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BEAT CF pulmonary exacerbations core protocol for evaluating the management of pulmonary exacerbations in people with cystic fibrosisCystic fibrosis (CF) is a rare, inherited, life-limiting condition predominantly affecting the lungs, for which there is no cure. The disease is characterized by recurrent pulmonary exacerbations (PEx), which are thought to drive progressive lung damage. Management of these episodes is complex and generally involves multiple interventions targeting different aspects of disease. The emergence of innovative trials and use of Bayesian statistical methods has created renewed opportunities for studying heterogeneous populations in rare diseases.
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A structure-function analysis of interspecies antagonism by the 2-heptyl-4-alkyl-quinolone signal molecule from Pseudomonas aeruginosaHere we show that antibacterial activity of 4-hydroxy-2-heptylquinoline against Vibrionaceae is species-specific
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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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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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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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Disruption of β-catenin/CBP signaling inhibits human airway epithelial-mesenchymal transition and repair.The airway epithelium of both children and adults with asthma is relatively undifferentiated characterized by a significantly increased proportion of...
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Side effects of medications used to treat childhood interstitial lung diseaseInterstitial lung disease in children comprises a range of different rare diseases
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Effects of human rhinovirus on epithelial barrier integrity and function in children with asthmaThis study demonstrates novel intrinsic differences in tight junctions gene and protein expression between airway epithelial cells of children with and without asthma
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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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Visualisation of Multiple Tight Junctional Complexes in Human Airway Epithelial CellsApically located tight junctions in airway epithelium perform a fundamental role in controlling macromolecule migration through paracellular spaces.