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Automatic bronchus and artery analysis on chest computed tomography to evaluate the effect of inhaled hypertonic saline in children aged 3-6 years with cystic fibrosis in a randomized clinical trialSHIP-CT showed that 48-week treatment with inhaled 7% hypertonic saline (HS) reduced airway abnormalities on chest CT using the manual PRAGMA-CF method relative to isotonic saline (IS) in children aged 3-6 years with cystic fibrosis (CF). An algorithm was developed and validated to automatically measure bronchus and artery (BA) dimensions of BA-pairs on chest CT. Aim of the study was to assess the effect of HS on bronchial wall thickening and bronchial widening using the BA-analysis.
Research
Overcoming Challenges to Make Bacteriophage Therapy Standard Clinical Treatment Practice for Cystic FibrosisIndividuals with cystic fibrosis (CF) are given antimicrobials as prophylaxis against bacterial lung infection, which contributes to the growing emergence of multidrug resistant (MDR) pathogens isolated. Pathogens such as Pseudomonas aeruginosa that are commonly isolated from individuals with CF are armed with an arsenal of protective and virulence mechanisms, complicating eradication and treatment strategies.
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Time to get serious about the detection and monitoring of early lung disease in cystic fibrosisStructural and functional defects within the lungs of children with cystic fibrosis (CF) are detectable soon after birth and progress throughout preschool years often without overt clinical signs or symptoms. By school age, most children have structural changes such as bronchiectasis or gas trapping/hypoperfusion and lung function abnormalities that persist into later life. Despite improved survival, gains in forced expiratory volume in one second (FEV1) achieved across successive birth cohorts during childhood have plateaued, and rates of FEV1 decline in adolescence and adulthood have not slowed. This suggests that interventions aimed at preventing lung disease should be targeted to mild disease and commence in early life.
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Hypoxia and sterile inflammation in cystic fibrosis airways: Mechanisms and potential therapiesIn this review, we consider recent evidence regarding hypoxia and sterile inflammation in cystic fibrosis airways
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The effect of 100% oxygen on tidal breathing parameters in preschool childrenThis study aimed to determine whether breathing 100% oxygen altered tidal breathing parameters in healthy young children and young children with CF.
Research
Protocol for a study of the psychosocial determinants of health in early childhood among children with cystic fibrosisThis protocol outlines the study aims to investigate the causal effect of psychosocial functioning, parenting and attachment on physical health outcomes in...
Research
Alpha-1 antitrypsin mitigates the inhibition of airway epithelial cell repair by neutrophil elastaseFree NE activity is deleterious for epithelial homeostasis and support the hypothesis that proteases in the airway contribute to CF structural lung disease
News & Events
Cystic fibrosis research a Eureka Prize finalistA 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 lifeThe lungs are one of the last organs in the body to develop as a baby grows. They're also one of the most important.
