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Research
Azithromycin reduces bronchial wall thickening in infants with cystic fibrosisCOMBAT-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.
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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.
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Clarithromycin therapy for patients with Cystic Fibrosis: A randomized controlled trialThe clinically significant actions of oral azithromycin in modifying progressive cystic fibrosis (CF) lung disease have been well documented.
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Air Trapping on Chest CT Is Associated with Worse Ventilation Distribution in Infants with Cystic FibrosisIn school-aged children with cystic fibrosis (CF) structural lung damage assessed using chest CT is associated with abnormal ventilation distribution.
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Exciting new clinical trials in cystic fibrosis: Infants need not applyThe recent announcement of the negative results of the TIGER- 2 phase 3 study of denufosol tetrasodium
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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).
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Feasibility of unsedated lung MRI in young children with cystic fibrosisKathryn Ramsey KR BSc (Hons), PhD Co-Head, Children's Lung Health kathryn.ramsey@telethonkids.org.au Co-Head, Children's Lung Health Associate
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Association between early respiratory viral infections and structural lung disease in infants with cystic fibrosisInfants with cystic fibrosis (CF) develop structural lung disease early in life, and viral infections are associated with progressive lung disease. We hypothesized that the presence of respiratory viruses would be associated with structural lung disease on computed tomography (CT) of the chest in infants with CF.
Research
A screening tool to identify risk for bronchiectasis progression in children with cystic fibrosisThe marked heterogeneity in cystic fibrosis (CF) disease complicates the selection of those most likely to benefit from existing or emergent treatments. We aimed to predict the progression of bronchiectasis in preschool children with CF.