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Early disease surveillance in young children with cystic fibrosis: A qualitative analysis of parent experiencesSensitive measures of early lung disease are being integrated into therapeutic trials and clinical practice in cystic fibrosis (CF). The impact of early disease surveillance (EDS) using these novel and often intensive techniques on young children and their families is not well researched.
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Airway epithelial repair in health and disease: Orchestrator or simply a player?This review attempts to highlight migration-specific and cell-extracellular matrix (ECM) aspects of repair used by epithelial cells
Research
Early lung surveillance of cystic fibrosis: what have we learnt?Newborn screening (NBS) for cystic fibrosis (CF) provides an opportunity to commence management and therapeutic interventions significantly earlier
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Evolution of pulmonary inflammation and nutritional status in infants and young children with cystic fibrosisImproved nutrition is the major proven benefit of newborn screening programmes for cystic fibrosis (CF) and is associated with better clinical outcomes.
Research
Value of serology in predicting Pseudomonas aeruginosa infection in young children with cystic fibrosiserology, predicting, Pseudomonas aeruginosa infection, young children, cystic fibrosis
News & Events
New drug therapy provides hope for kids with cystic fibrosisThe Kids Research Institute Australia spin-off company, Respirion, received $20 million in funding to develop a promising new therapy.
News & Events
New drug therapy provides hope for kids with cystic fibrosisThe family of two girls with cystic fibrosis are hopeful after The Kids Research Institute Australia spin-off company, Respirion, receives $20 million in funding to develop a promising new therapy.
Research
The SPEC score—A quantifiable CT scoring system for primary ciliary dyskinesiaStructural lung changes seen on computed tomography scans in persons with primary ciliary dyskinesia are currently described using cystic fibrosis derived scoring systems. Recent work has shown structural changes and frequencies that are unique to PCD, indicating the need for a unique PCD-derived scoring system.
Research
The effect of CFTR modulators on structural lung disease in cystic fibrosisNewly developed quantitative chest computed tomography (CT) outcomes designed specifically to assess structural abnormalities related to cystic fibrosis (CF) lung disease are now available. CFTR modulators potentially can reduce some structural lung abnormalities. We aimed to investigate the effect of CFTR modulators on structural lung disease progression using different quantitative CT analysis methods specific for people with CF (PwCF).
Research
Effect of posture on lung ventilation distribution and associations with structure in children with cystic fibrosisBackground: We assessed the effect of posture on ventilation distribution and the impact on associations with structural lung disease.