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New drug offers hope for people living with cystic fibrosisA promising new treatment pioneered in Western Australia for people with cystic fibrosis has commenced testing in a clinical trial in the United States and Australia.
Research
Monocytes from children with clinically stable cystic fibrosis show enhanced expression of Toll-like receptor 4Lung disease in patients with cystic fibrosis (CF) is characterized by recurrent bacterial respiratory infections and intense airway inflammation.
Research
Lung function testing in preschool-aged children with cystic fibrosis in the clinical settingThis study investigated the nature and prevalence of atypical pain responses in Rett syndrome and their relationships with specific MECP2 mutations.
The Airway Epithelial Research Team is investigating the role of the epithelium in the development of airway diseases including asthma, cystic fibrosis and lung transplant rejection.
Research
Role of Tris-CaEDTA as an adjuvant with nebulised tobramycin in cystic fibrosis patients with Pseudomonas aeruginosa lung infections: A randomised controlled trialWe tested if disrupting iron utilisation by P. aeruginosa by adding the Tris-buffered chelating agent CaEDTA to nebulised tobramycin would enhance bacterial clearance and improve lung function in CF patients.
Research
Changes in airway inflammation with pseudomonas eradication in early cystic fibrosisNeutrophil elastase is a significant risk factor for structural lung disease in cystic fibrosis, and Pseudomonas aeruginosa airway infection is linked with neutrophilic inflammation and substantial respiratory morbidity. We aimed to evaluate how neutrophil elastase (NE) activity changes after P. aeruginosa eradication and influences early disease outcomes. We assessed participants in the AREST CF cohort between 2000 and 2018 who had P. aeruginosa cultured from their routine annual bronchoalveolar lavage (BAL) fluid and who underwent eradication treatment and a post eradication BAL. Factors associated with persistent P. aeruginosa infection, persistent neutrophilic inflammation following eradication and worse structural lung disease one year post-eradication were evaluated.
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Lung Clearance Index and Structural Lung Disease on Computed Tomography in Early Cystic FibrosisLung clearance index may be a useful surveillance tool to monitor structural lung disease in preschool and school-age children with cystic fibrosis
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Respiratory tract exacerbations revisited: Ventilation, inflammation, perfusion, and structure (VIPS) monitoring to redefine treatmentFor cystic fibrosis (CF) patients older than 6 years there are convincing data that suggest respiratory tract exacerbations (RTE) play an important role in...
Research
Early cystic fibrosis lung diseaseThis paper is about lung disease in patients with cyctic Fibrosis and prevention strategies to slow the onset of lung disease.
Research
Impact of lung disease on respiratory impedance in young children with cystic fibrosisThe present study investigated whether lung function measured by forced oscillation technique would be impaired in the presence of infection,...