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Tracheobronchomalacia (TBM) is characterised by abnormal collapsibility of the trachea and bronchi, often seen in children with cystic fibrosis (CF). This study aims to determine the impact of TBM on hospital admissions in young children with CF.
Pulmonary exacerbations pose a significant clinical burden on people with cystic fibrosis (pwCF). Whether management of exacerbations should change in the context of modulator therapy is unclear. We describe the characteristics, treatment and lung function outcomes of pulmonary exacerbations requiring intravenous antibiotic therapy (PERITs) in a contemporary Australian cohort of pwCF, in an era of rapidly broadening access to modulator therapy.
Respiratory tract infections (RTIs) drive lung function decline in children with cystic fibrosis (CF). While the respiratory microbiota is clearly associated with RTI pathogenesis in infants without CF, data on infants with CF is scarce. We compared nasal microbiota development between infants with CF and controls and assessed associations between early-life nasal microbiota, RTIs, and antibiotic treatment in infants with CF.
Whether airway mucosal acidification drives early progressive lung disease is controversial
The lung clearance index may be a useful surveillance tool for monitoring the presence and extent of lower airway inflammation and infection
This review summarizes what we have learned about early lung disease in children with CF and discusses the implications for clinical practice and research
PRAGMA-CF, a new way of measuring early lung disease in young kids with cystic fibrosis is changing the way we detect and treat CF.
Dysregulated repair following epithelial injury is a key forerunner of disease in many organs, and the acquisition of a mesenchymal phenotype by the injured...
A The Kids Research Institute Australia spin-off company has received $20 million from the Medical Research Commercialisation Fund to develop a promising new therapy for the treatment of Cystic Fibrosis.
This protocol outlines the study aims to investigate the causal effect of psychosocial functioning, parenting and attachment on physical health outcomes in...