title: Sickle cell disease related mortality in the United States (1999-2009).
abstract: BACKGROUND
  Little is known about the national outcome of children and adults with sickle cell disease (SCD) given contemporary care.
  
  
  METHODS
  We investigated the number of deaths, standardized crude and age-adjusted mortality rates, and causes of death among individuals with SCD across the United States during 1999-2009 according to death certificates by using a publicly available website (http://wonder.cdc.gov/). Data were compared to mortality during 1979-1998.
  
  
  RESULTS
  When compared to 1979-1998, mortality significantly decreased by 61% in infants <1 year of age, by 67% in children aged 1-4 years, and by 22-35% in children aged 5-19 years. After 19 years of age, mortality rates increased from 0.6 in the 15-19 year group to 1.4/100,000 in the 20-24 year group, corresponding to the transition period from pediatric to adult medical care, and this increase was similar during 1979-1998. Although the age groups with the highest mortality were 35-44 years for males and 45-54 years for females, there was a tendency for longer survival because there were more deaths among those individuals 55-74 years of age compared to previous years. For all individuals, the causes of deaths were cardiac disease (31.6%), respiratory (28.1%), renal (16.4%), infectious (14.4%), neurologic (11.9%), and gastrointestinal and hepatobiliary (9.2%) in nature. Cancer was the cause of death in <1%.
  
  
  CONCLUSIONS
  Mortality during childhood has decreased significantly. However, the transition period from pediatric to adult care is critical. Risk-reduction, monitoring, and early treatment intervention of cardiovascular disease in adults is warranted.
authors: Division of Pediatric Hematology, University of Miami Miller School of Medicine, Miami, Florida, USA.
authors: Dima
authors: D
authors: Hamideh
authors: Ofelia
authors: O
authors: Alvarez
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D000755:Anemia, Sickle Cell / Q000150:complications / Q000401:mortality*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005767:Gastrointestinal Diseases / Q000209:etiology / Q000401:mortality; D006331:Heart Diseases / Q000209:etiology / Q000401:mortality; D006801:Humans; D007223:Infant; D007239:Infections / Q000209:etiology / Q000401:mortality; D007674:Kidney Diseases / Q000209:etiology / Q000401:mortality; D008107:Liver Diseases / Q000209:etiology / Q000401:mortality; D008297:Male; D008875:Middle Aged; D009369:Neoplasms / Q000209:etiology / Q000401:mortality; D009422:Nervous System Diseases / Q000209:etiology / Q000401:mortality; D012140:Respiratory Tract Diseases / Q000209:etiology / Q000401:mortality; D012189:Retrospective Studies; D012737:Sex Factors; D014481:United States / Q000453:epidemiology
keywords: mortality; sickle cell disease; survival; transition