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Article

Use of hyperpolarized helium-3 MRI to assess response to ivacaftor treatment in patients with cystic fibrosis.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society - 1 Mar 2017

Altes Talissa A, Johnson Mac, Fidler Meredith, Botfield Martyn, Tustison Nicholas J, Leiva-Salinas Carlos, de Lange Eduard E, Froh Deborah, Mugler John P

Abstract excerpt

BACKGROUND: This pilot study evaluated the effect of short- and long-term ivacaftor treatment on hyperpolarized 3He-magnetic resonance imaging (MRI)-defined ventilation defects in patients with cystic fibrosis aged ≥12years with a G551D-CFTR mutation. METHODS: Part A (single-blind) comprised 4weeks of ivacaftor treatment; Part B (open-label) comprised 48weeks of treatment. The primary outcome was change from...

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Identifiers

PMID: 28132845
DOI: 10.1016/j.jcf.2016.12.004

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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