{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,5]],"date-time":"2026-07-05T02:08:58Z","timestamp":1783217338744,"version":"3.54.6"},"reference-count":26,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2020,1,21]],"date-time":"2020-01-21T00:00:00Z","timestamp":1579564800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Novartis Institutes for Biomedical Research, Cambridge, MA, USA"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2020,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>To determine which outcome measures could detect early progression of disease in school\u2010age children with mild cystic fibrosis (CF) lung disease over a two\u2010year time interval utilizing chest computed tomography (CT) scores, quantitative CT air trapping (QAT), and spirometric measurements.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Thirty\u2010six school\u2010age children with mild CF lung disease (median [interquartile range] age 12 [3.7] years; percent predicted forced expiratory volume in 1\u2009second (ppFEV<jats:sub>1<\/jats:sub>) 99 [12.5]) were evaluated by serial spirometer\u2010controlled chest CT scans and spirometry at baseline, 3\u2010month, 1\u2010 and 2\u2010years.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>No significant changes were noted at 3\u2010month for any variable except for decreased ppFEV<jats:sub>1<\/jats:sub>. Mucus plugging score (MPS) and QAT<jats:sub>A1<\/jats:sub> <jats:sub>and<\/jats:sub> <jats:sub>A2<\/jats:sub> increased at 1\u2010 and 2\u2010years. The bronchiectasis score (BS), and total score (TS) were increased at 2\u2010year. All variables tested with the exception of bronchial wall thickness score, parenchymal score (PS), and ppFEV<jats:sub>1<\/jats:sub>, were consistent with longitudinal worsening of lung disease. Multivariate analysis revealed baseline PS, baseline TS, and 1\u2010year changes in BS and air trapping score were predictive of 2\u2010year changes in BS.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>MPS and QAT<jats:sub>A1\u2010A2<\/jats:sub> were the most sensitive indicators of progressive childhood CF lung disease. The 1\u2010year change in the bronchiectasis score had the most positive predictive power for 2\u2010year change in bronchiectasis.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.24646","type":"journal-article","created":{"date-parts":[[2020,1,21]],"date-time":"2020-01-21T20:27:30Z","timestamp":1579638450000},"page":"929-938","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":24,"title":["Mucus plugging, air trapping, and bronchiectasis are important outcome measures in assessing progressive childhood cystic fibrosis lung disease"],"prefix":"10.1002","volume":"55","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4555-3923","authenticated-orcid":false,"given":"Terry E.","family":"Robinson","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Center of Excellence in Pulmonary Biology Stanford University School of Medicine  Stanford California"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael 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