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==Overview==
==Overview==  
Sickle cell disease affects millions of patients throughout the world.<ref name="pmid27063958">{{cite journal| author=Alapan Y, Kim C, Adhikari A, Gray KE, Gurkan-Cavusoglu E, Little JA et al.| title=Sickle cell disease biochip: a functional red blood cell adhesion assay for monitoring sickle cell disease. | journal=Transl Res | year= 2016 | volume= 173 | issue=  | pages= 74-91.e8 | pmid=27063958 | doi=10.1016/j.trsl.2016.03.008 | pmc=4959913 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27063958  }} </ref> Sickle cell disease is known to have a high [[prevalence]] in Africa (specifically the sub-Saharan region), India, and the Middle East.<ref name="pmid23465615">{{cite journal| author=Ngo D, Bae H, Steinberg MH, Sebastiani P, Solovieff N, Baldwin CT et al.| title=Fetal hemoglobin in sickle cell anemia: genetic studies of the Arab-Indian haplotype. | journal=Blood Cells Mol Dis | year= 2013 | volume= 51 | issue= 1 | pages= 22-6 | pmid=23465615 | doi=10.1016/j.bcmd.2012.12.005 | pmc=3647015 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23465615  }} </ref> 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.<ref name="pmid24361300">{{cite journal| author=Saraf SL, Molokie RE, Nouraie M, Sable CA, Luchtman-Jones L, Ensing GJ et al.| title=Differences in the clinical and genotypic presentation of sickle cell disease around the world. | journal=Paediatr Respir Rev | year= 2014 | volume= 15 | issue= 1 | pages= 4-12 | pmid=24361300 | doi=10.1016/j.prrv.2013.11.003 | pmc=3944316 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24361300  }} </ref>


==Epidemiology and Demographics==
==Epidemiology and Demographics==
===Prevalence===
* Sickle cell disease affects millions of patients throughout the world.<ref name="pmid27063958">{{cite journal| author=Alapan Y, Kim C, Adhikari A, Gray KE, Gurkan-Cavusoglu E, Little JA et al.| title=Sickle cell disease biochip: a functional red blood cell adhesion assay for monitoring sickle cell disease. | journal=Transl Res | year= 2016 | volume= 173 | issue=  | pages= 74-91.e8 | pmid=27063958 | doi=10.1016/j.trsl.2016.03.008 | pmc=4959913 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27063958  }} </ref> Sickle cell disease is known to have a high [[prevalence]] in Africa (specifically the sub-Saharan region), India, and the Middle East.<ref name="pmid23465615">{{cite journal| author=Ngo D, Bae H, Steinberg MH, Sebastiani P, Solovieff N, Baldwin CT et al.| title=Fetal hemoglobin in sickle cell anemia: genetic studies of the Arab-Indian haplotype. | journal=Blood Cells Mol Dis | year= 2013 | volume= 51 | issue= 1 | pages= 22-6 | pmid=23465615 | doi=10.1016/j.bcmd.2012.12.005 | pmc=3647015 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23465615  }} </ref>
* 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.<ref name="pmid25143960">{{cite journal| author=Makani J, Ofori-Acquah SF, Nnodu O, Wonkam A, Ohene-Frempong K| title=Sickle cell disease: new opportunities and challenges in Africa. | journal=ScientificWorldJournal | year= 2013 | volume= 2013 | issue=  | pages= 193252 | pmid=25143960 | doi=10.1155/2013/193252 | pmc=3988892 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25143960  }} </ref>
* The [[prevalence]] of sickle cell disease in the United States is relatively low; sickle-cell disease affects approximately 100,000 people.<ref name="pmid24361300">{{cite journal| author=Saraf SL, Molokie RE, Nouraie M, Sable CA, Luchtman-Jones L, Ensing GJ et al.| title=Differences in the clinical and genotypic presentation of sickle cell disease around the world. | journal=Paediatr Respir Rev | year= 2014 | volume= 15 | issue= 1 | pages= 4-12 | pmid=24361300 | doi=10.1016/j.prrv.2013.11.003 | pmc=3944316 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24361300  }} </ref> Sickle-cell disease affects 1 in 400 African Americans.<ref name="pmid21927581">{{cite journal| author=Brandow AM, Liem R| title="Sickle Cell Disease in the Emergency Department: Atypical Complications and Management" | journal=Clin Pediatr Emerg Med | year= 2011 | volume= 12 | issue= 3 | pages= 202-212 | pmid=21927581 | doi=10.1016/j.cpem.2011.07.003 | pmc=3172721 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21927581  }} </ref>
===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.<ref name="pmid24361300">{{cite journal| author=Saraf SL, Molokie RE, Nouraie M, Sable CA, Luchtman-Jones L, Ensing GJ et al.| title=Differences in the clinical and genotypic presentation of sickle cell disease around the world. | journal=Paediatr Respir Rev | year= 2014 | volume= 15 | issue= 1 | pages= 4-12 | pmid=24361300 | doi=10.1016/j.prrv.2013.11.003 | pmc=3944316 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24361300  }} </ref>
===Developed Countries===
* HbS-beta-thalassemia is commonly found in Greece.<ref name="pmid24361300">{{cite journal| author=Saraf SL, Molokie RE, Nouraie M, Sable CA, Luchtman-Jones L, Ensing GJ et al.| title=Differences in the clinical and genotypic presentation of sickle cell disease around the world. | journal=Paediatr Respir Rev | year= 2014 | volume= 15 | issue= 1 | pages= 4-12 | pmid=24361300 | doi=10.1016/j.prrv.2013.11.003 | pmc=3944316 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24361300  }} </ref>
===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]].<ref name="pmid23465615">{{cite journal| author=Ngo D, Bae H, Steinberg MH, Sebastiani P, Solovieff N, Baldwin CT et al.| title=Fetal hemoglobin in sickle cell anemia: genetic studies of the Arab-Indian haplotype. | journal=Blood Cells Mol Dis | year= 2013 | volume= 51 | issue= 1 | pages= 22-6 | pmid=23465615 | doi=10.1016/j.bcmd.2012.12.005 | pmc=3647015 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23465615  }} </ref> 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.<ref name="pmid24361300">{{cite journal| author=Saraf SL, Molokie RE, Nouraie M, Sable CA, Luchtman-Jones L, Ensing GJ et al.| title=Differences in the clinical and genotypic presentation of sickle cell disease around the world. | journal=Paediatr Respir Rev | year= 2014 | volume= 15 | issue= 1 | pages= 4-12 | pmid=24361300 | doi=10.1016/j.prrv.2013.11.003 | pmc=3944316 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24361300  }} </ref>
* The heterozygote [[prevalence]] (HbAS) in India of sickle disease varies from 1-40%. Certain tribal populations in India have high [[prevalence]] of [[sickle cell trait]] and/or disease. For more information on the frequency of sickle-cell trait in India, please click [http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4510747/bin/IJMR-141-509-g001.jpg here].<ref name="pmid26139766">{{cite journal| author=Colah RB, Mukherjee MB, Martin S, Ghosh K| title=Sickle cell disease in tribal populations in India. | journal=Indian J Med Res | year= 2015 | volume= 141 | issue= 5 | pages= 509-15 | pmid=26139766 | doi= | pmc=4510747 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26139766  }} </ref>
* The frequency of sickle cell [[Allele|alleles]] are highest in regions of the country where [[malaria]] is endemic.<ref name="pmid24361300">{{cite journal| author=Saraf SL, Molokie RE, Nouraie M, Sable CA, Luchtman-Jones L, Ensing GJ et al.| title=Differences in the clinical and genotypic presentation of sickle cell disease around the world. | journal=Paediatr Respir Rev | year= 2014 | volume= 15 | issue= 1 | pages= 4-12 | pmid=24361300 | doi=10.1016/j.prrv.2013.11.003 | pmc=3944316 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24361300  }} </ref>
* 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.<ref name="pmid24361300">{{cite journal| author=Saraf SL, Molokie RE, Nouraie M, Sable CA, Luchtman-Jones L, Ensing GJ et al.| title=Differences in the clinical and genotypic presentation of sickle cell disease around the world. | journal=Paediatr Respir Rev | year= 2014 | volume= 15 | issue= 1 | pages= 4-12 | pmid=24361300 | doi=10.1016/j.prrv.2013.11.003 | pmc=3944316 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24361300  }} </ref>
* Hemoglobin Senegal has been found in Senegal. Others include hemoglobin Benin and hemoglobin Bantu.<ref name="pmid25143960">{{cite journal| author=Makani J, Ofori-Acquah SF, Nnodu O, Wonkam A, Ohene-Frempong K| title=Sickle cell disease: new opportunities and challenges in Africa. | journal=ScientificWorldJournal | year= 2013 | volume= 2013 | issue=  | pages= 193252 | pmid=25143960 | doi=10.1155/2013/193252 | pmc=3988892 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25143960  }} </ref>


==References==
==References==
{{reflist|2}}
{{reflist|2}}
[[Category:Hematology]]
{{WH}}
{{WS}}

Latest revision as of 15:01, 1 August 2018

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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 prevalence 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. ↑ 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. ↑ 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. ↑ 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. ↑ 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.
  5. ↑ 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.
  6. ↑ 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.
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