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

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 (FEV1) moderately, but predomina...

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Veröffentlicht in:Swiss medical weekly 2018-02, Vol.148 (78), p.w14593-w14593
Hauptverfasser: Murer, Christian, Huber, Lars Christian, Kurowski, Thomas, Hirt, Astrid, Robinson, Cécile A, Bürgi, Urs, Benden, Christian
Format: Artikel
Sprache:eng
Schlagworte:
CFTR-modulator
Cystic fibrosis
end-stage pulmonary disease
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Zusammenfassung: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 (FEV1) moderately, but predominantly reduce acute exacerbation rate (AER) in Phe508del homozygous cystic fibrosis patients; however, patients with FEV1
ISSN:1424-7860
1424-3997
DOI:10.4414/smw.2018.14593