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We wanted to resolve the role of CFTR dysfunction in people with idiopathic bronchiectasis by nasal potential difference (NPD) measurements.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>NPD was examined in people with cystic fibrosis (CF), healthy controls, 40 people with idiopathic bronchiectasis recruited from the local outpatient clinic and in 60 people with idiopathic bronchiectasis with the suspected etiology of CF who had been referred from 2010 \u2013 2022 to our electrophysiological laboratory to make a diagnosis by NPD.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>The unselected MHH cohort and the preselected diagnostic cohort of people with idiopathic bronchiectasis matched in their basal NPD potential with healthy controls. Conversely, after inhibition of the sodium conductance with amiloride, the distribution of the CFTR-mediated depolarization potential upon exposure to chloride-free solution and isoproterenol was in between those of healthy controls and CF patients with exocrine pancreatic insufficiency and overlapped with that of patients with exocrine pancreatic sufficiency. This intermediate phenotype was characteristic for the whole study population of 100 people with idiopathic bronchiectasis irrespectively of whether clinical features of CFTR dysfunction had been recognized before in an individual. Taking the Sermet Score that was developed to discriminate patients with CF from non-CF patients by NPD, the bronchiectasis population was significantly distinct from both healthy people and people with CF.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>A CFTR activity of the nasal surface epithelium in the lower quartile is typical for people with idiopathic bronchiectasis, but further CFTR-independent inherited susceptibilities and external insults are necessary to materialize the emergence of bronchiectasis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Trial registration<\/jats:title>\n                    <jats:p>Clinical trial number: not applicable.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1186\/s12931-026-03599-1","type":"journal-article","created":{"date-parts":[[2026,3,3]],"date-time":"2026-03-03T04:32:36Z","timestamp":1772512356000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["CFTR activity in nasal potential difference of adults with idiopathic bronchiectasis"],"prefix":"10.1186","volume":"27","author":[{"given":"Burkhard","family":"T\u00fcmmler","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angela","family":"Schulz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rebecca","family":"Minso","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nadine","family":"Alfeis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephanie","family":"Tamm","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jessica","family":"Rademacher","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Felix C.","family":"Ringshausen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,3,3]]},"reference":[{"key":"3599_CR1","doi-asserted-by":"publisher","unstructured":"Chalmers JD, Haworth CS, Flume P, Long MB, Burgel PR, Dimakou K, Blasi F, Herrero-Cortina B, Dhar R, Chotirmall SH, Ringshausen FC, Altenburg J, Morgan L, Nigro M, Crichton ML, Van Meel C, Sibila O, Timothy A, Kompatsiari E, Hedberg T, Vandendriessche T, McShane PJ, Tonia T, Winthrop K, Loebinger MR, Lorent N, Goeminne P, Shteinberg M, Polverino E, Aliberti S. 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Informed consent was obtained from all participants of the BE MHH study or their parental guides. Likewise, the Diagnostic BE study (assessment of healthy people, people with CF and people of the diagnostic BE cohort by CFTR biomarkers and the documentation of metadata) was approved by the Ethics Committee of Hannover Medical School (study nos. 6071 and 6790). Informed consent was obtained from all participants of the diagnostic BE study or their parental guides. The BE MHH study and the Diagnostic BE study were performed in accordance with the Declaration of Helsinki as revised in 2013.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Not applicable.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"Burkhard T\u00fcmmler reports grants from the Deutsche Forschungsgemeinschaft (DFG), the Volkswagen Stiftung, the German Center for Lung Research (DZL) and the German Center for Infection Research (DZIF); consulting fees from the Helmholtz-Zentrum f\u00fcr Infektionsforschung; honoraria for lectures and educational events from Vertex Pharmaceuticals Germany; payment for the attendance of advisory boards from Vertex Pharmaceutical Inc; unpaid roles as co-speaker of the Disease Spanning Working Group \u2018Microbiome\/Metagenome\u2019 of the DZL and as a member of the Scientific Advisory Board of the Christiane Herzog Stiftung. Jessica Rademacher reports payment or honoraria for lectures, presentations, speakers bureaus, or educational events from CSL Seqirus, Berlin-Chemie, GlaxoSmithKline, AstraZeneca, Chiesi, ThermoFisher, Boehringer Ingelheim, Pfizer, Shionogi, and INSMED; and participation on a data safety monitoring board or advisory board from GlaxoSmithKline, GILEAD, ThermoFischer, Shionogi, and INSMED. Felix Christian Ringshausen reports grants or contracts from the German Center for Lung Research (DZL), the German Center for Infection Research (DZIF), the Innovative Medicines Initiative (EU\/EFPIA), and the iABC Consortium (incl. Alaxia, Basilea, Novartis, and Polyphor), Mukoviszidose Institute, Novartis, Insmed Germany, Grifols, Bayer, InfectoPharm; consulting fees from Parion, Grifols, Zambon, Insmed, and Helmholtz-Zentrum f\u00fcr Infektionsforschung; payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing, or educational events from I!DE Werbeagentur GmbH, Interkongress GmbH, AstraZeneca, Insmed, Grifols, and Universit\u00e4tsklinikum Frankfurt am Main; payment for expert testimony from Social Court Cologne; support for attending meetings, travel, from German Kartagener Syndrome and Primary Ciliary Dyskinesia Patient Advocacy Group and Mukoviszidose e.V.; participation on a data safety monitoring board or advisory board for Insmed, Grifols, and Shionogi; leadership or fiduciary role, paid or unpaid, as coordinator of the European Reference Network (ERN)-LUNG Bronchiectasis Core Network, chair of the The Prospective German Non-CF-Bronchiectasis Registry (PROGNOSIS), member of the steering committee of the European Bronchiectasis Registry EMBARC, member of the steering committee of the European Nontuberculous Mycobacterial Pulmonary Disease Registry (EMBARC-NTM), co-speaker of the medical advisory board of the German Kartagener Syndrome and Primary Ciliary Dyskinesia Patient Advocacy Group, speaker of the respiratory infections and TB group of the German Respiratory Society, speaker of the cystic fibrosis group of German Respiratory Society (DGP), primary investigator of the German Center for Lung Research, member of the protocol review committee of the Primary Ciliary Dyskinesia-Clinical Trial Network (PCD-CTN), member of physician association of the German Cystic Fibrosis Patient Advocacy Group; and other financial or nonfinancial interests with AstraZeneca, Boehringer Ingelheim, Celtaxsys, Corbus, Insmed, Novartis, Parion, University of Dundee, Vertex, and Zambon. Angela Schulz, Rebecca Minso, Nadine Alfeis and Stephanie Tamm declare no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"160"}}