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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.
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Changing Prevalence of Lower Airway Infections in Young Children with Cystic FibrosisAspergillus species and P. aeruginosa are commonly present in the lower airways from infancy
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Use of a primary epithelial cell screening tool to investigate phage therapy in cystic fibrosisThis study demonstrates the feasibility of utilizing pre-clinical in vitro culture models to screen therapeutic candidates
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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.
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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"
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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.
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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.
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Early nasal microbiota and subsequent respiratory tract infections in infants with cystic fibrosisRespiratory tract infections (RTIs) drive lung function decline in children with cystic fibrosis (CF). While the respiratory microbiota is clearly associated with RTI pathogenesis in infants without CF, data on infants with CF is scarce. We compared nasal microbiota development between infants with CF and controls and assessed associations between early-life nasal microbiota, RTIs, and antibiotic treatment in infants with CF.
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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.
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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).