{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,19]],"date-time":"2026-08-19T20:12:41Z","timestamp":1787170361474,"version":"build-2736575974"},"reference-count":40,"publisher":"Oxford University Press (OUP)","issue":"5","license":[{"start":{"date-parts":[[2016,3,1]],"date-time":"2016-03-01T00:00:00Z","timestamp":1456790400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"funder":[{"DOI":"10.13039\/501100003130","name":"FWO","doi-asserted-by":"publisher","award":["12G8715N"],"award-info":[{"award-number":["12G8715N"]}],"id":[{"id":"10.13039\/501100003130","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2016,3,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Rationale<\/jats:title>\n                    <jats:p>After repeated cycles of lung infection and inflammation, patients with cystic fibrosis (CF) evolve to respiratory insufficiency. Although histology and imaging have provided descriptive information, a thorough morphometric analysis of end-stage CF lung disease is lacking.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>To quantify the involvement of small and large airways in end-stage CF.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Multidetector computed tomography (MDCT) and micro-CT were applied to 11 air-inflated CF explanted lungs and 7 control lungs to measure, count, and describe the airway and parenchymal abnormalities in end-stage CF lungs. Selected abnormalities were further investigated with thin section histology.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Measurements and Main Results<\/jats:title>\n                    <jats:p>On MDCT, CF explanted lungs showed an increased median (interquartile range) number (631 [511\u2013710] vs. 344 [277\u2013349]; P\u2009=\u20090.003) and size of visible airways (cumulative airway diameter 217 cm [209\u2013250] vs. 91 cm [80\u2013105]; P\u2009&amp;lt;\u20090.001) compared with controls. Airway obstruction was seen, starting from generation 6 and increasing to 40 to 50% of airways from generation 9 onward. Micro-CT showed that the total number of terminal bronchioles was decreased (2.9\/ml [2.6\u20134.4] vs. 5.3\/ml [4.8\u20135.7]; P\u2009&amp;lt;\u20090.001); 49% were obstructed, and the cross-sectional area of the open terminal bronchioles was reduced (0.093 mm2 [0.084\u20130.123] vs. 0.179 mm2 [0.140\u20130.196]; P\u2009&amp;lt;\u20090.001). On micro-CT, 41% of the obstructed airways reopened more distally. This remodeling was confirmed on histological analysis. Parenchymal changes were also seen, mostly in a patchy and peribronchiolar distribution.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Extensive changes of dilatation and obstruction in nearly all airway generations were observed in end-stage CF lung disease.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1164\/rccm.201507-1281oc","type":"journal-article","created":{"date-parts":[[2015,11,9]],"date-time":"2015-11-09T13:16:36Z","timestamp":1447074996000},"page":"516-526","source":"Crossref","is-referenced-by-count":63,"title":["Morphometric Analysis of Explant Lungs in Cystic Fibrosis"],"prefix":"10.1093","volume":"193","author":[{"given":"Mieke","family":"Boon","sequence":"first","affiliation":[{"name":"Pediatric Pulmonology and Cystic Fibrosis Unit, Department of Pediatrics"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stijn E.","family":"Verleden","sequence":"additional","affiliation":[{"name":"Lung Transplant Unit, Laboratory of Pulmonology, Department of Clinical and Experimental Medicine, KU Leuven\u2014University of Leuven, Leuven, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Barbara","family":"Bosch","sequence":"additional","affiliation":[{"name":"Pediatric Pulmonology and Cystic Fibrosis Unit, Department of Pediatrics"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elise J.","family":"Lammertyn","sequence":"additional","affiliation":[{"name":"Lung Transplant Unit, Laboratory of Pulmonology, Department of Clinical and Experimental Medicine, KU Leuven\u2014University of Leuven, Leuven, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John E.","family":"McDonough","sequence":"additional","affiliation":[{"name":"Lung Transplant Unit, Laboratory of Pulmonology, Department of Clinical and Experimental Medicine, KU Leuven\u2014University of Leuven, Leuven, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cindy","family":"Mai","sequence":"additional","affiliation":[{"name":"Department of Pulmonary Radiology, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Johny","family":"Verschakelen","sequence":"additional","affiliation":[{"name":"Department of Pulmonary Radiology, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mariette","family":"Kemner-van de Corput","sequence":"additional","affiliation":[{"name":"Department of Radiology and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Harm A. W.","family":"Tiddens","sequence":"additional","affiliation":[{"name":"Department of Radiology and"},{"name":"Department of Paediatrics, Respiratory Medicine and Allergology, Erasmus MC, Rotterdam, the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marijke","family":"Proesmans","sequence":"additional","affiliation":[{"name":"Pediatric Pulmonology and Cystic Fibrosis Unit, Department of Pediatrics"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fran\u00e7ois L.","family":"Vermeulen","sequence":"additional","affiliation":[{"name":"Pediatric Pulmonology and Cystic Fibrosis Unit, Department of Pediatrics"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erik K.","family":"Verbeken","sequence":"additional","affiliation":[{"name":"Department of Pathology, University Hospitals Leuven, Leuven, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joel","family":"Cooper","sequence":"additional","affiliation":[{"name":"Department of Thoracic Surgery, University of Pennsylvania, Philadelphia, Pennsylvania; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dirk E.","family":"Van Raemdonck","sequence":"additional","affiliation":[{"name":"Lung Transplant Unit, Laboratory of Pulmonology, Department of Clinical and Experimental Medicine, KU Leuven\u2014University of Leuven, Leuven, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marc","family":"Decramer","sequence":"additional","affiliation":[{"name":"Lung Transplant Unit, Laboratory of Pulmonology, Department of Clinical and Experimental Medicine, KU Leuven\u2014University of Leuven, Leuven, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Geert M.","family":"Verleden","sequence":"additional","affiliation":[{"name":"Lung Transplant Unit, 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