(Q30978102)

27 June 2017

title

Sustained Benefit from ivacaftor demonstrated by combining clinical trial and cystic fibrosis patient registry data (English)

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27 June 2017

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Gregory S Sawicki

1

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27 June 2017

Edward F McKone

2

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27 June 2017

David J Pasta

3

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27 June 2017

Stefanie J Millar

4

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27 June 2017

Jeffrey S Wagener

5

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27 June 2017

Charles A Johnson

6

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27 June 2017

Michael W Konstan

7

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27 June 2017

language of work or name

English

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publication date

1 July 2015

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American Journal of Respiratory and Critical Care Medicine

27 June 2017

published in

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27 June 2017

volume

192

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27 June 2017

issue

7

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27 June 2017

page(s)

836-842

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Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA

27 June 2017

cites work

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https://api.crossref.org/works/10.1164%2FRCCM.201503-0578OC

Modifying disease in cystic fibrosis: current and future therapies on the horizon

21 January 2018

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https://api.crossref.org/works/10.1164%2FRCCM.201503-0578OC

Longitudinal analysis of pulmonary function decline in patients with cystic fibrosis

21 January 2018

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https://api.crossref.org/works/10.1164%2FRCCM.201503-0578OC

Jointly modelling the relationship between survival and pulmonary function in cystic fibrosis patients.

21 January 2018

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https://api.crossref.org/works/10.1164%2FRCCM.201503-0578OC

Design and powering of cystic fibrosis clinical trials using rate of FEV(1) decline as an efficacy endpoint

21 January 2018

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https://api.crossref.org/works/10.1164%2FRCCM.201503-0578OC

Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770

21 January 2018

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https://api.crossref.org/works/10.1164%2FRCCM.201503-0578OC

A CFTR potentiator in patients with cystic fibrosis and the G551D mutation

21 January 2018

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https://api.crossref.org/works/10.1164%2FRCCM.201503-0578OC

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201503-0578OC

Long-term safety and efficacy of ivacaftor in patients with cystic fibrosis who have the Gly551Asp-CFTR mutation: a phase 3, open-label extension study (PERSIST).

21 January 2018

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https://api.crossref.org/works/10.1164%2FRCCM.201503-0578OC

Propensity score methods.

21 January 2018

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https://api.crossref.org/works/10.1164%2FRCCM.201503-0578OC

Accelerating implementation of biomedical research advances: critical elements of a successful 10 year Cystic Fibrosis Foundation healthcare delivery improvement initiative

21 January 2018

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https://api.crossref.org/works/10.1164%2FRCCM.201503-0578OC

Effect of genotype on phenotype and mortality in cystic fibrosis: a retrospective cohort study

21 January 2018

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https://api.crossref.org/works/10.1164%2FRCCM.201503-0578OC

Risk factors for rate of decline in forced expiratory volume in one second in children and adolescents with cystic fibrosis.

21 January 2018

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https://api.crossref.org/works/10.1164%2FRCCM.201503-0578OC

Risk factors for rate of decline in FEV1 in adults with cystic fibrosis.

21 January 2018

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Relationship between nutritional status and lung function in cystic fibrosis: cross sectional and longitudinal analyses from the German CF quality assurance (CFQA) project

21 January 2018

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Outcome of Burkholderia (Pseudomonas) cepacia colonisation in children with cystic fibrosis following a hospital outbreak

21 January 2018

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https://api.crossref.org/works/10.1164%2FRCCM.201503-0578OC

Multi-ethnic reference values for spirometry for the 3-95-yr age range: the global lung function 2012 equations

21 January 2018

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https://api.crossref.org/works/10.1164%2FRCCM.201503-0578OC

A critical appraisal of propensity-score matching in the medical literature between 1996 and 2003.

21 January 2018

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Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis. The Pulmozyme Study Group

21 January 2018

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https://api.crossref.org/works/10.1164%2FRCCM.201503-0578OC

A two-year randomized, placebo-controlled trial of dornase alfa in young patients with cystic fibrosis with mild lung function abnormalities

21 January 2018

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https://api.crossref.org/works/10.1164%2FRCCM.201503-0578OC

Clinical use of dornase alpha is associated with a slower rate of FEV1 decline in cystic fibrosis.

21 January 2018

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https://api.crossref.org/works/10.1164%2FRCCM.201503-0578OC

Clinical trial participants compared with nonparticipants in cystic fibrosis.

21 January 2018

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https://api.crossref.org/works/10.1164%2FRCCM.201503-0578OC

Children and young adults with CF in the USA have better lung function compared with the UK.

21 January 2018

1 reference

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10.1164/RCCM.201503-0578OC

21 January 2018

Identifiers

DOI

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27 June 2017

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