title: Impact of bronchiectasis and trapped air on quality of life and exacerbations in cystic fibrosis.
abstract: 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.
authors: Dept of Pediatric Pulmonology, Erasmus Medical Centre, Rotterdam, The Netherlands.
authors: Leonie A
authors: LA
authors: Tepper
authors: Elisabeth M W J
authors: EM
authors: Utens
authors: Daan
authors: D
authors: Caudri
authors: Aukje C
authors: AC
authors: Bos
authors: Karla
authors: K
authors: Gonzalez-Graniel
authors: Hugo J
authors: HJ
authors: Duivenvoorden
authors: Els C W
authors: EC
authors: van der Wiel
authors: Alexandra L
authors: AL
authors: Quittner
authors: Harm A W M
authors: HA
authors: Tiddens
mesh_terms: D000293:Adolescent; D000388:Air; D001987:Bronchiectasis / Q000150:complications* / Q000000981:diagnostic imaging / Q000523:psychology*; D002648:Child; D003550:Cystic Fibrosis / Q000150:complications* / Q000000981:diagnostic imaging / Q000523:psychology*; D005260:Female; D006801:Humans; D008168:Lung / Q000000981:diagnostic imaging; D008297:Male; D015999:Multivariate Analysis; D009154:Mutation; D015588:Observer Variation; D011788:Quality of Life; D012189:Retrospective Studies; D011795:Surveys and Questionnaires; D014057:Tomography, X-Ray Computed / Q000379:methods; D016896:Treatment Outcome; D055815:Young Adult