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Article

Immediate effects of lumacaftor/ivacaftor administration on lung function in patients with severe cystic fibrosis lung disease.

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

Popowicz Natalia, Wood Jamie, Tai Anna, Morey Sue, Mulrennan Siobhain

Abstract excerpt

Safety-data for lumacaftor/ivacaftor (LUM/IVA) combination therapy in patients with severe lung disease (percent predicted forced expiratory volume in 1s [ppFEV1] <40) remain limited. We report immediate post-dose respiratory-related adverse events in 12 patients with severe cystic fibrosis (CF) lung disease (median [IQR] ppFEV1: 34 [31-36]) prescribed LUM/IVA. All patients experienced a decline in ppFEV1 from...

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Identifiers

PMID: 28314539
DOI: 10.1016/j.jcf.2017.02.009

Genes

LUM

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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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