PT  - JOURNAL ARTICLE
AU  - Leonie A. Tepper
AU  - Elisabeth M.W.J. Utens
AU  - Daan Caudri
AU  - Aukje C. Bos
AU  - Karla Gonzalez-Graniel
AU  - Hugo J. Duivenvoorden
AU  - Els C.W. van der Wiel
AU  - Alexandra L. Quittner
AU  - Harm A.W.M. Tiddens
TI  - Impact of bronchiectasis and trapped air on quality of life and exacerbations in cystic fibrosis
AID  - 10.1183/09031936.00137612
DP  - 2013 Aug 01
TA  - European Respiratory Journal
PG  - 371--379
VI  - 42
IP  - 2
4099  - http://erj.ersjournals.com/content/42/2/371.short
4100  - http://erj.ersjournals.com/content/42/2/371.full
SO  - Eur Respir J2013 Aug 01; 42
AB  - Cystic fibrosis (CF) is primarily characterised by bronchiectasis and trapped air on chest computed tomography (CT). The revised Cystic Fibrosis Questionnaire respiratory symptoms scale (CFQ-R RSS) measures health-related quality of life. To validate bronchiectasis, trapped air and CFQ-R RSS as outcome measures, we investigated correlations and predictive values for pulmonary exacerbations. CF patients (aged 6–20 years) underwent CT, CFQ-R RSS and 1-year follow-up. Bronchiectasis and trapped air were scored using the CF-CT scoring system. Correlation coefficients and backward multivariate modelling were used to identify predictors of pulmonary exacerbations. 40 children and 32 adolescents were included. CF-CT bronchiectasis (r = -0.38, p<0.001) and CF-CT trapped air (r = -0.35, p = 0.003) correlated with CFQ-R RSS. Pulmonary exacerbations were associated with: bronchiectasis (rate ratio 1.10, 95% CI 1.02–1.19; p = 0.009), trapped air (rate ratio 1.02, 95% CI 1.00–1.05; p = 0.034) and CFQ-R RSS (rate ratio 0.95, 95% CI 0.91–0.98; p = 0.002). The CFQ-R RSS was an independent predictor of pulmonary exacerbations (rate ratio 0.96, 95% CI 0.94–0.97; p<0.001). Bronchiectasis, trapped air and CFQ-R RSS were associated with pulmonary exacerbations. The CFQ-R RSS was an independent predictor. This study further validated bronchiectasis, trapped air and CFQ-R RSS as outcome measures in CF.