دورية أكاديمية
Multiple-breath washout to detect lung disease in patients with inborn errors of immunity.
| العنوان: | Multiple-breath washout to detect lung disease in patients with inborn errors of immunity. |
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| المؤلفون: | Busack LM; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, Berlin, Germany., Thee S; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, Berlin, Germany.; Berlin Institute of Health at Charité - Universitätsmedizin Berlin, Berlin, Germany., Liu Y; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, Berlin, Germany., Allomba C; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, Berlin, Germany., Ziegahn N; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, Berlin, Germany., Tosolini A; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, Berlin, Germany., Pioch CO; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, Berlin, Germany., Schnorr AN; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, Berlin, Germany., Fuhlrott BR; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, Berlin, Germany., Staudacher O; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, Berlin, Germany., Völler M; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, Berlin, Germany.; German Center for Lung Research, associated partner site, Berlin, Germany., Steinke E; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, Berlin, Germany.; Berlin Institute of Health at Charité - Universitätsmedizin Berlin, Berlin, Germany.; German Center for Lung Research, associated partner site, Berlin, Germany., Hanitsch LG; Berlin Institute of Health at Charité - Universitätsmedizin Berlin, Berlin, Germany.; Institute of Medical Immunology, Charité - Universitätsmedizin Berlin, Berlin, Germany.; Berlin Center for Regenerative Therapies, Charité - Universitätsmedizin Berlin, Berlin, Germany., Röhmel J; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, Berlin, Germany.; Berlin Institute of Health at Charité - Universitätsmedizin Berlin, Berlin, Germany.; German Center for Lung Research, associated partner site, Berlin, Germany., Wahn V; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, Berlin, Germany., Krüger R; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, Berlin, Germany., Mall MA; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, Berlin, Germany.; Berlin Institute of Health at Charité - Universitätsmedizin Berlin, Berlin, Germany.; German Center for Lung Research, associated partner site, Berlin, Germany., von Bernuth H; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, Berlin, Germany.; Berlin Institute of Health at Charité - Universitätsmedizin Berlin, Berlin, Germany.; Berlin Center for Regenerative Therapies, Charité - Universitätsmedizin Berlin, Berlin, Germany.; Department of Immunology, Labor Berlin-Charité Vivantes GmbH, Berlin, Germany., Stahl M; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, Berlin, Germany.; Berlin Institute of Health at Charité - Universitätsmedizin Berlin, Berlin, Germany.; German Center for Lung Research, associated partner site, Berlin, Germany. |
| المصدر: | ERJ open research [ERJ Open Res] 2024 Mar 11; Vol. 10 (2). Date of Electronic Publication: 2024 Mar 11 (Print Publication: 2024). |
| نوع المنشور: | Journal Article |
| اللغة: | English |
| بيانات الدورية: | Publisher: European Respiratory Society Country of Publication: England NLM ID: 101671641 Publication Model: eCollection Cited Medium: Print ISSN: 2312-0541 (Print) Linking ISSN: 23120541 NLM ISO Abbreviation: ERJ Open Res Subsets: PubMed not MEDLINE |
| أسماء مطبوعة: | Original Publication: Sheffield : European Respiratory Society, [2015]- |
| مستخلص: | Background: Pulmonary manifestations are the major cause of morbidity and mortality in patients with inborn errors of immunity (IEI). New and more sensitive diagnostic methods can potentially lead to earlier recognition and treatment of IEI lung disease and improve outcome. The aim of this study was to compare multiple-breath washout (MBW) and spirometry in patients with IEI and cystic fibrosis (CF) as well as healthy controls (HC) and to evaluate the sensitivity of lung clearance index (LCI) to assess lung disease in IEI. Methods: IEI patients (n=114) were recruited from our paediatric and adult immunodeficiency outpatient clinics and compared to age-matched CF patients (n=114) and HC (n=114). MBW measurements and spirometry were performed in the study participants, and MBW testing was repeated after 63-707 days in IEI patients (n=70). Results: The LCI was significantly higher in IEI patients than in HC ( p <0.001) and significantly lower than in CF patients (p<0.001). The forced expiratory volume in 1 s (FEV Conclusion: MBW is useful to detect lung disease in IEI and is more sensitive than spirometry. Competing Interests: Conflict of interest: S. Thee declares grants to their institution from the German Innovation Fond; payment or honoraria from Chiesi (European Cystic Fibrosis Society meeting 2022) and Vertex (LEAD meeting, adherence forum); and travel expenses from Chiesi to attend the European Cystic Fibrosis Society meeting 2022. Conflict of interest: O. Staudacher declares a Travel Grant for Young Immunologists from Arbeitsgemeinschaft Pädiatrische Immunologie in the 36 months prior to manuscript submission. Conflict of interest: E. Steinke declares grants or contracts from the Clinician Scientist Program of the Berlin Institute of Health (funded by the German Federal Ministry of Education and Research); payment or honoraria for review of educational manuscripts from Vertex Pharmaceuticals Inc.; and a travel grant for conference attendance from the European Cystic Fibrosis Society, all in the 36 months prior to manuscript submission. Conflict of interest: J. Röhmel declares payment or honoraria, and support for attending meetings and/or travel from Vertex Pharmaceuticals in the 36 months prior to manuscript submission (with permission from their institution); and an unpaid role as a work package leader in BEAT-PCD, a European Research Society Clinical Research Collaboration. Conflict of interest: V. Wahn declares payment or honoraria from CSL Behring (related to www.immundefekt.de) and Pharming (related to a manuscript on APDS); and an honorarium and travel expenses from Pharming for attendance at the DGKJ congress, all in the 36 months prior to manuscript submission. Conflict of interest: H. von Bernuth declares consulting fees from Infill Healthcare Communication (2022); payment or honoraria from Deutsche Selbsthilfe Angeborene Immundefekte) (2023) and CSL Behring (2021); and payment for expert testimony from Bundessozialgericht (2023); as well as that they are a member of the Standing Comission on Vaccination at the Robert Koch Insitut, Ständige Impfkommission (STIKO). Conflict of interest: M.A. Mall declares funding to their institution in connection to the present work from the German Ministry for Education and Research (BMBF; Grant #82DZL009B1); and an unpaid role as a Fellow of the European Respiratory Society. Conflict of interest: M. Stahl declares funding to their institution (Heisenberg professorship) from the German Research Foundation (Deutsche Forschungsgemeinschaft) in connection to the present work; as well as unpaid roles as Chairman of the Forschungsgemeinschaft Mukoviszidose; Secretary of the Cystic Fibrosis Group of the European Respiratory Society; and Treasurer of the German Society of Paediatric Pulmonology. Conflict of interest: All other authors declare no competing interests. (Copyright ©The authors 2024.) |
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| تواريخ الأحداث: | Date Created: 20240312 Latest Revision: 20240313 |
| رمز التحديث: | 20240313 |
| مُعرف محوري في PubMed: | PMC10926008 |
| DOI: | 10.1183/23120541.01019-2023 |
| PMID: | 38469376 |
| قاعدة البيانات: | MEDLINE |
Full Text Finder | تدمد: | 2312-0541 |
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| DOI: | 10.1183/23120541.01019-2023 |