Article
The effects of ivacaftor on CF fatty acid metabolism: An analysis from the GOAL study.
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society - 1 Jan 2017
O'Connor Michael Glenn, Seegmiller Adam
Abstract excerpt
BACKGROUND: Ivacaftor has produced significant improvement in certain individuals with cystic fibrosis (CF), though the full metabolic effects of treatment remain unknown. Abnormalities in fatty acid metabolism have previously been shown to be a characteristic of CFTR dysfunction. We hypothesized that as a reflection of this clinical improvement, ivacaftor would improve plasma fatty acid levels and decrease urine...
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