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Research

Multiple-breath washout as a lung function test in cystic fibrosis: A cystic fibrosis foundation workshop report

Recent technological advances in equipment design have produced gains in popularity for this test among cystic fibrosis (CF) researchers and clinicians...

Research

Early pulmonary inflammation and lung damage in children with cystic fibrosis

Airway inflammation and infection are present from early in life, often before children are symptomatic.

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Psychosocial characteristics and predictors of health-care use in families of young children with cystic fibrosis in Western Australia

This study aimed to identify psychosocial predictors in cystic fibrosis that may inform intervention strategies.

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CFTR-dependent defect in alternatively-activated macrophages in cystic fibrosis

CFTR-dependent imbalance of macrophage phenotypes and functions could contribute to the exaggerated inflammatory response seen in CF lung disease

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Bile signalling promotes chronic respiratory infections and antibiotic tolerance

Bile acid signalling is a leading trigger for the development of chronic phenotypes underlying the pathophysiology of chronic respiratory disease

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Dissecting the regulation of bile-induced biofilm formation in staphylococcus aureus

Wall teichoic acid may be important for protecting S. aureus against exposure to bile

News & Events

NHMRC funding awarded to support child health research

The Kids Research Institute Australia researchers have been awarded more than $10 million in research funding from the National Health and Medical Research Council (NHMRC).

Research

MetaNeb Versus Usual Care During Exacerbations of Cystic Fibrosis: An RCT

During exacerbations, when symptom and treatment burden are increased, individuals with cystic fibrosis (CF) are likely to prefer airway clearance techniques (ACTs) that require minimal effort. Therefore, in adults with CF who were hospitalised with an exacerbation, we sought to compare the effect of the MetaNeb with usual ACTs on respiratory function and expectorated sputum.

Research

BEAT-CF (Bayesian Evidence Adaptive Treatment for people with Cystic Fibrosis): description of a prospective cohort for nested studies in cystic fibrosis

Despite recent improvements in treatment modalities for cystic fibrosis (CF), there is currently limited evidence and a lack of consensus regarding optimal treatment strategies for the different aspects of CF, including pulmonary exacerbations (PEx). We aimed to establish a prospective cohort of people with CF (pwCF) to evaluate alternative approaches to managing CF in the era of modulator therapies.

Research

Lung structural and functional impairments in young children with cystic fibrosis diagnosed following newborn screening – A nationwide observational study

Non-invasive and sensitive clinical endpoints are needed to monitor onset and progression of early lung disease in children with cystic fibrosis (CF). We compared lung clearance index (LCI), FEV1, functional and structural lung magnetic resonance imaging (MRI) outcomes in Swiss children with CF diagnosed following newborn screening.