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Article

Computed tomography correlates with improvement with ivacaftor in cystic fibrosis patients with G551D mutation.

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

Sheikh Shahid I, Long Frederick R, McCoy Karen S, Johnson Terri, Ryan-Wenger Nancy A, Hayes Don

Abstract excerpt

BACKGROUND: Ivacaftor corrects the cystic fibrosis transmembrane conductance regulator (CFTR) gating defect associated with G551D mutation and is quickly becoming an important treatment in patients with cystic fibrosis (CF) due to this genetic mutation. METHODS: A single-center study was performed in CF patients receiving ivacaftor to evaluate the usefulness of high resolution computed tomography (HRCT) of the...

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Source

Identifiers

PMID: 25049054
DOI: 10.1016/j.jcf.2014.06.011

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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