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Research

Side effects of medications used to treat childhood interstitial lung disease

Interstitial 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 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.

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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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European Respiratory Society guidelines for the diagnosis of primary ciliary dyskinesia

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

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Phage therapy to treat cystic fibrosis Burkholderia cepacia complex lung infections: perspectives and challengesge

Burkholderia 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. 

Research

Transcriptomic analysis of primary nasal epithelial cells reveals altered interferon signalling in preterm birth survivors at one year of age

Many survivors of preterm birth (<37 weeks gestation) have lifelong respiratory deficits, the drivers of which remain unknown. Influencers of pathophysiological outcomes are often detectable at the gene level and pinpointing these differences can help guide targeted research and interventions. This study provides the first transcriptomic analysis of primary nasal airway epithelial cells in survivors of preterm birth at approximately 1 year of age.

Research

Ca-EDTA restores the activity of ceftazidime-avibactam or aztreonam against carbapenemase-producing Klebsiella pneumoniae infections

Developing an effective therapy to overcome carbapenemase-positive Klebsiella pneumoniae (CPKp) is an important therapeutic challenge that must be addressed urgently. Here, we explored a Ca-EDTA combination with aztreonam or ceftazidime-avibactam in vitro and in vivo against diverse CPKp clinical isolates.

Research

Substrate-dependent metabolomic signatures of myeloperoxidase activity in airway epithelial cells: Implications for early cystic fibrosis lung disease

Myeloperoxidase 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.