Comparative sensitivity of early cystic fibrosis lung disease detection tools in school aged children.

Background: Effective detection of early lung disease in cystic fibrosis (CF) is critical to understanding early pathogenesis and evaluating early intervention strategies. We aimed to compare ability of several proposed sensitive functional tools to detect early CF lung disease as defined by CT structural disease in school aged children.

Methods: 50 CF subjects (mean±SD 11.

Implementation and evaluation of ultra-low dose CT in early cystic fibrosis lung disease.

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Curvilinearity provides additional information to lung clearance index only in a minority of children with early cystic fibrosis lung disease.

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Longitudinal assessment of lung clearance index to monitor disease progression in children and adults with cystic fibrosis.

Background: Lung clearance index (LCI) is a valuable research tool in cystic fibrosis (CF) but clinical application has been limited by technical challenges and uncertainty about how to interpret longitudinal change. In order to help inform clinical practice, this study aimed to assess feasibility, repeatability and longitudinal LCI change in children and adults with CF with predominantly mild baseline disease.

Methods: Prospective, 3-year, multicentre, observational study of repeated LCI measurement at time of clinical review in patients with CF >5 years, delivered using a rapid wash-in system.

Time to get serious about the detection and monitoring of early lung disease in cystic fibrosis.

Structural and functional defects within the lungs of children with cystic fibrosis (CF) are detectable soon after birth and progress throughout preschool years often without overt clinical signs or symptoms. By school age, most children have structural changes such as bronchiectasis or gas trapping/hypoperfusion and lung function abnormalities that persist into later life. Despite improved survival, gains in forced expiratory volume in one second (FEV) achieved across successive birth cohorts during childhood have plateaued, and rates of FEV decline in adolescence and adulthood have not slowed.

Nitrogen offset in N multiple washout method.

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Lung clearance index in healthy volunteers, measured using a novel portable system with a closed circuit wash-in.

Introduction: Lung clearance index (LCI) is a sensitive measure of early lung disease, but adoption into clinical practice has been slow. Challenges include the time taken to perform each test. We recently described a closed-circuit inert gas wash-in method that reduces overall testing time by decreasing the time to equilibration.

Simultaneous sulfur hexafluoride and nitrogen multiple-breath washout (MBW) to examine inherent differences in MBW outcomes.

Unlabelled: Multiple-breath washout (MBW) can be performed with different gases (sulfur hexafluoride (SF) and nitrogen (N)) and different devices, all of which give discrepant results. This study aimed to confirm previously reported differences and explore factors influencing discrepant results; equipment factors or the physical properties of gases used.

Methods: Healthy controls (HCs) and participants with cystic fibrosis (CF) completed MBW trials on two commercially available devices (Exhalyzer D (N) and Innocor (SF)).

Developments in multiple breath washout testing in children with cystic fibrosis.

Background: Lung clearance index (LCI) is becoming recognized as an important addition in the monitoring of pediatric cystic fibrosis (CF). The non-invasive technique is easy to perform in all ages, reproducible and increasingly being used in clinical trials. There is interest in utilizing it within the clinic setting but its current use is mostly as a research tool.

Repeated nebulisation of non-viral CFTR gene therapy in patients with cystic fibrosis: a randomised, double-blind, placebo-controlled, phase 2b trial.

Background: Lung delivery of plasmid DNA encoding the CFTR gene complexed with a cationic liposome is a potential treatment option for patients with cystic fibrosis. We aimed to assess the efficacy of non-viral CFTR gene therapy in patients with cystic fibrosis.

Methods: We did this randomised, double-blind, placebo-controlled, phase 2b trial in two cystic fibrosis centres with patients recruited from 18 sites in the UK.

Inspiratory loading intensity does not influence lactate clearance during recovery.

Purpose: This study examined the effects of different pressure threshold inspiratory loads on lactate clearance and plasma acid-base balance during recovery from maximal exercise.

Methods: Eight moderately trained males (V˙O(2peak) = 4.29 ± 0.