Search
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
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
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
Research
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
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
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
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
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.
Research
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.
Research
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