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Research

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.

Research

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

Research

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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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...

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Scoping review of the literature about family-centred care with caregivers of children with cystic fibrosis

Need for research into family-centred caring as a way of caring for children and families where one or more of the children have cystic fibrosis

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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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Multi-modality monitoring of cystic fibrosis lung disease: the role of chest computed tomography

Stratification of monitoring protocols based on the risk profile of the patient can help us in the future to better care for people with Cystic Fibrosis.

Research

Spring-infusors: How a simple and small solution can create king-sized complexity

The aims of the study were to investigate family and hospital staff views about the use of spring-infusor devices for administration of intravenous antibiotic medications, to examine if the device is acceptable and feasible and to map a process for implementation.

Research

Automatic bronchus and artery analysis on chest computed tomography to evaluate the effect of inhaled hypertonic saline in children aged 3-6 years with cystic fibrosis in a randomized clinical trial

SHIP-CT showed that 48-week treatment with inhaled 7% hypertonic saline (HS) reduced airway abnormalities on chest CT using the manual PRAGMA-CF method relative to isotonic saline (IS) in children aged 3-6 years with cystic fibrosis (CF). An algorithm was developed and validated to automatically measure bronchus and artery (BA) dimensions of BA-pairs on chest CT. Aim of the study was to assess the effect of HS on bronchial wall thickening and bronchial widening using the BA-analysis.

Research

Time to get serious about the detection and monitoring of early lung disease in cystic fibrosis

Structural and functional defects within the lungs of children with cystic fibrosis (CF) are detectable soon after birth and progress throughout preschool years often without overt clinical signs or symptoms. By school age, most children have structural changes such as bronchiectasis or gas trapping/hypoperfusion and lung function abnormalities that persist into later life. Despite improved survival, gains in forced expiratory volume in one second (FEV1) achieved across successive birth cohorts during childhood have plateaued, and rates of FEV1 decline in adolescence and adulthood have not slowed. This suggests that interventions aimed at preventing lung disease should be targeted to mild disease and commence in early life.