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Article

Lung function decline is delayed but not decreased in patients with cystic fibrosis and the R117H gene mutation.

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

Wagener Jeffrey S, Millar Stefanie J, Mayer-Hamblett Nicole, Sawicki Gregory S, McKone Edward F, Goss Christopher H, Konstan Michael W, Morgan Wayne J, Pasta David J, Moss Richard B

Abstract excerpt

BACKGROUND: Patients with cystic fibrosis (CF) experience variable lung disease phenotypes. The R117H mutation is often associated with preserved lung function. Our objective was to compare the rate of lung function decline in patients with the R117H mutation and patients homozygous for the F508del mutation. METHODS: Rate of decline in percentage-of-predicted FEV1 (ppFEV1) was analyzed using the 2006-2010 US CF...

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Identifiers

PMID: 29100868
DOI: 10.1016/j.jcf.2017.10.003
PMCID: PMC6547371

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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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