title: Changes in cystic fibrosis mortality in Australia, 1979-2005.
abstract: OBJECTIVE
  To assess mortality trends among people with cystic fibrosis (CF) in Australia.
  
  
  METHODS
  We augmented Australian summary data for deaths from CF registered during 1979-2005 with information from Australian transplant centres on lung transplantation among CF patients for 1989-2005 to allow us to follow trends in all "mortality events" (death or lung transplantation).
  
  
  METHODS
  Age at death or lung transplantation.
  
  
  RESULTS
  Between 1979 and 2005, the mean age at death increased from 12.2 years to 27.9 years for males and from 14.8 years to 25.3 years for females. Overall, female deaths in childhood (0-14 years) occurred at an age-standardised rate of 0.40 per 100,000 (95% CI, 0.34-0.45) during 1979-2005, which exceeded the corresponding rate for males of 0.24 (95% CI, 0.20-0.28) per 100,000. Among 0-14-year-old boys, event rates declined markedly after 1989, but they declined later and more gradually for girls, with the result that the age-standardised rate for girls was 2.38 times that of boys during 1989-2005 (95% CI, 1.69-3.36).
  
  
  CONCLUSIONS
  The pattern of CF mortality in Australia has changed substantially. Mortality rates continue to be higher for girls than for boys, but death in childhood has become uncommon. Survival has increased since 1979, but females continue to have reduced length of life.
authors: Department of Thoracic Medicine, The Prince Charles Hospital, Brisbane, QLD. David.Reid@qimr.edu.au
authors: David W
authors: DW
authors: Reid
authors: C Leigh
authors: CL
authors: Blizzard
authors: Dace M
authors: DM
authors: Shugg
authors: Ceri
authors: C
authors: Flowers
authors: Catherine
authors: C
authors: Cash
authors: Hugh M
authors: HM
authors: Greville
mesh_terms: D000293:Adolescent; D000328:Adult; D001315:Australia / Q000453:epidemiology; D002648:Child; D003550:Cystic Fibrosis / Q000175:diagnosis / Q000401:mortality* / Q000628:therapy; D005260:Female; D006801:Humans; D008017:Life Expectancy; D016040:Lung Transplantation; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D055815:Young Adult