Background Despite advances in treatment of cystic fibrosis (CF), pulmonary exacerbations remain common. The aim of this study was to determine if frequent pulmonary exacerbations are associated with greater declines in lung function, or an accelerated time to death or lung transplantation in adults with CF. Methods A 3-year prospective cohort study was conducted on 446 adult patients with CF from Ontario, Canada who could spontaneously produce sputum. Patients enrolled from 2005 to 2008 and were stratified into groups based upon their exacerbation rates over the 3 year study: <1 exacerbation/year (n¼140), 1e2 exacerbations/year (n¼160) and >2 exacerbations/year (n¼146). Exacerbations were defined as acute/subacute worsening of respiratory symptoms severe enough to warrant oral or intravenous antibiotics. Patient-related factors associated with frequent exacerbations were determined, and clinical outcomes were compared among the three exacerbation groups. Results Patients with frequent exacerbations were more likely to be female, diabetic and have poorer baseline lung function. Patients with >2 exacerbations/year had an increased risk of experiencing a 5% decline from baseline forced expiratory volume in 1 s (FEV 1 ); unadjusted HR 1.47 (95% CI 1.07 to 2.01, p¼0.02), adjusted HR 1.55 (95% CI 1.10 to 2.18, p¼0.01) compared with patients with <1 exacerbation/year. Patients with >2 exacerbations/year also had an increased risk of lung transplant or death over the 3 year study; unadjusted HR 12.74 (95% CI 3.92 to 41.36, p<0.0001), adjusted HR 4.05 (95% CI 1.15 to 14.28, p¼0.03). Conclusions Patients with CF with frequent exacerbations appear to experience an accelerated decline in lung function, and they have an increased 3 year risk of death or lung transplant.

SEUDOMONAS AERUGINOSA IS A gram-negative bacterium that causes chronic endobronchial infections in 60% to 70% of adult patients with cystic fibrosis (CF). 1 Infection with P aeruginosa is associated with increased morbidity and mortality for patients with CF, irrespective of lung function. 2,3 However, there is heterogeneity in the type and timing of outcome among those who are infected with P aeruginosa; some patients experience a rapid decline in pulmonary function after infection and others harbor the organism for extended periods without any obvious adverse effects. 4 The marked difference in prognosis among patients with P aeruginosa has not been adequately explained , but it may be due in part to differences among infecting strains. 5 P aeruginosa transmissible strains are genetically identical strains that infect Author Affiliations are listed at the end of this article.

In this large database analysis of PORT for thymic tumors, PORT was associated with longer OS, with the greatest relative benefits observed for stage IIB to III disease and positive margins. In the absence of randomized studies assessing the value of PORT, these data may inform clinical practice.

Use of a decision aid for patients with cystic fibrosis considering referral for lung transplantation, in addition to usual education and counseling, improves patient knowledge, realistic expectations, decisional conflict, and patient satisfaction. Clinical trial registered with www.clinicaltrials.gov (NCT00345449).

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