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BEAT-CF (Bayesian Evidence Adaptive Treatment for people with Cystic Fibrosis): description of a prospective cohort for nested studies in cystic fibrosisDespite recent improvements in treatment modalities for cystic fibrosis (CF), there is currently limited evidence and a lack of consensus regarding optimal treatment strategies for the different aspects of CF, including pulmonary exacerbations (PEx). We aimed to establish a prospective cohort of people with CF (pwCF) to evaluate alternative approaches to managing CF in the era of modulator therapies.
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MetaNeb Versus Usual Care During Exacerbations of Cystic Fibrosis: An RCTDuring exacerbations, when symptom and treatment burden are increased, individuals with cystic fibrosis (CF) are likely to prefer airway clearance techniques (ACTs) that require minimal effort. Therefore, in adults with CF who were hospitalised with an exacerbation, we sought to compare the effect of the MetaNeb with usual ACTs on respiratory function and expectorated sputum.
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Macrophage PD-1 associates with neutrophilia and reduced bacterial killing in early cystic fibrosis airway diseaseMacrophages are the major resident immune cells in human airways coordinating responses to infection and injury. In cystic fibrosis, neutrophils are recruited to the airways shortly after birth, and actively exocytose damaging enzymes prior to chronic infection, suggesting a potential defect in macrophage immunomodulatory function.
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Developing a smartphone application to support social connectedness and wellbeing in young people with cystic fibrosisThis study developed and tested a highly usable, and moderately acceptable, smartphone app to improve the psychosocial health of young people living with CF
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Early respiratory viral infections in infants with cystic fibrosisEarly viral infections were associated with greater neutrophilic inflammation and bacterial pathogens
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Early surveillance of infants and preschool children with cystic fibrosisSensitive and non-invasive surveillance tools are needed for the clinical management of infants and preschool children with cystic fibrosis (CF). The lung clearance index from the multiple breath washout and functional and morphological outcomes from magnetic resonance imaging provide promising alternatives to current gold standard techniques. Early detection and treatment of lung disease during this important period offers the opportunity to improve the quality of life for individuals with CF.
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Identifying pediatric lung disease: A comparison of forced oscillation technique outcomesThese findings suggest the utility of specific FOT outcomes is dependent on the respiratory disease being assessed
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Multi-modality monitoring of cystic fibrosis lung disease: the role of chest computed tomographyStratification of monitoring protocols based on the risk profile of the patient can help us in the future to better care for people with Cystic Fibrosis.
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Cyanide in bronchoalveolar lavage is not diagnostic for Pseudomonas aeruginosa in children with cystic fibrosisEarly detection of the cyanobacterium Pseudomonas aeruginosa in the lungs of young children with cystic fibrosis (CF) is considered the key to delaying...
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Inflammatory responses to individual microorganisms in the lungs of children with cystic fibrosisWe hypothesized that the inflammatory response in the lungs of children with cystic fibrosis (CF) would vary with the type of infecting organism, being...