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Assessment of p.Phe508del-CFTR functional restoration in pediatric primary cystic fibrosis airway epithelial cells

This 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.

Quantitative assessment of airway dimensions in young children with cystic fibrosis lung disease using chest computed tomography

To evaluate lung disease progression using AA dimensions on chest CT over 2-years in young CF patients longitudinally and compare to disease controls.

Airway surface liquid pH is not acidic in children with cystic fibrosis

Modulation of ASL pH has been proposed as a therapy for CF. However, evidence that ASL pH is reduced in CF is limited and conflicting.

The potential of phage therapy in cystic fibrosis

This review summarises the phage-microbe-human lung interactions in CF that must be addressed to successfully develop and deliver phage to CF airways

CFTR-dependent defect in alternatively-activated macrophages in cystic fibrosis

CFTR-dependent imbalance of macrophage phenotypes and functions could contribute to the exaggerated inflammatory response seen in CF lung disease

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

Of pigs, mice, and men: Understanding early triggers of cystic fibrosis lung disease

Understanding early triggers of Cystic Fibrosis Lung Disease

Dissecting the regulation of bile-induced biofilm formation in staphylococcus aureus

Wall teichoic acid may be important for protecting S. aureus against exposure to bile

Bile signalling promotes chronic respiratory infections and antibiotic tolerance

Bile acid signalling is a leading trigger for the development of chronic phenotypes underlying the pathophysiology of chronic respiratory disease

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