Genetic and non-genetic determinants of outcomes in cystic fibrosis

Paediatr Respir Rev. 2009 Mar;10(1):32-6. doi: 10.1016/j.prrv.2008.04.002. Epub 2008 Aug 3.


The treatment of cystic fibrosis has improved significantly over the past three decades. Median survival has improved by decades and is now estimated to be 37 years. Many factors contribute to improvements in disease severity and outcome. This paper reviews the current evidence of three groups of important factors: genetic, environmental and healthcare related.

Publication types

  • Review

MeSH terms

  • Cystic Fibrosis / genetics*
  • Cystic Fibrosis / therapy*
  • Environmental Exposure*
  • Humans
  • Severity of Illness Index
  • Socioeconomic Factors
  • Tobacco Smoke Pollution / adverse effects
  • Treatment Outcome


  • Tobacco Smoke Pollution