Sickle-cell disease risk factors: Difference between revisions

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==Overview==
==Overview==
There are no specific risk factors for sickle cell disease.
The most potent risk factor in the development of sickle-cell disease is race.  


==Risk Factors==
==Risk Factors==
Besides race, there are no specific risk factors for sickle cell disease. Sickle cell disease is a monogenetic disease, meaning that one gene mutations causes the disease. Thus environmental factors do not play a major role in acquisition of the disease. The disease occurs at birth after a person inherits 2 sickle cell alleles, one from each parent. Certain endemic regions contain a high prevalence of sickle cell alleles.<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 degree of fetal hemoglobin production is related to the severe of disease: higher fetal hemoglobin confers lower disease morbidity and thus is a favorable risk factor/modifier.<ref name="pmid22887019">{{cite journal| author=Kutlar A, Ataga K, Reid M, Vichinsky EP, Neumayr L, Blair-Britt L et al.| title=A phase 1/2 trial of HQK-1001, an oral fetal globin inducer, in sickle cell disease. | journal=Am J Hematol | year= 2012 | volume= 87 | issue= 11 | pages= 1017-21 | pmid=22887019 | doi=10.1002/ajh.23306 | pmc=3904792 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22887019  }} </ref>
===Common Risk Factors===
 
*Common risk factors in the development of sickle-cell disease include:<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><ref name="pmid22099364">{{cite journal |vauthors=Grosse SD, Odame I, Atrash HK, Amendah DD, Piel FB, Williams TN |title=Sickle cell disease in Africa: a neglected cause of early childhood mortality |journal=Am J Prev Med |volume=41 |issue=6 Suppl 4 |pages=S398–405 |date=December 2011 |pmid=22099364 |pmc=3708126 |doi=10.1016/j.amepre.2011.09.013 |url=}}</ref>
The racial groups who are at highest risk for sickle cell disease include Africans, African Americans, Indians, and persons of Mediterranean descent. These groups have the highest frequency of the sickle cell allele.
** Africans
 
**African Americans
There are some risk factors for precipitation of sickle cell crises, such as dehydration, low oxygen levels, medication nonadherence, and cold exposure. Patients should be educated about these risk factors so that sickle cell crises can be prevented.
**Indians
**Mediterranean descent
**Geographic location


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

Latest revision as of 15:14, 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

The most potent risk factor in the development of sickle-cell disease is race.

Risk Factors

Common Risk Factors

  • Common risk factors in the development of sickle-cell disease include:[1][2]
    • Africans
    • African Americans
    • Indians
    • Mediterranean descent
    • Geographic location

References

  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.
  2. Grosse SD, Odame I, Atrash HK, Amendah DD, Piel FB, Williams TN (December 2011). "Sickle cell disease in Africa: a neglected cause of early childhood mortality". Am J Prev Med. 41 (6 Suppl 4): S398–405. doi:10.1016/j.amepre.2011.09.013. PMC 3708126. PMID 22099364.