(Q34480471)

2 August 2017

Clinical mechanism of the cystic fibrosis transmembrane conductance regulator potentiator ivacaftor in G551D-mediated cystic fibrosis (English)

1 reference

2 August 2017

0 references

transmembrane protein

0 references

author name string

Steven M Rowe

1

1 reference

2 August 2017

Sonya L Heltshe

2

1 reference

2 August 2017

Tanja Gonska

3

1 reference

2 August 2017

Scott H Donaldson

4

1 reference

2 August 2017

Drucy Borowitz

5

1 reference

2 August 2017

Daniel Gelfond

6

1 reference

2 August 2017

Scott D Sagel

7

1 reference

2 August 2017

Umer Khan

8

1 reference

2 August 2017

Nicole Mayer-Hamblett

9

1 reference

2 August 2017

Jill M Van Dalfsen

10

1 reference

2 August 2017

Elizabeth Joseloff

11

1 reference

2 August 2017

Bonnie W Ramsey

12

1 reference

2 August 2017

GOAL Investigators of the Cystic Fibrosis Foundation Therapeutics Development Network

13

1 reference

2 August 2017

language of work or name

English

0 references

publication date

1 July 2014

1 reference

American Journal of Respiratory and Critical Care Medicine

2 August 2017

1 reference

2 August 2017

190

1 reference

2 August 2017

175-184

1 reference

2 August 2017

2

1 reference

Sixty-five years since the New York heat wave: advances in sweat testing for cystic fibrosis.

2 August 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4226057

Defining the disease liability of variants in the cystic fibrosis transmembrane conductance regulator gene

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4226057

The cystic fibrosis intestine

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4226057

Inflammation and airway microbiota during cystic fibrosis pulmonary exacerbations

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4226057

Efficacy and safety of ivacaftor in patients aged 6 to 11 years with cystic fibrosis with a G551D mutation

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4226057

Change in sweat chloride as a clinical end point in cystic fibrosis clinical trials: the ivacaftor experience

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4226057

Gastrointestinal complications of cystic fibrosis

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4226057

Progress in cystic fibrosis and the CF Therapeutics Development Network

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4226057

Sputum biomarkers of inflammation and lung function decline in children with cystic fibrosis

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4226057

Regulatory aspects of Phase 3 endpoints for new inhaled antibiotics for cystic fibrosis patients with chronic Pseudomonas aeruginosa infections

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4226057

Bicarbonate and functional CFTR channel are required for proper mucin secretion and link cystic fibrosis with its mucus phenotype.

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4226057

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

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4226057

Initial Pseudomonas aeruginosa treatment failure is associated with exacerbations in cystic fibrosis

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4226057

Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4226057

Role of epithelial HCO3⁻ transport in mucin secretion: lessons from cystic fibrosis

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4226057

Airway microbiota and pathogen abundance in age-stratified cystic fibrosis patients

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4226057

Patient-reported respiratory symptoms in cystic fibrosis

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4226057

Determination of the minimal clinically important difference scores for the Cystic Fibrosis Questionnaire-Revised respiratory symptom scale in two populations of patients with cystic fibrosis and chronic Pseudomonas aeruginosa airway infection

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4226057

Mucociliary clearance as an outcome measure for cystic fibrosis clinical research

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4226057

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4226057

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4226057

Spirometric reference values from a sample of the general U.S. population

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4226057

Standardization of Spirometry, 1994 Update. American Thoracic Society

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4226057

Intractable cough in a preterm infant with ventriculoperitoneal shunt

6 July 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201404-0703OC

Development and validation of The Cystic Fibrosis Questionnaire in the United States: a health-related quality-of-life measure for cystic fibrosis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201404-0703OC

Pulmonary function between 6 and 18 years of age.

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201404-0703OC

Multisite comparison of mucociliary and cough clearance measures using standardized methods.

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201404-0703OC

Inflammatory and microbiologic markers in induced sputum after intravenous antibiotics in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201404-0703OC

β-adrenergic sweat secretion as a diagnostic test for cystic fibrosis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201404-0703OC

Longitudinal development of mucoid Pseudomonas aeruginosa infection and lung disease progression in children with cystic fibrosis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201404-0703OC

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201404-0703OC

Pseudomonas aeruginosa and other predictors of mortality and morbidity in young children with cystic fibrosis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201404-0703OC

Mucus clearance and lung function in cystic fibrosis with hypertonic saline.

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.201404-0703OC

Personalized medicine in cystic fibrosis: dawning of a new era

21 January 2018

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/24927234

inferred from PubMed ID database lookup

12 December 2020

based on heuristic

Intestinal pH and gastrointestinal transit profiles in cystic fibrosis patients measured by wireless motility capsule

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/24927234

inferred from PubMed ID database lookup

12 December 2020

based on heuristic

Psychometric and clinimetric validity of the 20-Item Sino-Nasal Outcome Test (SNOT-20).

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/24927234

inferred from PubMed ID database lookup

12 December 2020

based on heuristic

Identifiers

DOI

10.1164/RCCM.201404-0703OC

1 reference

2 August 2017

1 reference

2 August 2017

1 reference