%0 Generic
%A Carrasco-Hernandez, Laura 
%A Giron-Moreno, Rosa M. 
%A Pelaez, Adrian
%A Gomez-Bonilla, Ainhoa 
%A Gomez-Crespo, Beatriz 
%A Diab-Caceres, Layla 
%A Tejedor-Ortiz, Ma Teresa 
%A Garcia-Clemente, Marta 
%A Solis-Garcia, Marta 
%A Gonzalez-Torres, Lucia 
%A Blanco-Aparicio, Marina 
%A Olveira-Fuster, Casilda
%A Giron-Fernandez, Ma Victoria 
%A Zamarron-de-Lucas, Esther 
%A Prados-Sanchez, Concha 
%A Quintana-Gallego, Esther 
%T Real Life With Tezacaftor and Ivacaftor in Adult Patients With Cystic Fibrosis: Spanish
%D 2022
%@ 0300-2896
%U http://hdl.handle.net/10668/22018
%X Cystic fibrosis (CF) is an autosomal recessive, multisystem genetic disease that mainly affects the exocrine glands due to the absence or alteration of a protein, called cystic fibrosis transmembrane conductance regulator (CFTR).1,2 Until a decade ago, the only treatments available tried to control or prevent the symptoms that were occurring. However, in recent years a line of treatments that improve the functionality of the altered protein has been developed, called CFTR modulators. Tezacaftor–ivacaftor (TEZ/IVA) is modulator of CFTR, indicated in a combined administration regimen for the treatment of CF patients ≥ 6 years, homozygous or heterozygous for the F508del mutation with residual function mutations.3 This drug has been available in Spain since October 1, 2019.
%K Cystic Fibrosis
%K Cystic Fibrosis Transmembrane Conductance Regulator
%K Tezacaftor
%K Ivacaftor
%~