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Small macrophages are present in early childhood respiratory diseaseRecently, an established "small macrophage" phenotype has been observed in the sputum of patients with CF and COPD. However, little is known about the...
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Watershed approach to improve cystic fibrosis treatmentThe Kids researchers are pioneering an exciting new approach to clinical trials, which aims to fast-track the best treatments for people with rare and complex diseases.
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Early lung disease in infants and preschool children with cystic fibrosis: What have we learned and what should we do about it?This review summarizes what we have learned about early lung disease in children with CF and discusses the implications for clinical practice and research
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Matrix metalloproteinase activation by free neutrophil elastase contributes to bronchiectasis progression in early cystic fibrosisThe aim of this study was to assess if MMP activation positively correlates with neutrophil elastase activity, disease severity and bronchiectasis in young...
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Feasibility of parental collected nasal swabs for virus detection in young children with cystic fibrosisNo evidence to demonstrate relationships with symptoms and viruses, prolonged symptoms, prolonged shedding or patterns of virus infections in CF
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Risk factors for bronchiectasis in children with cystic fibrosisWe sought to determine risk factors for the onset of bronchiectasis, using data collected by the Australian Respiratory Early Surveillance Team for Cystic...
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Lentiviral vector gene therapy and CFTR modulators show comparable effectiveness in cystic fibrosis rat airway modelsMutation-agnostic treatments such as airway gene therapy have the potential to treat any individual with cystic fibrosis (CF), irrespective of their CF transmembrane conductance regulator (CFTR) gene variants. The aim of this study was to employ two CF rat models, Phe508del and CFTR knockout to assess the comparative effectiveness of CFTR modulators and lentiviral vector-mediated gene therapy.
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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.
COMBAT CF is one of two long-standing international trials which have resulted in new early intervention options helping to reduce progressive lung damage in kids living with CF.
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.