{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T23:55:34Z","timestamp":1792713334738,"version":"4.5.0"},"reference-count":43,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2025,9,18]],"date-time":"2025-09-18T00:00:00Z","timestamp":1758153600000},"content-version":"vor","delay-in-days":17,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2025,9]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Rationale<\/jats:title>\n                    <jats:p>The longitudinal trajectory of airway disease on chest computed tomography (CT) and risk factors for progression in primary ciliary dyskinesia (PCD) is largely unknown.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Longitudinal analysis of structural airway disease among children with PCD enrolled in a prospective, multicenter observational study, stratified by ultrastructural defect group. CTs were scored utilizing the Melbourne\u2010Rotterdam Annotated Grid Morphometric Analysis for PCD, evaluating airway abnormalities in a hierarchical order: atelectasis, bronchiectasis (%BE), airway wall thickening, and mucus plugging\/tree in bud opacities (%MP). Volume fraction of each component was expressed as % of total lung volume. %Disease (%DIS) is the sum of all components. Mixed effects regression analyses were utilized to describe the association between clinical predictors and CT scores adjusted for relevant covariates.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Chest CTs (\n                      <jats:italic>N<\/jats:italic>\n                      \u2009=\u2009424) were obtained from 142 children over a mean (SD) of 4.9 (3.7) years. Mean (SD) age at first CT was 8.5 (4.6) years. Mean %DIS increased with age for the cohort (0.09% per year, 95% CI: 0.02\u20130.15,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.01), driven by increased %BE (0.06 higher per year, 95% CI: 0.04\u20130.08,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.0001) and increased odds of MP (1.14 higher odds per year, 95% CI: 1.08\u20131.21,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.0001). Children with inner dynein arm and microtubular defects had increased mean %DIS and increased odds of MP compared to other ciliary abnormalities (1.73% higher, 95% CI: 0.71\u20132.74,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.001; 3.21 higher odds per year, 95% CI: 1.73\u20135.95,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001). Annual increase in CT scores did not differ between defect groups.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Children with PCD experience progressive bronchiectasis and worsening mucus plugging with age.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ppul.71287","type":"journal-article","created":{"date-parts":[[2025,9,18]],"date-time":"2025-09-18T15:13:57Z","timestamp":1758208437000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Airway Disease Progression on Chest Computed Tomography in Children With Primary Ciliary Dyskinesia"],"prefix":"10.1002","volume":"60","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3522-5387","authenticated-orcid":false,"given":"BreAnna","family":"Kinghorn","sequence":"first","affiliation":[{"name":"Department of Pediatrics University of Washington School of Medicine Seattle Washington USA"},{"name":"Seattle Children\u2032s Research Institute, Seattle Children\u2032s Hospital Seattle Washington USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Federico","family":"Mollica","sequence":"additional","affiliation":[{"name":"Erasmus MC and Department of Pediatric Pulmonology and Radiology Erasmus MC Rotterdam the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8191-8598","authenticated-orcid":false,"given":"Daan","family":"Caudri","sequence":"additional","affiliation":[{"name":"Erasmus MC and Department of Pediatric Pulmonology and Radiology Erasmus MC Rotterdam the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephanie D.","family":"Davis","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of North Carolina School of Medicine Chapel Hill North Carolina USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sharon","family":"Dell","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of British Columbia Vancouver British Columbia Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas W.","family":"Ferkol","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of North Carolina School of Medicine Chapel Hill North Carolina USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9123-0762","authenticated-orcid":false,"given":"Robert Andrew","family":"Gardner","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Levine Children\u2032s Hospital Wake Forest University School of Medicine Charlotte North Carolina USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael R.","family":"Knowles","sequence":"additional","affiliation":[{"name":"Pulmonary Medicine Division, Marsico Lung Institute University of North Carolina School of Medicine Chapel Hill North Carolina USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlos","family":"Milla","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Stanford University Palo Alto California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jessica E.","family":"Pittman","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Washington University School of Medicine St. Louis Missouri USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margaret","family":"Rosenfeld","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Washington School of Medicine Seattle Washington USA"},{"name":"Seattle Children\u2032s Research Institute, Seattle Children\u2032s Hospital Seattle Washington 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