{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,4]],"date-time":"2026-06-04T05:25:54Z","timestamp":1780550754523,"version":"3.54.1"},"reference-count":30,"publisher":"American Physiological Society","issue":"3","funder":[{"DOI":"10.13039\/100000050","name":"HHS | NIH | National Heart, Lung, and Blood Institute (NHBLI)","doi-asserted-by":"publisher","award":["DP2 117744"],"award-info":[{"award-number":["DP2 117744"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"HHS | NIH | National Heart, Lung, and Blood Institute (NHBLI)","doi-asserted-by":"publisher","award":["T32 007638"],"award-info":[{"award-number":["T32 007638"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"HHS | NIH | National Heart, Lung, and Blood Institute (NHBLI)","doi-asserted-by":"publisher","award":["P01 051670"],"award-info":[{"award-number":["P01 051670"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"HHS | NIH | National Heart, Lung, and Blood Institute (NHBLI)","doi-asserted-by":"publisher","award":["P01 091842"],"award-info":[{"award-number":["P01 091842"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"name":"CFF","award":["STOLTZ14XX0"],"award-info":[{"award-number":["STOLTZ14XX0"]}]},{"name":"NIH","award":["T32 GM007337"],"award-info":[{"award-number":["T32 GM007337"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Journal of Applied Physiology"],"published-print":{"date-parts":[[2017,9,1]]},"abstract":"<jats:p> Mutations in the gene encoding the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) anion channel cause CF. The leading cause of death in the CF population is lung disease. Increasing evidence suggests that in utero airway development is CFTR-dependent and that developmental abnormalities may contribute to CF lung disease. However, relatively little is known about postnatal CF airway growth, largely because such studies are limited in humans. Therefore, we examined airway growth and lung volume in a porcine model of CF. We hypothesized that CF pigs would have abnormal postnatal airway growth. To test this hypothesis, we performed CT-based airway and lung volume measurements in 3-wk-old non-CF and CF pigs. We found that 3-wk-old CF pigs had tracheas of reduced caliber and irregular shape. Their bronchial lumens were reduced in size proximally but not distally, were irregularly shaped, and had reduced distensibility. Our data suggest that lack of CFTR results in aberrant postnatal airway growth and development, which could contribute to CF lung disease pathogenesis. <\/jats:p><jats:p> NEW &amp; NOTEWORTHY This CT scan-based study of airway morphometry in the cystic fibrosis (CF) postnatal period is unique, as analogous studies in humans are greatly limited for ethical and technical reasons. Findings such as reduced airway lumen area and irregular caliber suggest that airway growth and development are CF transmembrane conductance regulator-dependent and that airway growth defects may contribute to CF lung disease pathogenesis. <\/jats:p>","DOI":"10.1152\/japplphysiol.00263.2017","type":"journal-article","created":{"date-parts":[[2017,6,15]],"date-time":"2017-06-15T21:10:40Z","timestamp":1497561040000},"page":"526-533","source":"Crossref","is-referenced-by-count":7,"title":["Postnatal airway growth in cystic fibrosis piglets"],"prefix":"10.1152","volume":"123","author":[{"given":"Ryan J.","family":"Adam","sequence":"first","affiliation":[{"name":"Department of Internal Medicine, University of Iowa Roy J. and Lucille A. Carver College of Medicine, Iowa City, Iowa;"},{"name":"Department of Biomedical Engineering, University of Iowa Roy J. and Lucille A. 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