title: Mortality in sickle cell anemia in Africa: a prospective cohort study in Tanzania.
abstract: BACKGROUND
  The World Health Organization has declared Sickle Cell Anemia (SCA) a public health priority. There are 300,000 births/year, over 75% in Africa, with estimates suggesting that 6 million Africans will be living with SCA if average survival reaches half the African norm. Countries such as United States of America and United Kingdom have reduced SCA mortality from 3 to 0.13 per 100 person years of observation (PYO), with interventions such as newborn screening, prevention of infections and comprehensive care, but implementation of interventions in African countries has been hindered by lack of locally appropriate information. The objective of this study was to determine the incidence and factors associated with death from SCA in Dar-es-Salaam.
  
  
  RESULTS
  A hospital-based cohort study was conducted, with prospective surveillance of 1,725 SCA patients recruited from 2004 to 2009, with 209 (12%) lost to follow up, while 86 died. The mortality rate was 1.9 (95%CI 1.5, 2.9) per 100 PYO, highest under 5-years old [7.3 (4.8-11.0)], adjusting for dates of birth and study enrollment. Independent risk factors, at enrollment to the cohort, predicting death were low hemoglobin (<5 g/dL) [3.8 (1.8-8.2); p = 0.001] and high total bilirubin (≥102 µmol/L) [1.7 (1.0-2.9); p = 0.044] as determined by logistic regression.
  
  
  CONCLUSIONS
  Mortality in SCA in Africa is high, with the most vulnerable period being under 5-years old. This is most likely an underestimate, as this was a hospital cohort and may not have captured SCA individuals with severe disease who died in early childhood, those with mild disease who are undiagnosed or do not utilize services at health facilities. Prompt and effective treatment for anemia in SCA is recommended as it is likely to improve survival. Further research is required to determine the etiology, pathophysiology and the most appropriate strategies for management of anemia in SCA.
authors.affiliation: Department of Haematology and Blood Transfusion, Muhimbili University of Health and Allied Sciences, Dar-es-Salaam, Tanzania. jmakani@muhas.ac.tz
authors.forename: Julie
authors.initials: J
authors.lastname: Makani
authors.forename: Sharon E
authors.initials: SE
authors.lastname: Cox
authors.forename: Deogratius
authors.initials: D
authors.lastname: Soka
authors.forename: Albert N
authors.initials: AN
authors.lastname: Komba
authors.forename: Julie
authors.initials: J
authors.lastname: Oruo
authors.forename: Hadija
authors.initials: H
authors.lastname: Mwamtemi
authors.forename: Pius
authors.initials: P
authors.lastname: Magesa
authors.forename: Stella
authors.initials: S
authors.lastname: Rwezaula
authors.forename: Elineema
authors.initials: E
authors.lastname: Meda
authors.forename: Josephine
authors.initials: J
authors.lastname: Mgaya
authors.forename: Brett
authors.initials: B
authors.lastname: Lowe
authors.forename: David
authors.initials: D
authors.lastname: Muturi
authors.forename: David J
authors.initials: DJ
authors.lastname: Roberts
authors.forename: Thomas N
authors.initials: TN
authors.lastname: Williams
authors.forename: Kisali
authors.initials: K
authors.lastname: Pallangyo
authors.forename: Jesse
authors.initials: J
authors.lastname: Kitundu
authors.forename: Gregory
authors.initials: G
authors.lastname: Fegan
authors.forename: Fenella J
authors.initials: FJ
authors.lastname: Kirkham
authors.forename: Kevin
authors.initials: K
authors.lastname: Marsh
authors.forename: Charles R
authors.initials: CR
authors.lastname: Newton
mesh_terms: D000293:Adolescent; D000328:Adult; D000349:Africa / Q000453:epidemiology; D000755:Anemia, Sickle Cell / Q000175:diagnosis / Q000453:epidemiology / Q000401:mortality*; D002423:Cause of Death; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D012307:Risk Factors; D013636:Tanzania / Q000453:epidemiology; D055815:Young Adult