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Article

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

American journal of respiratory and critical care medicine - 1 Jun 2013

Davies Jane C, Wainwright Claire E, Canny Gerard J, Chilvers Mark A, Howenstine Michelle S, Munck Anne, Mainz Jochen G, Rodriguez Sally, Li Haihong, Yen Karl, Ordoñez Claudia L, Ahrens Richard

Abstract excerpt

RATIONALE: Ivacaftor (VX-770), a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator, has been shown to improve lung function, pulmonary exacerbation rate, respiratory symptoms, and weight gain compared with placebo in patients with cystic fibrosis aged 12 years or older with a G551D-CFTR mutation. OBJECTIVES: This randomized, double-blind, placebo-controlled trial evaluated ivacaftor in...

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Identifiers

PMID: 23590265
DOI: 10.1164/rccm.201301-0153OC
PMCID: PMC3734608

Genes

CFTR

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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