title: Survival of children with sickle cell disease in the comprehensive newborn screening programme in Minas Gerais, Brazil.
abstract: BACKGROUND
  Children in developing countries with sickle cell disease SCD have high rates of mortality, especially in some parts of Africa.
  
  
  OBJECTIVE
  To compare the 5-year estimated mortality rate in children born between 1999 and 2001 with that of children born between 2009 and 2011.
  
  
  METHODS
  During the period 1998-2012, sickle cell disease was diagnosed in 2591 of 3,617,919 newborns screened in Minas Gerais, Brazil (1 : 1,400). The estimated probability of death [1 - Survival] was calculated by the Kaplan-Meier method. The logrank test was used to compare groups of survival data.
  
  
  RESULTS
  Of the 2576 children (15 were excluded), 193 died (7.4%): 153 (79.3%) had SS/Sβ(0)-thalassaemia, 34 had SC (17.6%), and six (3.1%) had Sβ(+) thalassaemia. The 5-year estimated mortality was lower for children born between 2009 and 2011 (n=509) than for those born between 1999 and 2001 (n=624), although not significantly [mean (SD) 5.8% (1.1) vs 6.2% (1.0)], respectively).
  
  
  CONCLUSIONS
  Despite an effective ongoing comprehensive screening programme, mortality from SCD in Minas Gerais is still high. To decrease mortality rates, socio-economic development and SCD education programmes for health professionals and families are required.
authors.affiliation: Center for Newborn Screening and Genetic Diagnosis.
authors.forename: Alessandra Palhoni
authors.initials: AP
authors.lastname: Sabarense
authors.forename: Gabriella Oliveira
authors.initials: GO
authors.lastname: Lima
authors.forename: Lívia Maria Lellis
authors.initials: LM
authors.lastname: Silva
authors.forename: Marcos Borato
authors.initials: MB
authors.lastname: Viana
mesh_terms: D000755:Anemia, Sickle Cell / Q000175:diagnosis* / Q000401:mortality*; D001938:Brazil / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D005260:Female; D006266:Health Education; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008403:Mass Screening / Q000379:methods*; D009026:Mortality; D012959:Socioeconomic Factors; D016019:Survival Analysis
keywords: Death rate; Epidemiology; Haemoglobinopathies; Neonatal screening; Sickle cell disease; Socioeconomic status