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Article

Real-life experience with a generic formulation of lumacaftor-ivacaftor in patients with cystic fibrosis homozygous for the Phe508del CFTR mutation.

Pediatric pulmonology - 1 Dec 2023

Teper Alejandro, Lubovich Silvina, Rodríguez Viviana, Zaragoza Silvina, Rodríguez Ezequiel, Bournissen Facundo García

Abstract excerpt

INTRODUCTION: Cystic fibrosis (CF) is the most frequent recessive autosomal disorder in the Caucasian population. It is caused by mutations that result in a deficient or dysfunctional cystic fibrosis transmembrane conductance regulator (CFTR) protein activity. Among CFTR modulators, potentiator compounds increase channel opening, whereas corrector compounds increase CFTR quantity in the cell surface. OBJECTIVE:...

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PMID: 37712606
DOI: 10.1002/ppul.26690

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