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Aspergillus species and P. aeruginosa are commonly present in the lower airways from infancy
The aim of this study is to identify and quantify the preferences of patients with cystic fibrosis regarding treatment outcomes
Acinar ventilation inhomogeneity measured by single-breath washout was not associated with structural lung disease on CT
Structural changes identified on CT scans in primary ciliary dyskinesia are not identical to those previously described in cystic fibrosis patients
Protease elastase exocytosis by airway neutrophils occurs in all cystic fibrosis children, and its cellular measure correlates with early lung damage
This review will discuss the necessary steps required for a Bayesian adaptive platform trial to optimize treatment of pulmonary exacerbations of cystic fibrosis
In this Review, we consolidate the latest updates in the development of primary epithelial cellular model systems relevant for cystic fibrosis
Our data associates IL-1α with early structural lung damage in CF and suggests this pathway as a novel anti-inflammatory target
This study demonstrates the feasibility of utilizing pre-clinical in vitro culture models to screen therapeutic candidates
Whether airway mucosal acidification drives early progressive lung disease is controversial