Search
Research
Side effects of medications used to treat childhood interstitial lung diseaseInterstitial lung disease in children comprises a range of different rare diseases
Research
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
Research
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.
Research
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.
Research
Oxidative stress in early cystic fibrosis lung disease is exacerbated by airway glutathione deficiencyare oxidative stress or glutathione status associated with brochiectasis and whether glutathione deficiency is inherently linked to CF or from oxidative stress
Research
European Respiratory Society guidelines for the diagnosis of primary ciliary dyskinesiaGuideline to provide evidence-based recommendations on diagnostic testing for primary ciliary dyskinesia
Research
Pseudomonas aeruginosa modulates neutrophil granule exocytosis in an in vitro model of airway infectionA population of neutrophils recruited into cystic fibrosis (CF) airways is associated with proteolytic lung damage, exhibiting high expression of primary granule exocytosis marker CD63 and reduced phagocytic receptor CD16. Causative factors for this population are unknown, limiting intervention. Here we present a laboratory model to characterize responses of differentiated airway epithelium and neutrophils following respiratory infection.
Research
Substrate-dependent metabolomic signatures of myeloperoxidase activity in airway epithelial cells: Implications for early cystic fibrosis lung diseaseMyeloperoxidase is released by neutrophils in inflamed tissues. MPO oxidizes chloride, bromide, and thiocyanate to produce hypochlorous acid, hypobromous acid, and hypothiocyanous acid, respectively. These oxidants are toxic to pathogens, but may also react with host cells to elicit biological activity and potential toxicity. In cystic fibrosis and related diseases, increased neutrophil inflammation leads to increased airway MPO and airway epithelial cell exposure to its oxidants.
Research
Dysphagia in laryngomalacia: a prospective cohort studyDysphagia is an under recognised co-morbidity in patients with laryngomalacia. Its rate is variable reported in the literature. We aim to describe the incidence of dysphagia in laryngomalacia, the effect of interventions on this, and the period it persists in these infants.
Research
Phage therapy to treat cystic fibrosis Burkholderia cepacia complex lung infections: perspectives and challengesgeBurkholderia cepacia complex is a cause of serious lung infections in people with cystic fibrosis, exhibiting extremely high levels of antimicrobial resistance. These infections are difficult to treat and are associated with high morbidity and mortality.