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Research

Distribution of Early Structural Lung Changes due to Cystic Fibrosis Detected with Chest Computed Tomography

To examine the distribution of early structural lung changes in clinically stable infants and young children with cystic fibrosis using chest computed...

Research

Value of serology in predicting Pseudomonas aeruginosa infection in young children with cystic fibrosis

erology, predicting, Pseudomonas aeruginosa infection, young children, cystic fibrosis

Research

Evolution of pulmonary inflammation and nutritional status in infants and young children with cystic fibrosis

Improved nutrition is the major proven benefit of newborn screening programmes for cystic fibrosis (CF) and is associated with better clinical outcomes.

Research

SYNERGY CF: Getting the best start to life - preventing early cystic fibrosis lung disease by solving the host-inflammation infection conundrum

Cystic fibrosis related progressive lung disease characterised by inflammation and infection commences soon after birth.

News & Events

Promising results for new Western Australian-pioneered cystic fibrosis drug

Children with cystic fibrosis will be included in the next phase of a clinical trial of a promising new treatment pioneered in Western Australia aimed at boosting their immune responses to infections. Originally developed by researchers at The Kids Research Institute Australia and Perth Children’s Hospital.

News & Events

Telethon Institute awarded two national Centres of Research Excellence

Telethon Institute awarded two national Centres of Research Excellence

Research

Oxidative stress and abnormal bioactive lipids in early cystic fibrosis lung disease

Several lipid biomarkers of early cystic fibrosis lung disease were identified, which point toward potential disease monitoring and therapeutic approaches

Research

The longitudinal microbial and metabolic landscape of infant cystic fibrosis: the gut-lung axis

In cystic fibrosis, gastrointestinal dysfunction and lower airway infection occur early and are independently associated with poorer outcomes in childhood. This study aimed to define the relationship between the microbiota at each niche during the first 2 years of life, its association with growth and airway inflammation, and explanatory features in the metabolome. 

Research

Azithromycin reduces bronchial wall thickening in infants with cystic fibrosis

COMBAT-CF showed that children aged 0-3 years treated with azithromycin did clinically better than placebo but there was no effect on CT-scores. We reanalysed CTs using an automatic bronchus-artery (BA) analysis.

Research

Assessment of early lung disease in young children with CF: A comparison between pressure-controlled and free-breathing chest computed tomography

Our data suggest that FRC PC-CTs are less sensitive than TLC PC-CTs and that FB-CTs have similar sensitivity to PC-CTs in detecting lung disease