Human epididymis protein 4 (HE4) plasma concentration inversely correlates with the improvement of cystic fibrosis lung disease in p.Phe508del-CFTR homozygous cases treated with the CFTR modulator lumacaftor/ivacaftor combination

Author:

Pócsi Marianna,Fejes Zsolt,Bene Zsolt,Nagy Attila,Balogh IstvánORCID,Amaral Margarida D.ORCID,Macek Milan,Nagy Béla

Funder

National Research, Development and Innovation Office

Cystic Fibrosis Foundation

Ministerstvo Školství, Mládeže a Tělovýchovy

Fundação para a Ciência e a Tecnologia

Ministerstvo Zdravotnictví Ceské Republiky

Hungarian Scientific Research Fund

Publisher

Elsevier BV

Subject

Pulmonary and Respiratory Medicine,Pediatrics, Perinatology and Child Health

Reference33 articles.

1. Cystic fibrosis;Rowe;N Engl J Med,2005

2. ECFS Patient Registry Annual Report, 2020. (https://www.ecfs.eu/projects/ecfs-patient-egistry/annual-reports).

3. Progress in therapies for cystic fibrosis;De Boeck;Lancet Respir Med,2016

4. Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770;Van Goor;Proc Natl Acad Sci USA,2009

5. Efficacy and safety of ivacaftor in patients with cystic fibrosis and a non-G551D gating mutation;De Boeck;J Cyst Fibros,2014

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