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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

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

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.

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.

Stability Considerations for Bacteriophages in Liquid Formulations Designed for Nebulization

Pulmonary bacterial infections present a significant health risk to those with chronic respiratory diseases including cystic fibrosis and chronic-obstructive pulmonary disease. With the emergence of antimicrobial resistance, novel therapeutics are desperately needed to combat the emergence of resistant superbugs.

Simultaneous multiple breath washout and oxygen-enhanced magnetic resonance imaging in healthy adults

Lung function testing and lung imaging are commonly used techniques to monitor respiratory diseases, such as cystic fibrosis (CF). The nitrogen (N2) multiple-breath washout technique (MBW) has been shown to detect ventilation inhomogeneity in CF, but the underlying pathophysiological processes that are altered are often unclear.

Azithromycin reduces bronchial wall thickening in infants with cystic fibrosis

COMBAT-CF showed that children aged 0-3 years treated with azithromycin did clinically better than placebo but there was no effect on CT-scores. We reanalysed CTs using an automatic bronchus-artery (BA) analysis.

The longitudinal microbial and metabolic landscape of infant cystic fibrosis: the gut-lung axis

In cystic fibrosis, gastrointestinal dysfunction and lower airway infection occur early and are independently associated with poorer outcomes in childhood. This study aimed to define the relationship between the microbiota at each niche during the first 2 years of life, its association with growth and airway inflammation, and explanatory features in the metabolome.

Lung structural and functional impairments in young children with cystic fibrosis diagnosed following newborn screening – A nationwide observational study

Non-invasive and sensitive clinical endpoints are needed to monitor onset and progression of early lung disease in children with cystic fibrosis (CF). We compared lung clearance index (LCI), FEV1, functional and structural lung magnetic resonance imaging (MRI) outcomes in Swiss children with CF diagnosed following newborn screening.

Psychosocial characteristics and predictors of health-care use in families of young children with cystic fibrosis in Western Australia

This study aimed to identify psychosocial predictors in cystic fibrosis that may inform intervention strategies.