{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,28]],"date-time":"2026-07-28T11:20:47Z","timestamp":1785237647300,"version":"3.55.0"},"reference-count":30,"publisher":"European Respiratory Society (ERS)","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Eur Respir J"],"accepted":{"date-parts":[[2012,11,19]]},"published-print":{"date-parts":[[2013,8]]},"abstract":"<jats:p>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.<\/jats:p><jats:p>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\u201320 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.<\/jats:p><jats:p>40 children and 32 adolescents were included. CF-CT bronchiectasis (r = -0.38, p&lt;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\u20131.19; p = 0.009), trapped air (rate ratio 1.02, 95% CI 1.00\u20131.05; p = 0.034) and CFQ-R RSS (rate ratio 0.95, 95% CI 0.91\u20130.98; p = 0.002). The CFQ-R RSS was an independent predictor of pulmonary exacerbations (rate ratio 0.96, 95% CI 0.94\u20130.97; p&lt;0.001).<\/jats:p><jats:p>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.<\/jats:p>","DOI":"10.1183\/09031936.00137612","type":"journal-article","created":{"date-parts":[[2013,1,12]],"date-time":"2013-01-12T05:41:15Z","timestamp":1357969275000},"page":"371-379","source":"Crossref","is-referenced-by-count":58,"title":["Impact of bronchiectasis and trapped air on quality of life and exacerbations in cystic fibrosis"],"prefix":"10.1183","volume":"42","author":[{"given":"Leonie A.","family":"Tepper","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisabeth M.W.J.","family":"Utens","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daan","family":"Caudri","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aukje C.","family":"Bos","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karla","family":"Gonzalez-Graniel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hugo J.","family":"Duivenvoorden","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Els C.W.","family":"van der Wiel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandra L.","family":"Quittner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Harm A.W.M.","family":"Tiddens","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"81","published-online":{"date-parts":[[2013,1,11]]},"reference":[{"key":"2024102021295329000_42.2.371.1","doi-asserted-by":"publisher","DOI":"10.1136\/thoraxjnl-2011-200912"},{"key":"2024102021295329000_42.2.371.2","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0023932"},{"key":"2024102021295329000_42.2.371.3","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2009.05.005"},{"key":"2024102021295329000_42.2.371.4","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200901-0069OC"},{"key":"2024102021295329000_42.2.371.5","doi-asserted-by":"publisher","DOI":"10.1111\/j.1440-1843.2010.01765.x"},{"key":"2024102021295329000_42.2.371.6","doi-asserted-by":"publisher","DOI":"10.1136\/thx.2005.045146"},{"key":"2024102021295329000_42.2.371.7","doi-asserted-by":"publisher","DOI":"10.1378\/chest.10-1152"},{"key":"2024102021295329000_42.2.371.8","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200407-989OC"},{"key":"2024102021295329000_42.2.371.9","doi-asserted-by":"publisher","DOI":"10.1136\/thx.2008.110908"},{"key":"2024102021295329000_42.2.371.10","doi-asserted-by":"crossref","unstructured":"Quittner AL Alpern AN Kimberg CI . 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