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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 analy…

Initial acquisition and succession of the cystic fibrosis lung microbiome is associated with disease progression in infants and preschool children

We characterized the lower airways microbiome using BAL samples obtained from clinically stable CF young children who underwent bronchoscopy and ches…

Accumulation mode particles and LPS exposure induce TLR-4 dependent and independent inflammatory responses in the lung

We aimed to delineate the effects of LPS and AMP on airway inflammation, and potential contribution to airway disease by measuring airway inflammator…

Conditionally reprogrammed primary airway epithelial cells maintain morphology, lineage and disease specific functional characteristics

Here, we show that conditionally reprogrammed airway epithelial cells (CRAECs) can be established from both healthy and diseased phenotypes.

Oxidative stress in early cystic fibrosis lung disease is exacerbated by airway glutathione deficiency

are oxidative stress or glutathione status associated with brochiectasis and whether glutathione deficiency is inherently linked to CF or from oxidat…

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.

Effect of posture on lung ventilation distribution and associations with structure in children with cystic fibrosis

Background: We assessed the effect of posture on ventilation distribution and the impact on associations with structural lung disease.

Air trapping in early cystic fibrosis lung disease-Does CT tell the full story?

Mosaic attenuation on expiratory chest computed tomography (CT) is common in early life cystic fibrosis (CF) and often referred to as "air trapping"

Multiple-breath washout outcomes are sensitive to inflammation and infection in children with cystic fibrosis

The lung clearance index may be a useful surveillance tool for monitoring the presence and extent of lower airway inflammation and infection