, Liem R (2011). ""Sickle Cell Disease in the Emergency Department: Atypical Complications and Management"". Clin Pediatr Emerg Med. 12 (3): 202–212. doi:10.1016/j.cpem.2011.07.003. PMC 3172721. PMID 21927581.
- ↑ Colah RB, Mukherjee MB, Martin S, Ghosh K (2015). "Sickle cell disease in tribal populations in India". Indian J Med Res. 141 (5): 509–15. PMC 4510747. PMID 26139766.