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Pseudomonas aeruginosa modulates neutrophil granule exocytosis in an in vitro model of airway infectionA population of neutrophils recruited into cystic fibrosis (CF) airways is associated with proteolytic lung damage, exhibiting high expression of primary granule exocytosis marker CD63 and reduced phagocytic receptor CD16. Causative factors for this population are unknown, limiting intervention. Here we present a laboratory model to characterize responses of differentiated airway epithelium and neutrophils following respiratory infection.
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Lentiviral vector gene therapy and CFTR modulators show comparable effectiveness in cystic fibrosis rat airway modelsMutation-agnostic treatments such as airway gene therapy have the potential to treat any individual with cystic fibrosis (CF), irrespective of their CF transmembrane conductance regulator (CFTR) gene variants. The aim of this study was to employ two CF rat models, Phe508del and CFTR knockout to assess the comparative effectiveness of CFTR modulators and lentiviral vector-mediated gene therapy.
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Phage therapy to treat cystic fibrosis Burkholderia cepacia complex lung infections: perspectives and challengesgeBurkholderia cepacia complex is a cause of serious lung infections in people with cystic fibrosis, exhibiting extremely high levels of antimicrobial resistance. These infections are difficult to treat and are associated with high morbidity and mortality.
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Multiple-breath washout outcomes are sensitive to inflammation and infection in children with cystic fibrosisThe lung clearance index may be a useful surveillance tool for monitoring the presence and extent of lower airway inflammation and infection
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The association between Staphylococcus aureus and subsequent bronchiectasis in children with cystic fibrosisDe novo S. aureus acquisition at age 3 is associated with later bronchiectasis and FEF25-75 in children with CF
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Human Primary Epithelial Cell Models: Promising Tools in the Era of Cystic Fibrosis Personalized MedicineIn this Review, we consolidate the latest updates in the development of primary epithelial cellular model systems relevant for cystic fibrosis
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Trial refresh: A case for an adaptive platform trial for pulmonary exacerbations of cystic fibrosisThis review will discuss the necessary steps required for a Bayesian adaptive platform trial to optimize treatment of pulmonary exacerbations of cystic fibrosis
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Structural determinants of long term functional outcomes in young children with cystic fibrosisChest CT identifies children at an early age who have adverse long-term outcomes
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Elastase Exocytosis by Airway Neutrophils Associates with Early Lung Damage in Cystic Fibrosis ChildrenProtease elastase exocytosis by airway neutrophils occurs in all cystic fibrosis children, and its cellular measure correlates with early lung damage
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Early lung disease in infants and preschool children with cystic fibrosis: What have we learned and what should we do about it?This review summarizes what we have learned about early lung disease in children with CF and discusses the implications for clinical practice and research