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Article

Effects of ivacaftor in patients with cystic fibrosis who carry the G551D mutation and have severe lung disease.

Chest - 1 Jul 2014

Barry Peter J, Plant Barry J, Nair Arjun, Bicknell Stephen, Simmonds Nicholas J, Bell Nicholas J, Shafi Nadia T, Daniels Thomas, Shelmerdine Susan, Felton Imogen, Gunaratnam Cedric, Jones Andrew M, Horsley Alex R

Abstract excerpt

BACKGROUND: The development of ivacaftor represents a significant advance in therapeutics for patients with cystic fibrosis (CF) who carry the G551D mutation. Patients with an FEV1 < 40% predicted represent a considerable proportion of eligible patients but were excluded from phase 3 clinical trials, and the effectiveness of the drug in this population is, therefore, unknown. METHODS: Data were collected from...

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Identifiers

PMID: 24522694
DOI: 10.1378/chest.13-2397

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 5 Sept 2026. Manifest publication-graph-cohort-2026-09-05.1.

Source receipt: pubmed, retrieved 5 Sept 2026.

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Effects of ivacaftor in patients with cystic fibrosis who carry the G551D mutation and have severe lung disease.PMID 24522694Read ↗

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