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Article

Long-term effects of ivacaftor on nonpulmonary outcomes in individuals with cystic fibrosis, heterozygous for a S1251N mutation.

Pediatric pulmonology - 1 Jun 2020

Burghard M Marcella, Berkers G Gitte, Ghijsen S Sophie, Hollander-Kraaijeveld Fm Francis, de Winter-de Groot Km Karin, van der Ent Ck Kors, Heijerman Hgm Harry, Takken T Tim, Hulzebos Hj Erik

Abstract excerpt

OBJECTIVES: To describe the long-term effects of ivacaftor (Kalydeco®) in individuals with cystic fibrosis (CF) on body mass index (BMI), body composition (BC), pulmonary function (PF), resting energy expenditure (REE), and exercise capacity (EC) after ≥12 months of treatment. WORKING HYPOTHESIS: BMI, lean and fat mass, PF, and EC will increase and REE will decrease after treatment. STUDY DESIGN: Observational...

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Identifiers

PMID: 32233113
DOI: 10.1002/ppul.24745
PMCID: PMC7317816

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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