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.

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Published in Lancet Respir Med on September 10, 2013

Authors

Jane Davies1, Helen Sheridan2, Nicholas Bell3, Steve Cunningham4, Stephanie D Davis5, J Stuart Elborn6, Carlos E Milla7, Timothy D Starner8, Daniel J Weiner9, Po-Shun Lee10, Felix Ratjen11

Author Affiliations

1: Paediatric Respiratory Medicine, Royal Brompton Hospital and Dept of Gene Therapy, Imperial College London, England, UK. Electronic address: j.c.davies@imperial.ac.uk.
2: University of Edinburgh, Edinburgh, Scotland, UK.
3: Bristol Adult Cystic Fibrosis Centre, University Hospitals Bristol, Bristol, England, UK.
4: Royal Hospital for Sick Children, NHS, Lothian, Edinburgh, Scotland, UK.
5: Department of Pediatrics, University of North Carolina, Chapel Hill, NC, USA.
6: Centre for Infection and Immunity, Queen's University, Belfast, Northern Ireland, UK.
7: Center for Excellence in Pulmonary Biology, Stanford University, Palo Alto, CA, USA.
8: Department of Pediatrics, University of Iowa Carver College of Medicine, Iowa City, IA, USA.
9: The Antonio J and Janet Palumbo Cystic Fibrosis Center, University of Pittsburgh, Pittsburgh, PA, USA.
10: Vertex Pharmaceuticals Incorporated, Cambridge, MA, USA.
11: Division of Respiratory Medicine, Department of Paediatrics, Physiology and Experimental Medicine, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.

Associated clinical trials:

Study of the Effect of Ivacaftor on Lung Clearance Index in Subjects With Cystic Fibrosis and the G551D Mutation | NCT01262352

Hyperpolarized Xenon MRI in Cystic Fibrosis Pulmonary Exacerbations | NCT02606487

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