First experience in Switzerland in Phe508del homozygous cystic fibrosis patients with end-stage pulmonary disease enrolled in a lumacaftor-ivacaftor therapy trial – preliminary results

First experience in Switzerland in Phe508del homozygous cystic fibrosis patients with end-stage pulmonary disease enrolled in a lumacaftor-ivacaftor therapy trial – preliminary results

AIMS OF THE STUDY Cystic fibrosis is the most common genetic disorder in Caucasians. The combination of the cystic fibrosis transmembrane conductance regulator (CFTR) corrector lumacaftor / potentiator ivacaftor (LUM/IVA) has been shown to increase forced expiratory volume in 1 second (F...

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Bibliographic Details
Main Authors: Christian Murer, Lars C. Huber, Thomas Kurowski, Astrid Hirt, Cécile Robinson, Urs Bürgi, Christian Benden
Format: Article
Language:English
Published: SMW supporting association (Trägerverein Swiss Medical Weekly SMW) 2018-02-01
Series:Swiss Medical Weekly
Subjects:
CFTR-modulator
Cystic fibrosis
end-stage pulmonary disease
Online Access:https://www.smw.ch/index.php/smw/article/view/2449

Internet

https://www.smw.ch/index.php/smw/article/view/2449

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