{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Meyerholz DK"],"funding":["NIDDK NIH HHS","NIAID NIH HHS","NHLBI NIH HHS"],"pagination":["1251-61"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC3001264"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["182(10)"],"pubmed_abstract":["<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 h"],"journal":["American journal of respiratory and critical care medicine"],"pubmed_title":["Loss of cystic fibrosis transmembrane conductance regulator function produces abnormalities in tracheal development in neonatal pigs and young children."],"pmcid":["PMC3001264"],"funding_grant_id":["DK54759","P30 DK054759","HL091842","AI076671","HL51670"],"pubmed_authors":["Welsh MJ","Kao S","Namati E","Ramachandran S","Meyerholz DK","Smith AR","Stoltz DA","Suter MJ","McLennan G","Tearney GJ","Rector MV","Zabner J","McCray PB","Pezzulo AA"],"additional_accession":[]},"is_claimable":false,"name":"Loss of cystic fibrosis transmembrane conductance regulator function produces abnormalities in tracheal development in neonatal pigs and young children.","description":"<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 h","dates":{"release":"2010-01-01T00:00:00Z","publication":"2010 Nov","modification":"2026-05-04T13:58:37.771Z","creation":"2019-03-27T00:37:22Z"},"accession":"S-EPMC3001264","cross_references":{"pubmed":["20622026"],"doi":["10.1164/rccm.201004-0643OC","10.1164/rccm.201004-0643oc"]}}