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Assessment of p.Phe508del-CFTR functional restoration in pediatric primary cystic fibrosis airway epithelial cellsThis study assessed an alternative approach, using a small scale halide assay that can be adapted for a personalized high throughput setting to analyze CFTR function of pAEC.
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Quantitative assessment of airway dimensions in young children with cystic fibrosis lung disease using chest computed tomographyTo evaluate lung disease progression using AA dimensions on chest CT over 2-years in young CF patients longitudinally and compare to disease controls.
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Airway surface liquid pH is not acidic in children with cystic fibrosisModulation of ASL pH has been proposed as a therapy for CF. However, evidence that ASL pH is reduced in CF is limited and conflicting.
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The potential of phage therapy in cystic fibrosisThis review summarises the phage-microbe-human lung interactions in CF that must be addressed to successfully develop and deliver phage to CF airways
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CFTR-dependent defect in alternatively-activated macrophages in cystic fibrosisCFTR-dependent imbalance of macrophage phenotypes and functions could contribute to the exaggerated inflammatory response seen in CF lung disease
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Early lung surveillance of cystic fibrosis: what have we learnt?Newborn screening (NBS) for cystic fibrosis (CF) provides an opportunity to commence management and therapeutic interventions significantly earlier
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Of pigs, mice, and men: Understanding early triggers of cystic fibrosis lung diseaseUnderstanding early triggers of Cystic Fibrosis Lung Disease
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Dissecting the regulation of bile-induced biofilm formation in staphylococcus aureusWall teichoic acid may be important for protecting S. aureus against exposure to bile
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Bile signalling promotes chronic respiratory infections and antibiotic toleranceBile acid signalling is a leading trigger for the development of chronic phenotypes underlying the pathophysiology of chronic respiratory disease
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Airway epithelial repair in health and disease: Orchestrator or simply a player?This review attempts to highlight migration-specific and cell-extracellular matrix (ECM) aspects of repair used by epithelial cells