Tobacco Smoke Exposure Limits the Therapeutic Benefit of Tezacaftor/Ivacaftor in Pediatric Patients with Cystic Fibrosis

OBJECTIVES: Tobacco smoke exposure reduces CFTR functional expression in vitro and contributes to acquired CFTR dysfunction. We investigated whether it also inhibits the clinical benefit of CFTR modulators, focusing on tezacaftor/ivacaftor, approved in February 2018 for individuals with CF age ≥12 y...

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Udgivet i:J Cyst Fibros
Main Authors: Baker, Elizabeth, Harris, William T., Rowe, Steven M., Rutland, Sarah B., Oates, Gabriela R.
Format: Artigo
Sprog:Inglês
Udgivet: 2020
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Article
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC8018981/
https://ncbi.nlm.nih.gov/pubmed/33023836
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.jcf.2020.09.011
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