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Article

Ivacaftor as salvage therapy in a patient with cystic fibrosis genotype F508del/R117H/IVS8-5T.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society - 1 Jul 2015

Carter S, Kelly S, Caples E, Grogan B, Doyle J, Gallagher C G, McKone E F

Abstract excerpt

Ivacaftor is a novel CFTR potentiator that increases CFTR activity and improves clinical outcomes in cystic fibrosis (CF) patients with at least one copy of CFTR-G551D. Clinical trials have shown an improvement in lung function, weight and CF pulmonary exacerbation in adults with CFTR-G551D leading to the approval of ivacaftor as a novel CF therapy [1]. In vitro studies of ivacaftor have also shown significant...

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Source

Identifiers

PMID: 25698453
DOI: 10.1016/j.jcf.2015.01.010

Genes

CFTR

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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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