(Q54595809)

1 June 2018

title

Lumacaftor/Ivacaftor in Patients Aged 6-11 Years with Cystic Fibrosis and Homozygous for F508del-CFTR. (English)

1 reference

1 June 2018

0 references

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2

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1 June 2018

author name string

Carlos E Milla

1

1 reference

1 June 2018

Gautham Marigowda

3

1 reference

1 June 2018

Fang Liu

4

1 reference

1 June 2018

David Waltz

5

1 reference

1 June 2018

Margaret Rosenfeld

6

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1 June 2018

VX13-809-011 Part B Investigator Group *

7

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1 June 2018

language of work or name

English

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publication date

1 April 2017

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1 June 2018

American Journal of Respiratory and Critical Care Medicine

published in

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1 June 2018

volume

195

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1 June 2018

issue

7

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1 June 2018

page(s)

912-920

1 reference

1 June 2018

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5440888

1 reference

Safety, pharmacokinetics, and pharmacodynamics of ivacaftor in patients aged 2-5 years with cystic fibrosis and a CFTR gating mutation (KIWI): an open-label, single-arm study.

4 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5440888

Cystic fibrosis in young children: A review of disease manifestation, progression, and response to early treatment

4 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5440888

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).

4 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5440888

Efficacy and safety of ivacaftor in patients with cystic fibrosis and a non-G551D gating mutation

4 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5440888

Lung clearance index: evidence for use in clinical trials in cystic fibrosis

4 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5440888

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

4 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5440888

Ivacaftor in subjects with cystic fibrosis who are homozygous for the F508del-CFTR mutation

4 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5440888

Ivacaftor potentiation of multiple CFTR channels with gating mutations

4 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5440888

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

4 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5440888

Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809.

4 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5440888

Year-to-year changes in lung function in individuals with cystic fibrosis.

4 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5440888

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

4 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5440888

Inert gas washout: theoretical background and clinical utility in respiratory disease

4 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5440888

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

4 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5440888

Lung clearance index in the assessment of airways disease

4 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5440888

Systematic review of health-related quality of life measures for children with respiratory conditions

4 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5440888

Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic review

4 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5440888

Detection of cystic fibrosis transmembrane conductance regulator activity in early-phase clinical trials

4 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5440888

Rescue of DeltaF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules

4 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5440888

Endocytic trafficking routes of wild type and DeltaF508 cystic fibrosis transmembrane conductance regulator

4 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5440888

CDC growth charts: United States

4 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5440888

The diagnosis of cystic fibrosis: a consensus statement. Cystic Fibrosis Foundation Consensus Panel

4 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5440888

Conformational maturation of CFTR but not its mutant counterpart (delta F508) occurs in the endoplasmic reticulum and requires ATP

4 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5440888

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

4 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5440888

Practicability of nitrogen multiple-breath washout measurements in a pediatric cystic fibrosis outpatient setting

13 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5440888

Lung Clearance Index at 4 Years Predicts Subsequent Lung Function in Children with Cystic Fibrosis

13 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5440888

Multiple-breath inert gas washout and spirometry versus structural lung disease in cystic fibrosis.

13 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5440888

Validation of a Disease-Specific Measure of Health-Related Quality of Life for Children with Cystic Fibrosis

13 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5440888

Pulmonary function between 6 and 18 years of age.

13 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5440888

Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR.

13 August 2018

1 reference

Crossref

https://api.crossref.org/works/10.1164%2FRCCM.201608-1754OC

Lumacaftor–Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR

21 January 2018

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/27805836

inferred from PubMed ID database lookup

12 December 2020

based on heuristic

The effect of dornase alfa on ventilation inhomogeneity in patients with cystic fibrosis

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/27805836

inferred from PubMed ID database lookup

12 December 2020

based on heuristic

Hypertonic saline improves the LCI in paediatric patients with CF with normal lung function

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/27805836

inferred from PubMed ID database lookup

12 December 2020

based on heuristic

Identifiers

DOI

10.1164/RCCM.201608-1754OC

1 reference

1 June 2018

PMC publication ID

5440888

1 reference

1 June 2018

PubMed publication ID

27805836

1 reference

1 June 2018