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        The effect of CFTR modulators on structural lung disease in cystic fibrosis

        Newly 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 dyskinesia

        Structural 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

        Changing Prevalence of Lower Airway Infections in Young Children with Cystic Fibrosis

        Aspergillus species and P. aeruginosa are commonly present in the lower airways from infancy ...

        Research

        Effect of posture on lung ventilation distribution and associations with structure in children with cystic fibrosis

        Background: We assessed the effect of posture on ventilation distribution and the impact on associations with structural lung disease. ...

        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

        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

        Use of a primary epithelial cell screening tool to investigate phage therapy in cystic fibrosis

        This study demonstrates the feasibility of utilizing pre-clinical in vitro culture models to screen therapeutic candidates ...

        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

        Clarithromycin therapy for patients with Cystic Fibrosis: A randomized controlled trial

        The clinically significant actions of oral azithromycin in modifying progressive cystic fibrosis (CF) lung disease have been well documented. ...

        Research

        Air Trapping on Chest CT Is Associated with Worse Ventilation Distribution in Infants with Cystic Fibrosis

        In school-aged children with cystic fibrosis (CF) structural lung damage assessed using chest CT is associated with abnormal ventilation distribution. ...

        Research

        Exciting new clinical trials in cystic fibrosis: Infants need not apply

        The recent announcement of the negative results of the TIGER- 2 phase 3 study of denufosol tetrasodium ...

        Research

        Macrophage PD-1 associates with neutrophilia and reduced bacterial killing in early cystic fibrosis airway disease

        Macrophages are the major resident immune cells in human airways coordinating responses to infection and injury. In cystic fibrosis, neutrophils are recruited to the airways shortly after birth, and actively exocytose damaging enzymes prior to chronic infection, suggesting a potential defect in macrophage immunomodulatory function. ...

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