Clinical Pharmacogenetics Implementation Consortium (CPIC) guidelines for ivacaftor therapy in the context of CFTR genotype.

Clancy JP1, Johnson SG2, Yee SW3, McDonagh EM4, Caudle KE5, Klein TE4, Cannavo M5, Giacomini KM3.

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Abstract

Cystic fibrosis (CF) is a life-shortening disease arising as a consequence of mutations within the CFTR gene. Novel therapeutics for CF are emerging that target CF transmembrane conductance regulator protein (CFTR) defects resulting from specific CFTR variants. Ivacaftor is a drug that potentiates CFTR gating function and is specifically indicated for CF patients with a particular CFTR variant, G551D-CFTR (rs75527207). Here, we provide therapeutic recommendations for ivacaftor based on preemptive CFTR genotype results.

PMID:: 24598717; [PubMed - indexed for MEDLINE]
PMCID:: PMC4026598

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National Guideline Clearinghouse | Clinical Pharmacogenetics Implementation Consortium (CPIC) guidelines for ivacaftor therapy in the context of <em>CFTR</em> genotype.

Clinical Pharmacogenetics Implementation Consortium

Guideline Title

Clinical Pharmacogenetics Implementation Consortium (CPIC) guidelines for ivacaftor therapy in the context of CFTRgenotype.

Bibliographic Source(s)

Clancy JP, Johnson SG, Yee SW, McDonagh EM, Caudle KE, Klein TE, Cannavo M, Giacomini KM. Clinical Pharmacogenetics Implementation Consortium (CPIC) guidelines for ivacaftor therapy in the context of CFTR genotype. Clin Pharmacol Ther. 2014 Jun;95(6):592-7. [40 references] PubMed External Web Site Policy