المؤلفون: Vijftigschild LA; Dept of Pediatric Pulmonology, University Medical Center, Utrecht, The Netherlands Laboratory of Translational Immunology, University Medical Center, Utrecht, The Netherlands These two authors contributed equally to this work., Berkers G; Dept of Pediatric Pulmonology, University Medical Center, Utrecht, The Netherlands These two authors contributed equally to this work., Dekkers JF; Dept of Pediatric Pulmonology, University Medical Center, Utrecht, The Netherlands Laboratory of Translational Immunology, University Medical Center, Utrecht, The Netherlands These two authors contributed equally to this work., Zomer-van Ommen DD; Dept of Pediatric Pulmonology, University Medical Center, Utrecht, The Netherlands Laboratory of Translational Immunology, University Medical Center, Utrecht, The Netherlands These two authors contributed equally to this work., Matthes E; CF Translational Research Centre, Dept of Physiology, McGill University, Montréal, QC, Canada., Kruisselbrink E; Dept of Pediatric Pulmonology, University Medical Center, Utrecht, The Netherlands Laboratory of Translational Immunology, University Medical Center, Utrecht, The Netherlands., Vonk A; Dept of Pediatric Pulmonology, University Medical Center, Utrecht, The Netherlands Laboratory of Translational Immunology, University Medical Center, Utrecht, The Netherlands., Hensen CE; Dept of Pediatric Pulmonology, University Medical Center, Utrecht, The Netherlands., Heida-Michel S; Dept of Pediatric Pulmonology, University Medical Center, Utrecht, The Netherlands., Geerdink M; Dept of Pediatric Pulmonology, University Medical Center, Utrecht, The Netherlands., Janssens HM; Dept of Pediatric Pulmonology, Erasmus Medical Center/Sophia Children's Hospital, Rotterdam, The Netherlands., van de Graaf EA; Dept of Pulmonology, University Medical Center, Utrecht, The Netherlands., Bronsveld I; Dept of Pulmonology, University Medical Center, Utrecht, The Netherlands., de Winter-de Groot KM; Dept of Pediatric Pulmonology, University Medical Center, Utrecht, The Netherlands., Majoor CJ; Dept of Respiratory Medicine, Academic Medical Center, Amsterdam, The Netherlands., Heijerman HG; Dept of Pulmonology and Cystic Fibrosis, Haga Teaching Hospital, The Hague, The Netherlands., de Jonge HR; Dept of Gastroenterology and Hepatology, Erasmus University Medical Center, Rotterdam, The Netherlands., Hanrahan JW; CF Translational Research Centre, Dept of Physiology, McGill University, Montréal, QC, Canada., van der Ent CK; Dept of Pediatric Pulmonology, University Medical Center, Utrecht, The Netherlands., Beekman JM; Dept of Pediatric Pulmonology, University Medical Center, Utrecht, The Netherlands Regenerative Medicine Center Utrecht, University Medical Center, Utrecht, The Netherlands jbeekman@umcutrecht.nl.

المصدر: The European respiratory journal [Eur Respir J] 2016 Sep; Vol. 48 (3), pp. 768-79. Date of Electronic Publication: 2016 Jul 28.

نوع المنشور: Clinical Trial, Phase II; Journal Article; Randomized Controlled Trial; Research Support, Non-U.S. Gov't

بيانات الدورية: Publisher: European Respiratory Society Country of Publication: England NLM ID: 8803460 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1399-3003 (Electronic) Linking ISSN: 09031936 NLM ISO Abbreviation: Eur Respir J Subsets: MEDLINE

مواضيع طبية MeSH: Adrenergic beta-2 Receptor Agonists/*pharmacology , Cystic Fibrosis/*drug therapy , Cystic Fibrosis/*metabolism , Cystic Fibrosis Transmembrane Conductance Regulator/*metabolism, Administration, Oral ; Albuterol/administration & dosage ; Biological Assay ; Bronchi/pathology ; Cell Line ; Cells, Cultured ; Chlorides/chemistry ; Cystic Fibrosis/genetics ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Drug Evaluation, Preclinical ; Epithelial Cells/metabolism ; Epithelium/metabolism ; Humans ; Mutation ; Nasal Mucosa/drug effects ; Nasal Mucosa/metabolism ; Organoids ; Pilot Projects ; Respiratory System/metabolism ; Signal Transduction