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Article

Ivacaftor withdrawal syndrome in cystic fibrosis patients with the G551D mutation.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society - 1 Mar 2018

Trimble Aaron T, Donaldson Scott H

Abstract excerpt

Ivacaftor use can lead to dramatic health improvements in cystic fibrosis (CF) patients with gating mutations. Here, we report five instances of dramatic clinical decline following withdrawal of ivacaftor in three individuals with the G551D-CFTR mutation. In each case, the patient's lung function and symptoms rapidly deteriorated after cessation of treatment. Awareness of this phenomenon should inform both...

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Source

Identifiers

PMID: 29079142
DOI: 10.1016/j.jcf.2017.09.006

Genes

CFTR

Provenance

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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