title: Sickle cell disease in Africa: a neglected cause of early childhood mortality.
abstract: Sickle cell disease (SCD) is common throughout much of sub-Saharan Africa, affecting up to 3% of births in some parts of the continent. Nevertheless, it remains a low priority for many health ministries. The most common form of SCD is caused by homozygosity for the β-globin S gene mutation (SS disease). It is widely believed that this condition is associated with very high child mortality, but reliable contemporary data are lacking. We have reviewed available African data on mortality associated with SS disease from published and unpublished sources, with an emphasis on two types of studies: cross-sectional population surveys and cohort studies. We have concluded that, although current data are inadequate to support definitive statements, they are consistent with an early-life mortality of 50%-90% among children born in Africa with SS disease. Inclusion of SCD interventions in child survival policies and programs in Africa could benefit from more precise estimates of numbers of deaths among children with SCD. A simple, representative, and affordable approach to estimate SCD child mortality is to test blood specimens already collected through large population surveys targeting conditions such as HIV, malaria, and malnutrition, and covering children of varying ages. Thus, although there is enough evidence to justify investments in screening, prophylaxis, and treatment for African children with SCD, better data are needed to estimate the numbers of child deaths preventable by such interventions and their cost effectiveness.
authors.affiliation: Division of Blood Disorders, National Center on Birth Defects and Developmental Disabilities, CDC, Atlanta, Georgia 30333, USA. sgrosse@cdc.gov
authors.forename: Scott D
authors.initials: SD
authors.lastname: Grosse
authors.forename: Isaac
authors.initials: I
authors.lastname: Odame
authors.forename: Hani K
authors.initials: HK
authors.lastname: Atrash
authors.forename: Djesika D
authors.initials: DD
authors.lastname: Amendah
authors.forename: Frédéric B
authors.initials: FB
authors.lastname: Piel
authors.forename: Thomas N
authors.initials: TN
authors.lastname: Williams
mesh_terms: D000349:Africa / Q000453:epidemiology; D000755:Anemia, Sickle Cell / Q000145:classification / Q000453:epidemiology / Q000235:genetics / Q000401:mortality*; D002648:Child; D046688:Child Mortality; D002675:Child, Preschool; D015331:Cohort Studies; D006801:Humans; D007223:Infant; D011446:Prospective Studies
references.citation: Blood Rev. 1989 Mar;3(1):18-28
references.pmid: 2650773
references.citation: Ann Trop Med Parasitol. 2007 Jan;101(1):3-14
references.pmid: 17244405
references.citation: Hum Hered. 1980;30(4):251-8
references.pmid: 6993342
references.citation: Trop Geogr Med. 1993 Mar;45(1):18-21
references.pmid: 8470299
references.citation: Appl Health Econ Health Policy. 2005;4(4):239-47
references.pmid: 16466275
references.citation: Blood. 2010 Apr 29;115(17):3447-52
references.pmid: 20194891
references.citation: Ann Hum Genet. 1956 Jul;21(1):67-89
references.pmid: 13340560
references.citation: Hemoglobin. 2002 May;26(2):113-20
references.pmid: 12144053
references.citation: Trans R Soc Trop Med Hyg. 2010 Nov;104(11):713-9
references.pmid: 20800861
references.citation: Ann Soc Belg Med Trop (1920). 1954 Aug 31;34(4):501-16
references.pmid: 13229103
references.citation: Bull Acad Natl Med. 2008 Oct;192(7):1361-72; discussion 1372-3
references.pmid: 19445362
references.citation: Ann Soc Belg Med Trop (1920). 1955 Feb 28;35(1):53-7
references.pmid: 14388457
references.citation: Hum Genet. 1984;65(3):300-2
references.pmid: 6538181
references.citation: Br Med J. 1957 Mar 30;1(5021):738-9
references.pmid: 13404287
references.citation: Lancet. 2002 Apr 13;359(9314):1311-2
references.pmid: 11965279
references.citation: PLoS One. 2011 Feb 16;6(2):e14699
references.pmid: 21358818
references.citation: Ann N Y Acad Sci. 1989;565:126-36
references.pmid: 2672962
references.citation: Pediatrics. 1988 Jun;81(6):749-55
references.pmid: 3368274
references.citation: JAMA. 1981 May 8;245(18):1839-42
references.pmid: 7230369
references.citation: Bull World Health Organ. 2001;79(8):704-12
references.pmid: 11545326
references.citation: Trop Med Int Health. 2008 Jan;13(1):76-82
references.pmid: 18291005
references.citation: Trans R Soc Trop Med Hyg. 1971;65(4):529-30
references.pmid: 5097856
references.citation: Ann Trop Med Parasitol. 1979 Apr;73(2):161-72
references.pmid: 315211
references.citation: Trends Parasitol. 2011 Jul;27(7):315-20
references.pmid: 21429801
references.citation: Microbes Infect. 2006 Apr;8(5):1260-8
references.pmid: 16679042
references.citation: Br Med J. 1956 Aug 11;2(4988):333-6
references.pmid: 13342481
references.citation: Blood. 2010 Sep 9;116(10):1663-8
references.pmid: 20530796
references.citation: Bull World Health Organ. 2008 Jun;86(6):480-7
references.pmid: 18568278
references.citation: J Clin Pathol. 2009 Jan;62(1):46-8
references.pmid: 19103860
references.citation: Public Health. 2008 Sep;122(9):933-41
references.pmid: 18555498
references.citation: Hemoglobin. 2006;30(3):355-64
references.pmid: 16840226
references.citation: Trans R Soc Trop Med Hyg. 1970;64(5):733-9
references.pmid: 5533328
references.citation: J Clin Pathol. 2009 Jan;62(1):35-8
references.pmid: 19103857
references.citation: J Pediatr. 2009 Apr;154(4):541-5
references.pmid: 19028391
references.citation: J Infect Dis. 2005 Jul 1;192(1):178-86
references.pmid: 15942909
references.citation: J Med Screen. 2007;14(3):117-22
references.pmid: 17925083
references.citation: Lancet. 2009 Oct 17;374(9698):1364-70
references.pmid: 19747721
references.citation: J Med Screen. 2007;14(3):113-6
references.pmid: 17925082
references.citation: Trans R Soc Trop Med Hyg. 1992 Sep-Oct;86(5):494-8
references.pmid: 1475814
references.citation: Ann Trop Med Parasitol. 1979 Aug;73(4):301-10
references.pmid: 315213
references.citation: Lancet. 2010 Dec 11;376(9757):2018-31
references.pmid: 21131035
references.citation: Am J Trop Med Hyg. 2005 Jan;72(1):47-59
references.pmid: 15728867
references.citation: J Med Screen. 2006;13(3):129-31
references.pmid: 17007653
references.citation: Blood. 2010 Jun 3;115(22):4331-6
references.pmid: 20233970
references.citation: Am J Prev Med. 2010 Apr;38(4 Suppl):S568-70
references.pmid: 20331959
references.citation: Blood. 2010 Jun 3;115(22):4551-8
references.pmid: 20231425
references.citation: East Afr Med J. 1994 Jun;71(6):388-91
references.pmid: 7835262
references.citation: Arch Dis Child. 2009 Sep;94(9):713-6
references.pmid: 19414433
references.citation: Nat Commun. 2010 Nov 02;1:104
references.pmid: 21045822
references.citation: Trans R Soc Trop Med Hyg. 1954 Jul;48(4):332-6
references.pmid: 13187564
references.citation: BMJ. 2005 Feb 26;330(7489):432-3
references.pmid: 15731125