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Rhinovirus Infection Is Associated With Airway Epithelial Cell Necrosis and Inflammation via Interleukin-1 in Young Children With Cystic FibrosisThis study found rhinovirus infection drives necrotic cell death in cystic fibrosis airway epithelial cells
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Predicting disease progression in cystic fibrosisComprehensive scores incorporating clinical, lung function, imaging and laboratory data will become essential in the future for predicting disease progression and for use in clinical trials
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Assessment of p.Phe508del-CFTR functional restoration in pediatric primary cystic fibrosis airway epithelial cellsThis study assessed an alternative approach, using a small scale halide assay that can be adapted for a personalized high throughput setting to analyze CFTR function of pAEC.
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Progressive ventilation inhomogeneity in infants with cystic fibrosis after pulmonary infectionThis study aimed to determine how pulmonary inflammation & infection impacts on ventilation distribution throughout early life in people with cystic fibrosis.
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PRAGMA-CF. A Quantitative Structural Lung Disease Computed Tomography Outcome in Young Children with Cystic FibrosisChest computed tomography (CT) is the gold standard for demonstrating cystic fibrosis (CF) airway disease. However, there are no standardized outcome...
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Biomarkers in paediatric Cystic Fibrosis lung diseaseBiomarkers in cystic fibrosis are used for the measurement of cystic fibrosis transmembrane regulator function in order to diagnose cystic fibrosis,...
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What did we learn from two decades of chest computed tomography in cystic fibrosis?Despite our current treatment, many cystic fibrosis (CF) patients still show progressive bronchiectasis and small airways disease.
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Interpretation of lung function in infants and young children with cystic fibrosisThis paper is a review of lung function tests best suited to use for early detection of lung disease in Cystic Fibrosis.
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Airway macrophages display decreased expression of receptors mediating and regulating scavenging in early cystic fibrosis lung diseaseCystic 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.
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Prevalence of tracheobronchomalacia is higher than previously reported in children with cystic fibrosisTracheobronchomalacia (TBM) is estimated to be present in 1 in 2100 children. Previous reports suggest the prevalence is higher in children with cystic fibrosis (CF). This has clinical implications with potential to influence airway clearance and lung health.