Effects of Lumacaftor-Ivacaftor on Lung Clearance Index, Magnetic Resonance Imaging, and Airway Microbiome in Phe508del Homozygous Patients with Cystic Fibrosis

Autor:	Marcus A. Mall, Mark O. Wielpütz, Hans-Ulrich Kauczor, Alexander H. Dalpke, Simon Y. Graeber, Olaf Sommerburg, Cornelia Joachim, Mirjam Stahl, Dario L. Frey, Sébastien Boutin, Sabine Wege
Rok vydání:	2021
Předmět:	Pulmonary and Respiratory Medicine congenital hereditary and neonatal diseases and abnormalities Pathology medicine.medical_specialty Cystic Fibrosis Aminopyridines Cystic Fibrosis Transmembrane Conductance Regulator Lung Clearance Index Quinolones Aminophenols Cystic fibrosis Ivacaftor chemistry.chemical_compound medicine Humans Microbiome Benzodioxoles Prospective Studies Lung medicine.diagnostic_test business.industry Microbiota Lumacaftor Magnetic resonance imaging medicine.disease Magnetic Resonance Imaging Transmembrane protein chemistry Mutation business Airway medicine.drug
Zdroj:	Annals of the American Thoracic Society. 18(6)
ISSN:	2325-6621
Popis:	Rationale: Previous studies showed that lumacaftor–ivacaftor therapy results in partial rescue of CFTR (cystic fibrosis [CF] transmembrane conductance regulator) activity and a moderate improvement...
Databáze:	OpenAIRE
Externí odkaz:	https://explore.openaire.eu/search/publication?articleId=doi_dedup___::5d495120bb016c0c7146e74a3415ce35 https://pubmed.ncbi.nlm.nih.gov/33600745 Zobrazit plný text záznamu