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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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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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CF derived scoring systems do not fully describe the range of structural changes seen on CT scans in PCDStructural changes identified on CT scans in primary ciliary dyskinesia are not identical to those previously described in cystic fibrosis patients
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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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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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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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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
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Quality of life is poorly correlated to lung disease severity in school-aged children with cystic fibrosisThere is no data exclusively on the relationship between health-related quality-of-life (HRQOL) and lung disease severity in early school-aged children with cystic fibrosis (CF). Using data from the Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF) we assessed the relationships between HRQOL, lung function and structure.
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Methods used to evaluate the immediate effects of airway clearance techniques in adults with cystic fibrosis: A systematic review and meta-analysisThis review reports on methods used to evaluate airway clearance techniques (ACT) in adults with CF and examined data for evidence of any effect. Sixty-eight studies described ACT in adequate detail and were included in this review.