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Research

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.

Research

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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Hypoglycemia does not change the threshold for arousal from sleep in adolescents with type 1 diabetes

In adolescents with type 1 diabetes, hypoglycemia does not impair arousal from slow-wave sleep induced by an external auditory stimulus.

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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

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Assessment of p.Phe508del-CFTR functional restoration in pediatric primary cystic fibrosis airway epithelial cells

This 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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Oxidative stress in early cystic fibrosis lung disease is exacerbated by airway glutathione deficiency

are oxidative stress or glutathione status associated with brochiectasis and whether glutathione deficiency is inherently linked to CF or from oxidative stress

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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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Progressive ventilation inhomogeneity in infants with cystic fibrosis after pulmonary infection

This study aimed to determine how pulmonary inflammation & infection impacts on ventilation distribution throughout early life in people with cystic fibrosis.

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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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Visualisation of Multiple Tight Junctional Complexes in Human Airway Epithelial Cells

Apically located tight junctions in airway epithelium perform a fundamental role in controlling macromolecule migration through paracellular spaces.