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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
Progress in Model Systems of Cystic Fibrosis Mucosal Inflammation to Understand Aberrant Neutrophil ActivityHere we examine the latest findings of neutrophils in pediatric CF lung disease and proposed mechanisms of their pathogenicity
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.
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
Single-breath washout and association with structural lung disease in children with cystic fibrosisAcinar ventilation inhomogeneity measured by single-breath washout was not associated with structural lung disease on CT
Research
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
Reversibility of trapped air on chest computed tomography in cystic fibrosis patientsTo investigate changes in trapped air volume and distribution over time and compare computed tomography (CT) with pulmonary function tests for determining...
Research
Parental experiences of early pulmonary surveillance for children with cystic fibrosis: A research proposal for improved family psychosocial outcomesThe proposed study intends to explore parental experiences, including coping, related to their child's involvement in the early surveillance program.
Research
Interleukin-1 is associated with inflammation and structural lung disease in young children with cystic fibrosisOur data associates IL-1α with early structural lung damage in CF and suggests this pathway as a novel anti-inflammatory target