Assessment of clinical response to ivacaftor with lung clearance index in cystic fibrosis patients with a G551D- CFTR mutation and preserved spirometry: a randomised controlled trial
- 10 September 2013
- journal article
- research article
- Published by Elsevier BV in The Lancet Respiratory Medicine
- Vol. 1 (8), 630-638
- https://doi.org/10.1016/s2213-2600(13)70182-6
Abstract
No abstract availableKeywords
This publication has 24 references indexed in Scilit:
- The Sensitivity of Lung Disease Surrogates in Detecting Chest CT Abnormalities in Children With Cystic FibrosisPediatric Pulmonology, 2011
- A CFTR Potentiator in Patients with Cystic Fibrosis and theG551DMutationThe New England Journal of Medicine, 2011
- Effect of VX-770 in Persons with Cystic Fibrosis and the G551D-CFTRMutationThe New England Journal of Medicine, 2010
- Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770Proceedings of the National Academy of Sciences of the United States of America, 2009
- Cystic fibrosisThe Lancet, 2009
- Power Considerations for Studies of Lung Function in Cystic FibrosisProceedings of the American Thoracic Society, 2007
- Cystic FibrosisThe New England Journal of Medicine, 2005
- Quantitative air-trapping analysis in children with mild cystic fibrosis lung diseasePediatric Pulmonology, 2004
- High-resolution computed tomography in young patients with cystic fibrosis: Distribution of abnormalities and correlation with pulmonary function testsThe Journal of Pediatrics, 2004
- Molecular basis of defective anion transport in L cells expressing recombinant forms of CFTRHuman Molecular Genetics, 1993