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Research

Randomised controlled trials in cystic fibrosis: What, when and how?

The major morbidity and mortality from cystic fibrosis (CF) comes from progressive lung disease with bronchiectasis leading to respiratory failure

Research

Effects of human rhinovirus on epithelial barrier integrity and function in children with asthma

This 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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Side effects of medications used to treat childhood interstitial lung disease

Interstitial lung disease in children comprises a range of different rare diseases

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A structure-function analysis of interspecies antagonism by the 2-heptyl-4-alkyl-quinolone signal molecule from Pseudomonas aeruginosa

Here we show that antibacterial activity of 4-hydroxy-2-heptylquinoline against Vibrionaceae is species-specific

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Predicting disease progression in cystic fibrosis

Comprehensive 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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Chest computed tomography: a validated surrogate endpoint of cystic fibrosis lung disease?

Clinical trials for the treatment of cystic fibrosis (CF) lung disease are important to test and optimise new therapeutic interventions.

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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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PRAGMA-CF. A Quantitative Structural Lung Disease Computed Tomography Outcome in Young Children with Cystic Fibrosis

Chest 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 disease

Biomarkers in cystic fibrosis are used for the measurement of cystic fibrosis transmembrane regulator function in order to diagnose cystic fibrosis,...

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European Respiratory Society guidelines for the diagnosis of primary ciliary dyskinesia

Guideline to provide evidence-based recommendations on diagnostic testing for primary ciliary dyskinesia