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Research

Standardised treatment and monitoring protocol to assess safety and tolerability of bacteriophage therapy for adult and paediatric patients (STAMP study): protocol for an open-label, single-arm trial

There has been renewed interest in the therapeutic use of bacteriophages (phages); however, standardised therapeutic protocols are lacking, and there is a paucity of rigorous clinical trial data assessing efficacy.

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The effect of azithromycin on structural lung disease in infants with cystic fibrosis (COMBAT CF): a phase 3, randomised, double-blind, placebo-controlled clinical trial

Structural lung disease and neutrophil-dominated airway inflammation is present from 3 months of age in children diagnosed with cystic fibrosis after newborn screening. We hypothesised that azithromycin, given three times weekly to infants with cystic fibrosis from diagnosis until age 36 months, would reduce the extent of structural lung disease as captured on chest CT scans.

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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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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...

Research

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...

Research

European Respiratory Society guidelines for the diagnosis of primary ciliary dyskinesia

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

Research

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.

Advancing Innovation in Respiratory Health

The Advancing Innovation in Respiratory (AIR) Health Team is a multi-disciplinary group with skills in clinical medicine, physiology, psychology, and in cellular and molecular biology, that are committed to improving the lives of children with respiratory diseases and their families.

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

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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,...