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RASSEGNE - Reviews

Volume:

Biochimica Clinica 2023; 47(2) 127-131

Pubblicato on-line:

May 8, 2023

DOI:

10.19186/BC_2029.027

Scarica in PDF:
Autenticazione richiesta

Monitoraggio terapeutico di Ivacaftor, Tezacaftor e Elexacaftor in pazienti con fibrosi cistica
Therapeutic monitoring of Ivacaftor, Tezacaftor and Elexacaftor in patients with cystic fibrosis

AUTORI

Federica Pigliasco1, Sebastiano Barco1, Nicoletta Pedemonte2, Federico Cresta3, Rosaria Casciaro3, Carlo Castellani3, Laura Barbagallo1, Angelo Maffia1, Giuliana Cangemi1 Alessia Cafaro1,4
1IRCCS Istituto Giannina Gaslini, U.O.C. Laboratorio Centrale di Analisi Settore Cromatografia e Spettrometria di Massa, Genova
2UOC Genetica Medica, IRCCS Istituto Giannina Gaslini, 16147 Genoa
3Cystic Fibrosis Center, IRCCS Istituto Giannina Gaslini, 16147 Genoa
4Dipartimento di medicina Interna, Farmacologia e Tossicologia, Università di Genova

ABSTRACT

Therapeutic monitoring of Ivacaftor, Tezacaftor and Elexacaftor in patients with cystic fibrosis

The revolutionary new cystic fibrosis (CF) drug combination of ivacaftor (IVA), tezacaftor (TEZ) and elexacaftor (ELX) directly modulates the activity and trafficking of the transmembrane conductance defect (CFTR) regulatory protein underlying CF disease. Limited information is currently available in the literature on plasmatic concentrations of IVA, TEZ and ELX. However, significant interindividual pharmacokinetic variability has been reported in patients receiving the IVA/TEZ/ELX combination, exacerbated by several previously documented drug-drug interactions. Therapeutic drug monitoring (TDM) could therefore be useful in optimizing treatments with CFTR modulators. The purpose of this paper is to provide an overview of currently available information on pharmacokinetics/pharmacodynamics (PK/PD) of the drugs and their relationships to better understand the basis for differences in treatment response. Relevant articles reporting PK/PD data on IVA, TEZ, ELX administered for the treatment of CF were obtained from the PubMed database.

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