Abstract
Cystic fibrosis (CF) is primarily characterized by bronchiectasis and trapped air on chest computed tomography (CT). The Cystic Fibrosis Questionnaire-Revised 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 (6–20 years) had a 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 modeling 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 (RR) 1.10, 95% Confidence Interval (CI95%) 1.02 to 1.19, p=0.009), trapped air (RR 1.02, CI95% 1.00 to 1.05, p=0.034), and CFQ-R RSS (RR 0.95, CI95% 0.91 to 0.98, p=0.002). The CFQ-R RSS was an independent predictor of pulmonary exacerbations (RR 0.96, CI95% 0.94 to 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.
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