Early referral to cystic fibrosis specialist centre impacts on respiratory outcome

J Cyst Fibros. 2009 Jan;8(1):26-30. doi: 10.1016/j.jcf.2008.07.005. Epub 2008 Oct 5.


Background: Published studies concerning the impact of specialist care on lung disease in cystic fibrosis remain limited and most are either biased due to comparison with historical controls and/or underpowered.

Methods: In this retrospective multicentric study, data from all CF children fulfilling the following criteria were collected: 1) Age 6-<18 at the end of 2003; 2) diagnosis before 8 y; 3) follow-up in an accredited CF Belgian centre; 4) at least 1 spirometry and respiratory culture available for 2003. Group A included children referred > or =2 years after the diagnosis. Patients from Group A were then matched with a single early referred patient on the basis of 2 criteria: same centre, as closest age as possible (Group B).

Results: Data from 217 children were collected (Group A: 67/217). Late referred patients had a lower FEV(1) (77.2%+/-22.4 vs 86.7% pred.+/-19.4, p=0.01) and a higher prevalence of Pseudomonas aeruginosa (38.6 vs 17.5%, p<0.05).

Conclusion: In this population of CF children, a delay of 6.1 y (vs 0.1 y) between diagnosis and referral to a specialist clinic resulted in poorer respiratory outcome at age 13.

Publication types

  • Multicenter Study

MeSH terms

  • Adolescent
  • Belgium
  • Child
  • Cystic Fibrosis / diagnosis
  • Cystic Fibrosis / microbiology
  • Cystic Fibrosis / therapy*
  • Disease Progression
  • Humans
  • Outcome Assessment, Health Care
  • Pseudomonas aeruginosa / isolation & purification
  • Referral and Consultation*
  • Retrospective Studies
  • Time Factors
  • Treatment Outcome
  • Vital Capacity