Search
Research
Minimal structural lung disease in early life represents significant pathologyIn this cohort, as compared with the AREST CF cohort, the authors highlight the limited correlation between infection and inflammation with lung function and structural impairment, and that this was mainly explained by the mild changes identified in lung function and on chest CT scan.
Research
The measurement properties of tests and tools used in cystic fibrosis studies: a systematic reviewThere is no consensus on how best to measure responses to interventions among children and adults with cystic fibrosis (CF). We have systematically reviewed and summarised the characteristics and measurement properties of tests and tools that have been used to capture outcomes in studies among people with CF, including their reliability, validity and responsiveness. This review is intended to guide researchers when selecting tests or tools for measuring treatment effects in CF trials. A consensus set of these tests and tools could improve consistency in how outcomes are captured and thereby facilitate comparisons and synthesis of evidence across studies.
Research
Novel method to select meaningful outcomes for evaluation in clinical trialsA standardised framework for selecting outcomes for evaluation in trials has been proposed by the Core Outcome Measures in Effectiveness Trials working group. However, this method does not specify how to ensure that the outcomes that are selected are causally related to the disease and the health intervention being studied. Causal network diagrams may help researchers identify outcomes that are both clinically meaningful and likely to be causally dependent on the intervention, and endpoints that are, in turn, causally dependent on those outcomes.
Research
Chest imaging in cystic fibrosis studies: What counts, and can be counted?The aim of this study is to characterize the role of currently available CT and MRI markers in clinical studies, and to discuss challenges with CF studies.
Research
The AREST CF experience in biobanking - More than just tissues, tubes and time.Research to further improve outcomes for people with CF is dependent upon well characterised, archived and accessible clinical specimens.
Research
Air trapping in early cystic fibrosis lung disease-Does CT tell the full story?Mosaic attenuation on expiratory chest computed tomography (CT) is common in early life cystic fibrosis (CF) and often referred to as "air trapping"
Research
Effect of posture on lung ventilation distribution and associations with structure in children with cystic fibrosisBackground: We assessed the effect of posture on ventilation distribution and the impact on associations with structural lung disease.
Research
Feasibility of unsedated lung MRI in young children with cystic fibrosisKathryn Ramsey BSc (Hons), PhD Co-Head, Children's Lung Health kathryn.ramsey@thekids.org.au Co-Head, Children's Lung Health Associate Professor
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).
Research
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.