biostudies-literatureMeyerholz DKNIDDK NIH HHSNIAID NIH HHSNHLBI NIH HHS1251-61https://www.ebi.ac.uk/biostudies/studies/S-EPMC3001264biostudies-literatureUnknown182(10)<h4>Rationale</h4>Although airway abnormalities are common in patients with cystic fibrosis (CF), it is unknown whether they are all secondary to postnatal infection and inflammation, which characterize the disease.<h4>Objectives</h4>To learn whether loss of the cystic fibrosis transmembrane conductance regulator (CFTR) might affect major airways early in life, before the onset of inflammation and infection.<h4>Methods</h4>We studied newborn CFTR⁻(/)⁻ pig trachea, using computed tomography (CT) scans, pathology, and morphometry. We retrospectively analyzed trachea CT scans in young children with CF and also previously published data of infants with CF.<h4>Measurements and main results</h4>We discovered three abnormalities in the porcine CF trachea. First, the trachea and mainstem bronchi hAmerican journal of respiratory and critical care medicineLoss of cystic fibrosis transmembrane conductance regulator function produces abnormalities in tracheal development in neonatal pigs and young children.PMC3001264DK54759P30 DK054759HL091842AI076671HL51670Welsh MJKao SNamati ERamachandran SMeyerholz DKSmith ARStoltz DASuter MJMcLennan GTearney GJRector MVZabner JMcCray PBPezzulo AAfalseLoss of cystic fibrosis transmembrane conductance regulator function produces abnormalities in tracheal development in neonatal pigs and young children.<h4>Rationale</h4>Although airway abnormalities are common in patients with cystic fibrosis (CF), it is unknown whether they are all secondary to postnatal infection and inflammation, which characterize the disease.<h4>Objectives</h4>To learn whether loss of the cystic fibrosis transmembrane conductance regulator (CFTR) might affect major airways early in life, before the onset of inflammation and infection.<h4>Methods</h4>We studied newborn CFTR⁻(/)⁻ pig trachea, using computed tomography (CT) scans, pathology, and morphometry. We retrospectively analyzed trachea CT scans in young children with CF and also previously published data of infants with CF.<h4>Measurements and main results</h4>We discovered three abnormalities in the porcine CF trachea. First, the trachea and mainstem bronchi h2010-01-01T00:00:00Z2010 Nov2026-05-04T13:58:37.771Z2019-03-27T00:37:22ZS-EPMC30012642062202610.1164/rccm.201004-0643OC10.1164/rccm.201004-0643oc