Gender and survival in cystic fibrosis

Curr Opin Pulm Med. 2013 Nov;19(6):692-7. doi: 10.1097/MCP.0b013e328365af49.


Purpose of review: Survival for patients with cystic fibrosis (CF) continues to improve. The proportion of CF patients over the age of 18 years is nearly 50%, and care providers will need to better understand this patient population. Despite these improvements, young females continue to have a worse prognosis and lower median survival compared with their male counterparts. Contributing factors to the difference in survival remain uncertain.

Recent findings: The 'gender gap' remains an area of controversy. Recent data suggest that it still exists, though exact reasons remain unclear. For those patients diagnosed in adulthood, outcomes are also improving. Some evidence suggests persistence of the gender gap. Other data suggest a reversal of this effect. Additional work and study are needed.

Summary: This review finds supporting evidence for persistence of the gender gap and outlines the effect of age and sex on survival in CF. The majority of patients with CF will now be adults; thus, care providers must be aware of the impact this will have on ongoing patient management.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Adult
  • Age Distribution
  • Age of Onset
  • Cystic Fibrosis / diagnosis
  • Cystic Fibrosis / genetics
  • Cystic Fibrosis / mortality*
  • Cystic Fibrosis Transmembrane Conductance Regulator / genetics
  • Disease Progression*
  • Female
  • Humans
  • Male
  • Sex Distribution*
  • Sex Factors
  • Survival Analysis


  • CFTR protein, human
  • Cystic Fibrosis Transmembrane Conductance Regulator