{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,20]],"date-time":"2026-09-20T22:49:15Z","timestamp":1789944555510,"version":"4.0.1"},"reference-count":31,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2014,2,12]],"date-time":"2014-02-12T00:00:00Z","timestamp":1392163200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100005564","name":"Gilead Sciences","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100005564","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2014,12]]},"abstract":"<jats:title>Summary<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Cystic fibrosis (CF) lung disease is characterized by bronchiectasis and trapped air on chest computed tomography (CT).<\/jats:p><\/jats:sec><jats:sec><jats:title>Objective<\/jats:title><jats:p>We aim to validate bronchiectasis and trapped air as outcome measures by evaluating associations between changes in bronchiectasis, trapped air and patient\u2010reported respiratory symptoms.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A longitudinal cohort study has been conducted. CF patients (aged 6\u201319 years) who had two routine CTs and completed twice a Cystic Fibrosis Questionnaire\u2010Revised within 2 years (referred to as T<jats:sub>1<\/jats:sub> and T<jats:sub>2<\/jats:sub>), in the period of July 2007 to January 2012 were included. Bronchiectasis and trapped air were scored using the CF\u2010CT scoring system. Correlation coefficients and student's paired <jats:italic>t<\/jats:italic> tests were performed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>In total 40 patients were included with a median age at T<jats:sub>1<\/jats:sub> of 12.6 years (range 6\u201317 years), and at T<jats:sub>2<\/jats:sub> 14.5 years (range 8\u201319 years). At T<jats:sub>1<\/jats:sub>, bronchiectasis (r\u2009=\u2009\u22120.49, <jats:italic>P<\/jats:italic>\u2009&lt;\u20090.01) and trapped air (r\u2009=\u2009\u22120.34, <jats:italic>P<\/jats:italic>\u2009=\u20090.04) correlated with CFQ\u2010R Respiratory Symptoms Scores (CFQ\u2010R RSS). At T<jats:sub>2<\/jats:sub> similar correlations were found with the CFQ\u2010R RSS. Over 2 years, there was significant progression in bronchiectasis (<jats:italic>P<\/jats:italic>\u2009=\u20090.03) and trapped air (<jats:italic>P<\/jats:italic>\u2009=\u20090.03), but not in CFQ\u2010R RSS. Changes in bronchiectasis and trapped air were not associated with changes in CFQ\u2010R RSS.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Our results indicate that bronchiectasis and trapped are sensitive outcome measures in CF lung disease, showing a significant association with CFQ\u2010R RSS at two\u2010time points. However, progression of bronchiectasis and trapped air over 2 year does not necessarily correlate to changes in quality of life. <jats:bold>Pediatr Pulmonol. 2014; 49:1182\u20131189.<\/jats:bold> \u00a9 2014 Wiley Periodicals, Inc.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.22991","type":"journal-article","created":{"date-parts":[[2014,2,12]],"date-time":"2014-02-12T12:42:55Z","timestamp":1392208975000},"page":"1182-1189","source":"Crossref","is-referenced-by-count":32,"title":["Tracking CF disease progression with CT and respiratory symptoms in a cohort of children aged 6\u201319 years"],"prefix":"10.1002","volume":"49","author":[{"given":"Leonie A.","family":"Tepper","sequence":"first","affiliation":[{"name":"Department of Pediatric Pulmonology Erasmus Medical Centre\u2014Sophia Children's Hospital Rotterdam The Netherlands"},{"name":"Department of Radiology Erasmus MC Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daan","family":"Caudri","sequence":"additional","affiliation":[{"name":"Department of Pediatric Pulmonology Erasmus Medical Centre\u2014Sophia Children's Hospital Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisabeth M.W.J.","family":"Utens","sequence":"additional","affiliation":[{"name":"Department of Child and Adolescent Psychiatry\/Psychology Erasmus MC Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Els C.","family":"van der Wiel","sequence":"additional","affiliation":[{"name":"Department of Pediatric Pulmonology Erasmus Medical Centre\u2014Sophia Children's Hospital Rotterdam The Netherlands"},{"name":"Department of Radiology Erasmus MC Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandra L.","family":"Quittner","sequence":"additional","affiliation":[{"name":"Departments of Psychology and Pediatrics University of Miami Coral Gables Florida"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Harm A.W.M.","family":"Tiddens","sequence":"additional","affiliation":[{"name":"Department of Pediatric Pulmonology Erasmus Medical Centre\u2014Sophia Children's Hospital Rotterdam The Netherlands"},{"name":"Department of Radiology Erasmus MC Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2014,2,12]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1513\/pats.200703-041BR"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1513\/pats.200611-178HT"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.03.00006603"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.prrv.2006.04.002"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1136\/thx.2010.150375"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2009.05.005"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200901-0069OC"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1136\/thoraxjnl-2011-200912"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1136\/thx.2008.110908"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.10-1152"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200407-989OC"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00137612"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.20091"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0023932"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.128.4.2347"},{"key":"e_1_2_9_17_1","unstructured":"US Food and Drug Administration. 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