Changes in sputum viscoelastic properties and airway inflammation in primary ciliary dyskinesia are comparable to cystic fibrosis on elexacaftor/tezacaftor/ivacaftor therapy.

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Title: Changes in sputum viscoelastic properties and airway inflammation in primary ciliary dyskinesia are comparable to cystic fibrosis on elexacaftor/tezacaftor/ivacaftor therapy.
Authors: Nussstein H; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.; German Center for Lung Research (DZL), associated partner site Berlin, Berlin, Germany.; German Center for Child and Adolescent Health (DZKJ), partner site Berlin, Berlin, Germany., Urbantat RM; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.; German Center for Lung Research (DZL), associated partner site Berlin, Berlin, Germany.; German Center for Child and Adolescent Health (DZKJ), partner site Berlin, Berlin, Germany.; Berlin Institute of Health at Charité - Universitätsmedizin Berlin, Berlin, Germany., Fentker K; Proteomics Platform, Max Delbrück Center for Molecular Medicine, Berlin, Germany.; Institute for Chemistry and Biochemistry, Freie Universität Berlin, Berlin, Germany., Loewe A; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.; German Center for Lung Research (DZL), associated partner site Berlin, Berlin, Germany.; German Center for Child and Adolescent Health (DZKJ), partner site Berlin, Berlin, Germany., Duerr J; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.; German Center for Lung Research (DZL), associated partner site Berlin, Berlin, Germany.; German Center for Child and Adolescent Health (DZKJ), partner site Berlin, Berlin, Germany., Haji M; Proteomics Platform, Max Delbrück Center for Molecular Medicine, Berlin, Germany., Doellinger F; Department of Radiology, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany., Stahl M; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.; German Center for Lung Research (DZL), associated partner site Berlin, Berlin, Germany.; German Center for Child and Adolescent Health (DZKJ), partner site Berlin, Berlin, Germany.; Berlin Institute of Health at Charité - Universitätsmedizin Berlin, Berlin, Germany., Graeber SY; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.; German Center for Lung Research (DZL), associated partner site Berlin, Berlin, Germany.; German Center for Child and Adolescent Health (DZKJ), partner site Berlin, Berlin, Germany.; Berlin Institute of Health at Charité - Universitätsmedizin Berlin, Berlin, Germany., Gradzielski M; Stranski-Laboratorium für Physikalische und Theoretische Chemie, Institut für Chemie, Technische Universität Berlin, Berlin, Germany., Röhmel J; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.; German Center for Lung Research (DZL), associated partner site Berlin, Berlin, Germany.; German Center for Child and Adolescent Health (DZKJ), partner site Berlin, Berlin, Germany.; Berlin Institute of Health at Charité - Universitätsmedizin Berlin, Berlin, Germany., Mertins P; German Center for Child and Adolescent Health (DZKJ), partner site Berlin, Berlin, Germany.; Berlin Institute of Health at Charité - Universitätsmedizin Berlin, Berlin, Germany.; Proteomics Platform, Max Delbrück Center for Molecular Medicine, Berlin, Germany., Schaupp L; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.; German Center for Lung Research (DZL), associated partner site Berlin, Berlin, Germany.; German Center for Child and Adolescent Health (DZKJ), partner site Berlin, Berlin, Germany.; L. Schaupp and M.A. Mall contributed equally as senior authors., Mall MA; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany marcus.mall@charite.de.; German Center for Lung Research (DZL), associated partner site Berlin, Berlin, Germany.; German Center for Child and Adolescent Health (DZKJ), partner site Berlin, Berlin, Germany.; Berlin Institute of Health at Charité - Universitätsmedizin Berlin, Berlin, Germany.; L. Schaupp and M.A. Mall contributed equally as senior authors.
Source: The European respiratory journal [Eur Respir J] 2026 Feb 05; Vol. 67 (2). Date of Electronic Publication: 2026 Feb 05 (Print Publication: 2026).
Publication Type: Journal Article; Comparative Study
Journal Info: Publisher: European Respiratory Society Country of Publication: England NLM ID: 8803460 Publication Model: Electronic-Print Cited Medium: Internet ISSN: 1399-3003 (Electronic) Linking ISSN: 09031936 NLM ISO Abbreviation: Eur Respir J Subsets: MEDLINE
Database: MEDLINE Ultimate
Description
ISSN:1399-3003
DOI:10.1183/13993003.00616-2025