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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.

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

Developing a smartphone application to support social connectedness and wellbeing in young people with cystic fibrosis

This study developed and tested a highly usable, and moderately acceptable, smartphone app to improve the psychosocial health of young people living with CF

Research

Early respiratory viral infections in infants with cystic fibrosis

Early viral infections were associated with greater neutrophilic inflammation and bacterial pathogens

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

Research

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

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

The effect of inhaled hypertonic saline on lung structure in children aged 3-6 years with cystic fibrosis (SHIP-CT): a multicentre, randomised, double-blind, controlled trial

In the Saline Hypertonic in Preschoolers (SHIP) study, inhaled 7% hypertonic saline improved the lung clearance index in children aged 3-6 years with cystic fibrosis, but it remained unclear whether improvement is also seen in structural lung disease. We aimed to assess the effect of inhaled hypertonic saline on chest CT imaging in children aged 3-6 years with cystic fibrosis.

News & Events

On a quest for kids with cystic fibrosis

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.