title: Cystic fibrosis in Australia, 2009: results from a data registry.
abstract: OBJECTIVE
  To describe the demographics, clinical features and outcomes among people with cystic fibrosis (CF) in Australia and to estimate incidence of the disease.
  
  
  METHODS
  Cross-sectional analysis using data from the Australian Cystic Fibrosis Data Registry for 2009.
  
  
  METHODS
  Numbers of diagnoses, pulmonary and anthropometric measurements, microbiological culture results, rates of hospitalisation and transplantation, and numbers of medical complications and deaths.
  
  
  RESULTS
  In 2009, data were submitted on 2986 people (48% female). Median age was 17.6 years and 49% of people were aged 18 years or over. Seventy-eight people were newly diagnosed. Fourteen people died and 14 people underwent lung transplantation in the year. Lung function and nutrition were relatively normal among children but deteriorated (more rapidly) among adolescents. With increasing age, progressive respiratory disease was apparent, and the frequency of CF-related complications and use of health care resources increased. In all age groups, there was a wide range in severity of lung disease and nutritional status.
  
  
  CONCLUSIONS
  CF remains a progressive respiratory disease and is associated with multisystem complications. The acceleration in disease severity in adolescence and early adulthood suggests that better treatment at these stages is required to further improve survival.
authors: Department of Thoracic Medicine, The Prince Charles Hospital, Brisbane, QLD. scott_bell@health.qld.gov.au
authors: Scott C
authors: SC
authors: Bell
authors: Peter T P
authors: PT
authors: Bye
authors: Peter J
authors: PJ
authors: Cooper
authors: A James
authors: AJ
authors: Martin
authors: Karen O
authors: KO
authors: McKay
authors: Phillip J
authors: PJ
authors: Robinson
authors: Gerard F
authors: GF
authors: Ryan
authors: Geoff C
authors: GC
authors: Sims
mesh_terms: D000293:Adolescent; D000328:Adult; D001315:Australia / Q000453:epidemiology; D002648:Child; D003430:Cross-Sectional Studies; D003550:Cystic Fibrosis / Q000175:diagnosis / Q000453:epidemiology* / Q000628:therapy; D005260:Female; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012042:Registries; D015996:Survival Rate; D055815:Young Adult