{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T23:14:22Z","timestamp":1791242062973,"version":"4.1.0"},"reference-count":26,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2013,10,25]],"date-time":"2013-10-25T00:00:00Z","timestamp":1382659200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Division of Pediatric Allergy, Immunology, and Pulmonary Medicine at the University of Iowa Children's Hospital"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2014,10]]},"abstract":"<jats:title>Summary<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Tracheomalacia (TM) occurs in approximately 1 in 2,100 children. Because the trachea develops abnormally in animal models of cystic fibrosis (CF), we hypothesized this may also occur in children with CF, increasing their risk of TM.<\/jats:p><\/jats:sec><jats:sec><jats:title>Purpose<\/jats:title><jats:p>To examine the prevalence and clinical consequences of TM in children with CF.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We studied children with CF born between 1995 and 2012. TM was defined as dynamic collapse of the trachea, and the severity was recorded as described in the chart. The effect of TM on patient outcomes, including FEV<jats:sub>1<\/jats:sub>, CT changes, and acquisition of CF pathogens, was assessed using a longitudinal patient dataset.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Eighty\u2010nine percent of children with CF had at least one bronchoscopy (n\u2009=\u200997\/109). Fifteen percent of these children had TM described in any bronchoscopy report (n\u2009=\u200915\/97). Of the patients with TM, eight had meconium ileus (<jats:italic>P<\/jats:italic>\u2009=\u20090.003) and all were pancreatic insufficient. <jats:italic>Pseudomonas aeruginosa<\/jats:italic> infection occurred 1.3 years earlier among children with TM (<jats:italic>P<\/jats:italic>\u2009=\u20090.01). Starting FEV<jats:sub>1<\/jats:sub> values by age 8 were diminished by over 18% of predicted for patients with TM. Life\u2010threatening episodes of airway obstruction occurred in 3 of 15 patients with CF and TM, including one leading to death. Gender, prematurity, and hepatic disease were not associated with TM. No difference was observed in the frequency of bronchiectasis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>TM is significantly more common in infants and children with CF than in the general population and is associated with airway obstruction and earlier <jats:italic>Pseudomonas<\/jats:italic> acquisition. <jats:bold>Pediatr Pulmonol. 2014; 49:960\u2013970.<\/jats:bold> \u00a9 2013 Wiley Periodicals, Inc.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.22922","type":"journal-article","created":{"date-parts":[[2013,10,26]],"date-time":"2013-10-26T04:13:12Z","timestamp":1382760792000},"page":"960-970","source":"Crossref","is-referenced-by-count":50,"title":["Tracheomalacia is associated with lower FEV<sub>1<\/sub> and <i>Pseudomonas<\/i> acquisition in children with CF"],"prefix":"10.1002","volume":"49","author":[{"given":"Anthony J.","family":"Fischer","sequence":"first","affiliation":[{"name":"Department of Pediatrics University of Iowa Iowa City Iowa"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sachinkumar B.","family":"Singh","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Iowa Iowa City Iowa"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryan J.","family":"Adam","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine University of Iowa Iowa City Iowa"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David A.","family":"Stoltz","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine University of Iowa Iowa City Iowa"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher F.","family":"Baranano","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology University of Iowa Iowa City Iowa"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simon","family":"Kao","sequence":"additional","affiliation":[{"name":"Department of Radiology 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