Sickle-cell disease epidemiology and demographics: Difference between revisions

Jump to navigation Jump to search
VisualWikitext
No edit summary
No edit summary
 
(4 intermediate revisions by 2 users not shown)
Line 1: Line 1:
__NOTOC__
__NOTOC__
{{Sickle-cell disease}}
{{Sickle-cell disease}}
{{CMG}} {{AE}} {{shyam}}
{{CMG}}; {{AE}} {{shyam}}


==Overview==  
==Overview==  
Certain races and geographic territories are more commonly affected by sickle cell disease. These geographic areas include Africa, India, and Middle East.
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==
Sickle cell disease affects millions of patients throughout the world. It is associated with high morbidity and sometimes high mortality if left untreated<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> Different regions of the world display different frequencies of genotypes of the various sickle cell subtypes. Sickle cell disease largely affects people of African descent. 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> 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> 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>
===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>


People from India also 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=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 (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 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>  
* 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>


http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4510747/bin/IJMR-141-509-g001.jpg
* 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>
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>


The frequency of sickle cell 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> The reason for this is that, from an evolutionary standpoint, the presence of sickle cell alleles and abnormal hemoglobin confers a protective advantage against malaria. The causative agents of malaria, Plasmodium, is less likely to infect diseased red blood cells.<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> This concept is known as the heterozygote advantage.
===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>


Besides Africa and India, other geographic regions are affected by particular sickle cell phenotypes. 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> 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> The nomenclature of certain sickle cell hemoglobinopathies and haplotypes is based on the region in which the prevalence is high. For example, 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>
===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>


The prevalence of sickle cell disease in the United States is relatively low: approximately 100,000 people are affected.<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> However, sickle cell disease poses a significant challenge to the American health care system. There is a high frequency of emergency department visits by patients with sickle cell disease. Over a two-year period, there were more than 97,000 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> Among these visits, 52,107 warranted hospitalization.
===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

Sickle-cell disease Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Sickle-cell disease from other Diseases

Epidemiology & Demographics

Risk Factors

Screening

Natural History, Complications & Prognosis

Diagnosis

History & Symptoms

Physical Examination

Laboratory Findings

X Ray

CT

MRI

Echocardiography or Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Future or Investigational Therapies

Case Studies

Case #1

Sickle-cell disease epidemiology and demographics On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Sickle-cell disease epidemiology and demographics

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

National Guidelines Clearinghouse

NICE Guidance

FDA on Sickle-cell disease epidemiology and demographics

CDC on Sickle-cell disease epidemiology and demographics

Sickle-cell disease epidemiology and demographics in the news

Blogs onSickle-cell disease epidemiology and demographics

Directions to Hospitals Treating Sickle-cell disease

Risk calculators and risk factors for Sickle-cell disease epidemiology and demographics

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.
Retrieved from "https://www.wikidoc.org/index.php?title=Sickle-cell_disease_epidemiology_and_demographics&oldid=1487331"