Mucus plugs have been previously recognized as an important pathological feature in asthma and chronic obstructive pulmonary disease (COPD), but thei…
Cystic fibrosis (CF) is a genetic condition caused by mutation of the cystic fibrosis transmembrane regulator (CFTR) gene. Recently licensed modulato…
Elexacaftor/tezacaftor/ivacaftor(ELX/TEZ/IVA) was safe and efficacious in children with CF 2-5y with at least one F508del allele in 24-week phase 3 T…
For decades, cystic fibrosis (CF) meant a life shaped by daily treatments, frequent hospitalisations, and an uncertain future. The arrival of CFTR mo…
Functional studies of how early-life interventions shape the airway microbiome remain scarce. Here, we performed metagenomic sequencing of 704 longit…
Pulmonary exacerbations pose a significant clinical burden on people with cystic fibrosis (pwCF). Whether management of exacerbations should change i…
Tracheobronchomalacia (TBM) is characterised by abnormal collapsibility of the trachea and bronchi, often seen in children with cystic fibrosis (CF).…
Positive expiratory pressure devices are frequently used for airway clearance in children with cystic fibrosis and tracheobronchomalacia. This study…
Children with cystic fibrosis will be included in the next phase of a clinical trial of a promising new treatment pioneered in Western Australia aime…
During exacerbations, when symptom and treatment burden are increased, individuals with cystic fibrosis (CF) are likely to prefer airway clearance te…