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Research

Alpha-1 antitrypsin mitigates the inhibition of airway epithelial cell repair by neutrophil elastase

Free NE activity is deleterious for epithelial homeostasis and support the hypothesis that proteases in the airway contribute to CF structural lung disease

Research

Hypoxia and sterile inflammation in cystic fibrosis airways: Mechanisms and potential therapies

In this review, we consider recent evidence regarding hypoxia and sterile inflammation in cystic fibrosis airways

Research

The effect of 100% oxygen on tidal breathing parameters in preschool children

This study aimed to determine whether breathing 100% oxygen altered tidal breathing parameters in healthy young children and young children with CF.

Research

Regional Differences in Susceptibiity of Bronchial Epithelium to Mesenchymal Transition and Inhibition by the Macrolide Antibiotic Azithromycin

Dysregulated repair following epithelial injury is a key forerunner of disease in many organs, and the acquisition of a mesenchymal phenotype by the injured...

Research

Infection, inflammation,and lung function decline in infants with cystic fibrosis

Better understanding of evolution of lung function in infants with cystic fibrosis...

News & Events

On a quest for kids with cystic fibrosis

PRAGMA-CF, a new way of measuring early lung disease in young kids with cystic fibrosis is changing the way we detect and treat CF.

News & Events

Cystic fibrosis research a Eureka Prize finalist

A world-leading cystic fibrosis research program, based at The Kids Research Institute Australia, is a finalist in the 2015 Australian Museum Eureka Prizes.

News & Events

Healthy lungs, healthy life

The lungs are one of the last organs in the body to develop as a baby grows. They're also one of the most important.

Research

Airway macrophages display decreased expression of receptors mediating and regulating scavenging in early cystic fibrosis lung disease

Cystic fibrosis (CF) airway disease is characterized by chronic inflammation, featuring neutrophil influx to the lumen. Airway macrophages (AMs) can promote both inflammation and resolution, and are thus critical to maintaining and restoring homeostasis. CF AM functions, specifically scavenging activity and resolution of inflammation, have been shown to be impaired, yet underlying processes remain unknown.