{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T06:14:44Z","timestamp":1790835284874,"version":"4.1.0"},"reference-count":31,"publisher":"Oxford University Press (OUP)","issue":"10","license":[{"start":{"date-parts":[[2015,5,1]],"date-time":"2015-05-01T00:00:00Z","timestamp":1430438400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2015,5,15]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Rationale<\/jats:title>\n                    <jats:p>Chest computed tomography (CT) is the gold standard for demonstrating cystic fibrosis (CF) airway disease. However, there are no standardized outcome measures appropriate for children younger than 6 years.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>We developed the Perth-Rotterdam Annotated Grid Morphometric Analysis for CF (PRAGMA-CF), a quantitative measure of airway disease, and compared it with the commonly used CF-CT scoring method.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>CT scans from the Australian Respiratory Early Surveillance Team for CF (AREST CF) cohort in Western Australia were included. PRAGMA-CF was performed by annotating a grid overlaid on 10 axial slices for the presence of bronchiectasis, mucous plugging, or other airway abnormalities (inspiratory scans) and trapped air (expiratory scans). The separate proportions of total disease (%Dis), bronchiectasis (%Bx), and trapped air (%TA) were determined. Thirty scans were used for observer reliability, and 30 paired scans obtained at 1 and 3 years old were used for comparison with a validated standard and biologic plausibility.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Measurements and Main Results<\/jats:title>\n                    <jats:p>Intraobserver, intraclass correlation coefficients (95% confidence interval) for %Dis, %Bx, and %TA were 0.93 (0.86\u20130.97), 0.93 (0.85\u20130.96), and 0.96 (0.91\u20130.98), respectively. The change in %Dis (P\u2009=\u20090.004) and %Bx (P\u2009=\u20090.001) with PRAGMA-CF was related to neutrophil elastase presence at age 3, whereas only the change in bronchiectasis score was related to neutrophil elastase (P\u2009&amp;lt;\u20090.001) with CF-CT. Sample-size calculations for various effect sizes are presented.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>PRAGMA-CF is a sensitive and reproducible outcome measure for assessing the extent of lung disease in very young children with CF.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1164\/rccm.201501-0061oc","type":"journal-article","created":{"date-parts":[[2015,3,10]],"date-time":"2015-03-10T17:18:58Z","timestamp":1426007938000},"page":"1158-1165","source":"Crossref","is-referenced-by-count":225,"title":["PRAGMA-CF. A Quantitative Structural Lung Disease Computed Tomography Outcome in Young Children with Cystic Fibrosis"],"prefix":"10.1093","volume":"191","author":[{"given":"Tim","family":"Rosenow","sequence":"first","affiliation":[{"name":"School of Paediatrics and Child Health and"},{"name":"Telethon Kids Institute, The University of Western Australia, Perth, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Merel C. J.","family":"Oudraad","sequence":"additional","affiliation":[{"name":"Department of Pediatric Pulmonology, Erasmus MC-Sophia Children\u2019s Hospital, Rotterdam, the Netherlands"},{"name":"Faculty of Medicine, University of Utrecht, Utrecht, the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Conor P.","family":"Murray","sequence":"additional","affiliation":[{"name":"Department of Diagnostic Imaging, Princess Margaret Hospital for Children, Western Australia, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lidija","family":"Turkovic","sequence":"additional","affiliation":[{"name":"Telethon Kids Institute, The University of Western Australia, Perth, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wieying","family":"Kuo","sequence":"additional","affiliation":[{"name":"Department of Pediatric Pulmonology, Erasmus MC-Sophia Children\u2019s Hospital, Rotterdam, the Netherlands"},{"name":"Department of Radiology and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marleen","family":"de Bruijne","sequence":"additional","affiliation":[{"name":"Biomedical Imaging Group Rotterdam, Department of Medical Informatics, Erasmus MC, Rotterdam, the Netherlands"},{"name":"Department of Computer Science, University of Copenhagen, Copenhagen, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarath C.","family":"Ranganathan","sequence":"additional","affiliation":[{"name":"Infection and Immunity Theme, Murdoch Children\u2019s Research Institute, Melbourne, Australia"},{"name":"Department of Respiratory Medicine, Royal Children\u2019s Hospital Melbourne, Melbourne, Australia"},{"name":"Department of Paediatrics, University of Melbourne, Melbourne, Australia; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Harm A. 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M.","family":"Tiddens","sequence":"additional","affiliation":[{"name":"Department of Pediatric Pulmonology, Erasmus MC-Sophia Children\u2019s Hospital, Rotterdam, the Netherlands"},{"name":"Department of Radiology and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen M.","family":"Stick","sequence":"additional","affiliation":[{"name":"Telethon Kids Institute, The University of Western Australia, Perth, Australia"},{"name":"Department of Respiratory and Sleep Medicine, Princess Margaret Hospital for Children, Perth, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2015,5,15]]},"reference":[{"key":"2026032100195347900_bib1","doi-asserted-by":"crossref","first-page":"509","DOI":"10.1136\/thoraxjnl-2011-200912","article-title":"Progression of early structural lung disease in young children with cystic fibrosis assessed using 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