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==Epidemiology and Demographics== | ==Epidemiology and Demographics== | ||
Sickle cell disease is known to have a high prevalence in Africa (specifically the sub-Saharan region), India, and the Middle East. Difference regions of the world display different frequencies of genotypes of the various sickle cell subtypes. | Sickle cell disease is known to have a high prevalence in Africa (specifically the sub-Saharan region), India, and the Middle East. Difference regions of the world display different frequencies of genotypes of the various sickle cell subtypes. | ||
Sickle cell disease is a condition that largely affects people of African descent. In a study of three cohorts in Nigeria and Sudan, the frequency of HbSS was 88-96%. The frequency of HbSC was 4-12%.<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=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24361300 }} </ref> It affects 1 in 400 African Americans, and a total of 89,000 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> In contrast, people 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=http://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 in India of sickle disease disease varies from 1-40%.<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> | Sickle cell disease is a condition that largely affects people of African descent. In a study of three cohorts in Nigeria and Sudan, the frequency of HbSS was 88-96%. The frequency of HbSC was 4-12%.<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=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24361300 }} </ref> It affects 1 in 400 African Americans, and a total of 89,000 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> In contrast, people 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=http://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 in India of sickle disease disease varies from 1-40%. Certain tribal populations in India have high prevalences of sickle cell trait and/or disease. For example, Madhya Pradesh has the highest prevalence, with more tan 960000 heterozygotes and nearly 68000 homozogotes. <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> | ||
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4510747/bin/IJMR-141-509-g001.jpg | |||
Frequency of sickle cell trait in various provinces of India.<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> | |||
There is a high frequency of emergency department visits by patients with sickle cell disease. Over a two-year period, there were a total of 97.579 visits amongst 8 states.<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> Amongst these visits, 52,107 warranted hospitalization. | There is a high frequency of emergency department visits by patients with sickle cell disease. Over a two-year period, there were a total of 97.579 visits amongst 8 states.<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> Amongst these visits, 52,107 warranted hospitalization. | ||
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Shyam Patel [2]
Overview
Epidemiology and Demographics
Sickle cell disease is known to have a high prevalence in Africa (specifically the sub-Saharan region), India, and the Middle East. Difference regions of the world display different frequencies of genotypes of the various sickle cell subtypes. Sickle cell disease is a condition that largely affects people of African descent. In a study of three cohorts in Nigeria and Sudan, the frequency of HbSS was 88-96%. The frequency of HbSC was 4-12%.[1] It affects 1 in 400 African Americans, and a total of 89,000 Americans.[2] In contrast, people from India have a lower frequency of the HbSS genotype (30-70%) and a higher frequency of sickle cell-beta-thalassemia (HbSB) genotype.[1] The heterozygote prevalence in India of sickle disease disease varies from 1-40%. Certain tribal populations in India have high prevalences of sickle cell trait and/or disease. For example, Madhya Pradesh has the highest prevalence, with more tan 960000 heterozygotes and nearly 68000 homozogotes. [3]
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4510747/bin/IJMR-141-509-g001.jpg Frequency of sickle cell trait in various provinces of India.[3]
There is a high frequency of emergency department visits by patients with sickle cell disease. Over a two-year period, there were a total of 97.579 visits amongst 8 states.[2] Amongst these visits, 52,107 warranted hospitalization.
References
- ↑ 1.0 1.1 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.
- ↑ 2.0 2.1 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.
- ↑ 3.0 3.1 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.