{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,25]],"date-time":"2026-07-25T00:03:52Z","timestamp":1784937832178,"version":"3.55.0"},"reference-count":31,"publisher":"MDPI AG","issue":"13","license":[{"start":{"date-parts":[[2025,7,7]],"date-time":"2025-07-07T00:00:00Z","timestamp":1751846400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Objectives: Although patients with bronchiectasis tend to have obstructive nonreversible lung functions, some have bronchodilator response (BDR), and a relatively large number are treated with bronchodilators. We assessed BDR in patients with cystic fibrosis (CF) and other bronchiectatic diseases and healthy controls (HCs) in a randomized controlled setup. Methods: Patients with cystic fibrosis (CF), primary ciliary dyskinesia (PCD) and non-CF non-PCD bronchiectasis (non-CF\/PCD), as well as HCs, were recruited. Participants were randomly assigned to receive salbutamol (four puffs) and then a placebo or a placebo and then salbutamol. BDR was calculated using the American Thoracic Society (ATS)\/European Respiratory Society (ERS) standard, defined as the change related to the individual\u2019s predicted value (new method) or to the initial value (old method). Results: Sixty-nine patients (CF = 30, PCD = 20, non-CF\/PCD = 19) and 20 HCs were included. Patients with CF and PCD (but not non-CF\/PCD) had a statistically greater mean response to salbutamol compared with the placebo, (CF\u2013salbutamol first: 2.82 vs. 0.85%, p = 0.01; placebo first: 2.39 vs. \u22120.27%, p = 0.02; PCD\u2013salbutamol first: 5.32 vs. 1.88%, p = 0.01; placebo first: 2.24 vs. 0.77%, p = 0.05). Few patients had significant BDR (new method, &gt;10%)\u2014CF (0), PCD (2), non-CF\/PCD (0) and HCs (2)): using the old method, an additional PCD patient and three non-CF\/PCD had significant BDR (&gt;12%). Conclusions: Significant BDR seems to be rare in patients with bronchiectasis. In CF and PCD, the response was greater than the placebo; the clinical significance of this difference and its therapeutic implications, as well as the best method to determine BDR, have yet to be determined.<\/jats:p>","DOI":"10.3390\/jcm14134778","type":"journal-article","created":{"date-parts":[[2025,7,7]],"date-time":"2025-07-07T06:03:13Z","timestamp":1751868193000},"page":"4778","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Assessment of Bronchodilator Response in Patients with CF and Non-CF Bronchiectasis\u2014A Randomized Controlled Study"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3793-200X","authenticated-orcid":false,"given":"Mordechai","family":"Pollak","sequence":"first","affiliation":[{"name":"Pediatric Pulmonary Institute, Rappaport Children\u2019s Hospital, Rambam Healthcare Campus, P.O. Box 9602, Haifa 3109601, Israel"},{"name":"The Adelson School of Medicine, Ariel University, Ariel 40700, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0055-5415","authenticated-orcid":false,"given":"Ronen","family":"Bar-Yoseph","sequence":"additional","affiliation":[{"name":"Pediatric Pulmonary Institute, Rappaport Children\u2019s Hospital, Rambam Healthcare Campus, P.O. Box 9602, Haifa 3109601, Israel"},{"name":"Faculty of Medicine, Technion Israel Institute of Technology, Haifa 32000, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Moneera","family":"Hanna","sequence":"additional","affiliation":[{"name":"Pediatric Pulmonary Institute, Rappaport Children\u2019s Hospital, Rambam Healthcare Campus, P.O. Box 9602, Haifa 3109601, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Noa","family":"Serruya","sequence":"additional","affiliation":[{"name":"Pediatric Pulmonary Institute, Rappaport Children\u2019s Hospital, Rambam Healthcare Campus, P.O. Box 9602, Haifa 3109601, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guy","family":"Gut","sequence":"additional","affiliation":[{"name":"Pediatric Pulmonary Institute, Rappaport Children\u2019s Hospital, Rambam Healthcare Campus, P.O. Box 9602, Haifa 3109601, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lea","family":"Bentur","sequence":"additional","affiliation":[{"name":"Pediatric Pulmonary Institute, Rappaport Children\u2019s Hospital, Rambam Healthcare Campus, P.O. Box 9602, Haifa 3109601, Israel"},{"name":"The Adelson School of Medicine, Ariel University, Ariel 40700, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9874-7617","authenticated-orcid":false,"given":"Michal","family":"Gur","sequence":"additional","affiliation":[{"name":"Pediatric Pulmonary Institute, Rappaport Children\u2019s Hospital, Rambam Healthcare Campus, P.O. Box 9602, Haifa 3109601, Israel"},{"name":"The Adelson School of Medicine, Ariel University, Ariel 40700, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,7,7]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1446","DOI":"10.1183\/09031936.00119114","article-title":"Management of Bronchiectasis in Adults","volume":"45","author":"Chalmers","year":"2015","journal-title":"Eur. Respir. J."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1693","DOI":"10.1056\/NEJMra2216474","article-title":"Cystic Fibrosis","volume":"389","author":"Grasemann","year":"2023","journal-title":"N. Engl. J. Med."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1016\/j.jpeds.2020.11.040","article-title":"Understanding Primary Ciliary Dyskinesia and Other Ciliopathies","volume":"230","author":"Horani","year":"2021","journal-title":"J. Pediatr."},{"key":"ref_4","doi-asserted-by":"crossref","unstructured":"Wall, L.A., Wisner, E.L., Gipson, K.S., and Sorensen, R.U. (2020). Bronchiectasis in Primary Antibody Deficiencies: A Multidisciplinary Approach. Front. Immunol., 11.","DOI":"10.3389\/fimmu.2020.00522"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1383","DOI":"10.1056\/NEJMra012519","article-title":"Bronchiectasis","volume":"346","author":"Barker","year":"2002","journal-title":"N. Engl. J. Med."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"120","DOI":"10.1016\/j.rmed.2018.10.031","article-title":"A Comprehensive Approach to Lung Function in Bronchiectasis","volume":"145","author":"Radovanovic","year":"2018","journal-title":"Respir. Med."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"179","DOI":"10.1136\/thx.39.3.179","article-title":"Atopy, Immunological Changes, and Respiratory Function in Bronchiectasis","volume":"39","author":"Murphy","year":"1984","journal-title":"Thorax"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"423","DOI":"10.1046\/j.1440-1843.1999.00215.x","article-title":"Bronchodilator Response to Inhaled Beta-2 Agonist and Anticholinergic Drugs in Patients with Bronchiectasis","volume":"4","author":"Hassan","year":"1999","journal-title":"Respirology"},{"key":"ref_9","first-page":"14","article-title":"Bronchodilator Response in Adults with Bronchiectasis: Correlation with Clinical Parameters and Prognostic Implications","volume":"8","author":"Guan","year":"2016","journal-title":"J. Thorac. Dis."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"2757","DOI":"10.2147\/COPD.S115581","article-title":"Effects of Long-Term Bronchodilators in Bronchiectasis Patients with Airflow Limitation Based on Bronchodilator Response at Baseline","volume":"11","author":"Jeong","year":"2016","journal-title":"Int. J. Chronic Obstr. Pulm. Dis."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"2036","DOI":"10.1002\/ppul.25409","article-title":"Bronchodilator Responsiveness in Cystic Fibrosis Children Treated for Pulmonary Exacerbations","volume":"56","author":"Pollak","year":"2021","journal-title":"Pediatr. Pulmonol."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"652","DOI":"10.1016\/j.jcf.2016.01.003","article-title":"Reversible Airway Obstruction in Cystic Fibrosis: Common, but Not Associated with Characteristics of Asthma","volume":"15","author":"Levine","year":"2016","journal-title":"J. Cyst. Fibros."},{"key":"ref_13","doi-asserted-by":"crossref","unstructured":"Seidl, E., Gatt, D., Wee, W.B., Wilson, D., Ratjen, F., and Grasemann, H. (2024). Bronchodilator Responsiveness in Children with Primary Ciliary Dyskinesia. ERJ Open Res., 10.","DOI":"10.1183\/23120541.00611-2023"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"205","DOI":"10.1002\/ppul.22934","article-title":"Asthma and Cystic Fibrosis: A Tangled Web","volume":"49","author":"Kent","year":"2014","journal-title":"Pediatr. Pulmonol."},{"key":"ref_15","first-page":"339","article-title":"Positive Expiratory Pressure (PEP-Mask) Physiotherapy Improves Ventilation and Reduces Volume of Trapped Gas in Cystic Fibrosis","volume":"21","author":"Groth","year":"1985","journal-title":"Bull. Eur. Physiopathol. Respir."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"524","DOI":"10.1093\/ptj\/84.6.524","article-title":"Physiologic Evidence for the Efficacy of Positive Expiratory Pressure as an Airway Clearance Technique in Patients with Cystic Fibrosis","volume":"84","author":"Darbee","year":"2004","journal-title":"Phys. Ther."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"2101499","DOI":"10.1183\/13993003.01499-2021","article-title":"ERS\/ATS Technical Standard on Interpretive Strategies for Routine Lung Function Tests","volume":"60","author":"Stanojevic","year":"2022","journal-title":"Eur. Respir. J."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"1324","DOI":"10.1183\/09031936.00080312","article-title":"Multi-Ethnic Reference Values for Spirometry for the 3\u201395-Yr Age Range: The Global Lung Function 2012 Equations","volume":"40","author":"Quanjer","year":"2012","journal-title":"Eur. Respir. J."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"119","DOI":"10.1164\/rccm.202309-1614OC","article-title":"The Association between Bronchiectasis and Chronic Obstructive Pulmonary Disease: Data from the European Bronchiectasis Registry (EMBARC)","volume":"210","author":"Polverino","year":"2024","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1136\/thoraxjnl-2018-212463","article-title":"British Thoracic Society Guideline for Bronchiectasis in Adults","volume":"74","author":"Sullivan","year":"2019","journal-title":"Thorax"},{"key":"ref_21","first-page":"71","article-title":"South African Thoracic Society Position Statement on the Management of Non-Cystic Fibrosis Bronchiectasis in Adults: 2023","volume":"29","author":"Maasdorp","year":"2023","journal-title":"Afr. J. Thorac. Crit. Care Med."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"637","DOI":"10.1056\/NEJM199409083311003","article-title":"Effect of Aerosolized Recombinant Human DNase on Exacerbations of Respiratory Symptoms and on Pulmonary Function in Patients with Cystic Fibrosis","volume":"331","author":"Fuchs","year":"1994","journal-title":"N. Engl. J. Med."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"229","DOI":"10.1056\/NEJMoa043900","article-title":"A Controlled Trial of Long-Term Inhaled Hypertonic Saline in Patients with Cystic Fibrosis","volume":"354","author":"Elkins","year":"2006","journal-title":"N. Engl. J. Med."},{"key":"ref_24","doi-asserted-by":"crossref","unstructured":"Levine, H., Bar-On, O., Nir, V., West, N., Dizitzer, Y., Mussaffi, H., and Prais, D. (2022). Reversible Bronchial Obstruction in Primary Ciliary Dyskinesia. J. Clin. Med., 11.","DOI":"10.3390\/jcm11226791"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"973","DOI":"10.2147\/COPD.S127336","article-title":"Measures of Bronchodilator Response of FEV1, FVC and SVC in a Swedish General Population Sample Aged 50-64 Years, the SCAPIS Pilot Study","volume":"12","author":"Bake","year":"2017","journal-title":"Int. J. Chronic Obstr. Pulm. Dis."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"718","DOI":"10.1136\/thoraxjnl-2011-201445","article-title":"Worldwide Patterns of Bronchodilator Responsiveness: Results from the Burden of Obstructive Lung Disease Study","volume":"67","author":"Tan","year":"2012","journal-title":"Thorax"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"e2449795","DOI":"10.1001\/jamanetworkopen.2024.49795","article-title":"Asthma Among Children With Primary Ciliary Dyskinesia","volume":"7","author":"Zein","year":"2024","journal-title":"JAMA Netw. Open"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"429","DOI":"10.1136\/thx.47.6.429","article-title":"Interpretation of Bronchodilator Response in Patients with Obstructive Airways Disease. The Dutch Chronic Non-Specific Lung Disease (CNSLD) Study Group","volume":"47","author":"Brand","year":"1992","journal-title":"Thorax"},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"367","DOI":"10.1081\/JAS-200062992","article-title":"Acute Bronchodilator Response Has Limited Value in Differentiating Bronchial Asthma from COPD","volume":"42","author":"Chhabra","year":"2005","journal-title":"J. Asthma"},{"key":"ref_30","doi-asserted-by":"crossref","unstructured":"Hatic, E., F\u00e4rdig, M., Amaral, A.F.S., Jarvis, D., Accordini, S., Bousquet, J., Garcia-Aymerich, J., Tan, W.C., Zuberbier, T., and Janson, C. (2025). Evaluation of Recently Proposed Thresholds to Define Spirometry Bronchodilator Response. ERJ Open Res., 11.","DOI":"10.1183\/23120541.01162-2024"},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"2497179","DOI":"10.1080\/25310429.2025.2497179","article-title":"Bronchodilators in Bronchiectasis: A Story Difficult to Understand","volume":"31","author":"Oscullo","year":"2025","journal-title":"Pulmonology"}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/14\/13\/4778\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,9]],"date-time":"2025-10-09T18:05:33Z","timestamp":1760033133000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/14\/13\/4778"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,7,7]]},"references-count":31,"journal-issue":{"issue":"13","published-online":{"date-parts":[[2025,7]]}},"alternative-id":["jcm14134778"],"URL":"https:\/\/doi.org\/10.3390\/jcm14134778","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,7,7]]}}}