{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,27]],"date-time":"2026-03-27T17:28:24Z","timestamp":1774632504113,"version":"3.50.1"},"reference-count":43,"publisher":"European Respiratory Society (ERS)","issue":"1","funder":[{"name":"COST Action","award":["BM 1407"],"award-info":[{"award-number":["BM 1407"]}]}],"content-domain":{"domain":["publications.ersnet.org"],"crossmark-restriction":true},"short-container-title":["ERJ Open Res"],"accepted":{"date-parts":[[2018,12,5]]},"published-print":{"date-parts":[[2019,2]]},"abstract":"<jats:p>Pulmonary exacerbations are a cause of significant morbidity in patients with primary ciliary dyskinesia (PCD) and are frequently used as an outcome measure in clinical research into chronic lung diseases. So far, there has been no consensus on the definition of pulmonary exacerbations in PCD.<\/jats:p><jats:p>30 multidisciplinary experts and patients developed a consensus definition for children and adults with PCD. Following a systematic review, the panel used a modified Delphi process with a combination of face-to-face meetings and e-surveys to develop a definition that can be used in research settings for children and adults with PCD.<\/jats:p><jats:p>A pulmonary exacerbation was defined by the presence of three or more of the following seven items: 1) increased cough, 2) change in sputum volume and\/or colour, 3) increased shortness of breath perceived by the patient or parent, 4) decision to start or change antibiotic treatment because of perceived pulmonary symptoms, 5) malaise, tiredness, fatigue or lethargy, 6) new or increased haemoptysis, and 7) temperature &gt;38\u00b0C.<\/jats:p><jats:p>The consensus panel proposed that the definition should be used for future clinical trials. The definition should be validated and the usability assessed during these studies.<\/jats:p>","DOI":"10.1183\/23120541.00147-2018","type":"journal-article","created":{"date-parts":[[2019,2,1]],"date-time":"2019-02-01T14:00:43Z","timestamp":1549029643000},"page":"00147-2018","update-policy":"https:\/\/doi.org\/10.1183\/ers-crossmark-policy","source":"Crossref","is-referenced-by-count":59,"title":["Pulmonary exacerbations in patients with primary ciliary dyskinesia: an expert consensus definition for use in clinical trials"],"prefix":"10.1183","volume":"5","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8701-9975","authenticated-orcid":false,"given":"Jane S.","family":"Lucas","sequence":"first","affiliation":[]},{"given":"Florian","family":"Gahleitner","sequence":"additional","affiliation":[]},{"given":"Adelina","family":"Amorim","sequence":"additional","affiliation":[]},{"given":"Mieke","family":"Boon","sequence":"additional","affiliation":[]},{"given":"Philippa","family":"Brown","sequence":"additional","affiliation":[]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6596-1596","authenticated-orcid":false,"given":"Carolina","family":"Constant","sequence":"additional","affiliation":[]},{"given":"Simon","family":"Cook","sequence":"additional","affiliation":[]},{"given":"Suzanne","family":"Crowley","sequence":"additional","affiliation":[]},{"given":"Damien M.S.","family":"Destouches","sequence":"additional","affiliation":[]},{"given":"Ernst","family":"Eber","sequence":"additional","affiliation":[]},{"given":"Huda","family":"Mussaffi","sequence":"additional","affiliation":[]},{"given":"Eric","family":"Haarman","sequence":"additional","affiliation":[]},{"given":"Amanda","family":"Harris","sequence":"additional","affiliation":[]},{"given":"Cordula","family":"Koerner-Rettberg","sequence":"additional","affiliation":[]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8957-2002","authenticated-orcid":false,"given":"Claudia E.","family":"Kuehni","sequence":"additional","affiliation":[]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5239-1571","authenticated-orcid":false,"given":"Philipp","family":"Latzin","sequence":"additional","affiliation":[]},{"given":"Michael R.","family":"Loebinger","sequence":"additional","affiliation":[]},{"given":"Natalie","family":"Lorent","sequence":"additional","affiliation":[]},{"given":"Bernard","family":"Maitre","sequence":"additional","affiliation":[]},{"given":"Antonio","family":"Moreno-Gald\u00f3","sequence":"additional","affiliation":[]},{"given":"Kim G.","family":"Nielsen","sequence":"additional","affiliation":[]},{"given":"U\u011fur","family":"\u00d6z\u00e7elik","sequence":"additional","affiliation":[]},{"given":"Lue Katrine Drasb\u00e6k","family":"Philipsen","sequence":"additional","affiliation":[]},{"given":"Petr","family":"Pohunek","sequence":"additional","affiliation":[]},{"given":"Eva","family":"Polverino","sequence":"additional","affiliation":[]},{"given":"Jessica","family":"Rademacher","sequence":"additional","affiliation":[]},{"given":"Phil","family":"Robinson","sequence":"additional","affiliation":[]},{"given":"Deborah","family":"Snijders","sequence":"additional","affiliation":[]},{"given":"Panayiotis","family":"Yiallouros","sequence":"additional","affiliation":[]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0580-2478","authenticated-orcid":false,"given":"Siobh\u00e1n B.","family":"Carr","sequence":"additional","affiliation":[]}],"member":"81","published-online":{"date-parts":[[2019,2,1]]},"reference":[{"key":"2024101707153954000_5.1.00147-2018.1","doi-asserted-by":"crossref","unstructured":"Lucas JS , Walker WT , Kuehni CE , et al. Primary ciliary dyskinesia. In: Cordier J-F , ed. Orphan Lung Diseases (ERS Monograph) . Sheffield, European Respiratory Society, 2011; pp. 1\u201319.","DOI":"10.1183\/1025448x.10008310"},{"key":"2024101707153954000_5.1.00147-2018.2","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00001010"},{"key":"2024101707153954000_5.1.00147-2018.3","doi-asserted-by":"publisher","DOI":"10.1136\/adc.2009.158493"},{"key":"2024101707153954000_5.1.00147-2018.4","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2012.11.002"},{"key":"2024101707153954000_5.1.00147-2018.5","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01090-2016"},{"key":"2024101707153954000_5.1.00147-2018.6","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01551-2015"},{"key":"2024101707153954000_5.1.00147-2018.7","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.02018-2015"},{"key":"2024101707153954000_5.1.00147-2018.8","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00175614"},{"key":"2024101707153954000_5.1.00147-2018.9","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00736-2016"},{"key":"2024101707153954000_5.1.00147-2018.10","doi-asserted-by":"crossref","first-page":"1093","DOI":"10.1016\/j.cmi.2015.08.020","article-title":"A longitudinal study of lung bacterial pathogens in patients with primary ciliary dyskinesia","volume":"21","author":"Alanin","year":"2015","journal-title":"Clin Microbiol Infect"},{"key":"2024101707153954000_5.1.00147-2018.11","doi-asserted-by":"crossref","first-page":"779","DOI":"10.1080\/17476348.2017.1360770","article-title":"Clinical care of children with primary ciliary dyskinesia","volume":"11","author":"Lucas","year":"2017","journal-title":"Expert Rev Respir Med"},{"key":"2024101707153954000_5.1.00147-2018.12","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00612-2017"},{"key":"2024101707153954000_5.1.00147-2018.13","doi-asserted-by":"crossref","first-page":"2265","DOI":"10.1007\/s11136-017-1564-y","article-title":"The patient's experience of primary ciliary dyskinesia: a systematic review","volume":"26","author":"Behan","year":"2017","journal-title":"Qual Life Res"},{"key":"2024101707153954000_5.1.00147-2018.14","doi-asserted-by":"publisher","DOI":"10.3109\/09593985.2013.845863"},{"key":"2024101707153954000_5.1.00147-2018.15","doi-asserted-by":"crossref","first-page":"1362","DOI":"10.1002\/ppul.23479","article-title":"Recovery of baseline lung function after pulmonary exacerbation in children with primary ciliary dyskinesia","volume":"51","author":"Sunther","year":"2016","journal-title":"Ped Pulm"},{"key":"2024101707153954000_5.1.00147-2018.16","doi-asserted-by":"crossref","first-page":"104","DOI":"10.1186\/s12890-016-0261-x","article-title":"Study protocol, rationale and recruitment in a European multi-centre randomized controlled trial to determine the efficacy and safety of azithromycin maintenance therapy for 6\u2005months in primary ciliary dyskinesia","volume":"16","author":"Kobbernagel","year":"2016","journal-title":"BMC Pulm Med"},{"key":"2024101707153954000_5.1.00147-2018.17","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01390-2015"},{"key":"2024101707153954000_5.1.00147-2018.18","doi-asserted-by":"publisher","DOI":"10.1378\/chest.13-2590"},{"key":"2024101707153954000_5.1.00147-2018.19","doi-asserted-by":"publisher","DOI":"10.1183\/16000617.0023-2017"},{"issue":"Suppl. 2","key":"2024101707153954000_5.1.00147-2018.20","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1186\/s12919-018-0098-9","article-title":"Proceedings of the 2nd BEAT-PCD conference and 3rd PCD training school: part 1","volume":"12","author":"Halbeisen","year":"2018","journal-title":"BMC Proc"},{"key":"2024101707153954000_5.1.00147-2018.21","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01770-2016"},{"key":"2024101707153954000_5.1.00147-2018.22","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199409083311003"},{"key":"2024101707153954000_5.1.00147-2018.23","doi-asserted-by":"publisher","DOI":"10.1016\/S0149-2918(99)80035-6"},{"key":"2024101707153954000_5.1.00147-2018.24","doi-asserted-by":"publisher","DOI":"10.1067\/mpd.2001.117288"},{"key":"2024101707153954000_5.1.00147-2018.25","doi-asserted-by":"crossref","first-page":"400","DOI":"10.1002\/ppul.20023","article-title":"Pulmonary exacerbations in cystic fibrosis","volume":"37","author":"Rabin","year":"2004","journal-title":"Ped Pulm"},{"key":"2024101707153954000_5.1.00147-2018.26","doi-asserted-by":"publisher","DOI":"10.1016\/j.cct.2009.01.003"},{"key":"2024101707153954000_5.1.00147-2018.27","doi-asserted-by":"publisher","DOI":"10.1001\/jama.2010.563"},{"issue":"Suppl. 2","key":"2024101707153954000_5.1.00147-2018.28","doi-asserted-by":"crossref","first-page":"S79","DOI":"10.1016\/S1569-1993(11)60012-X","article-title":"Pulmonary exacerbation: towards a definition for use in clinical trials. Report from the EuroCareCF Working Group on outcome parameters in clinical trials","volume":"10","author":"Bilton","year":"2011","journal-title":"J Cyst Fibros"},{"key":"2024101707153954000_5.1.00147-2018.29","doi-asserted-by":"crossref","first-page":"2269","DOI":"10.1001\/jama.2012.5214","article-title":"Inhaled hypertonic saline in infants and children younger than 6\u2005years with cystic fibrosis: the ISIS randomized controlled trial","volume":"307","author":"Rosenfeld","year":"2012","journal-title":"JAMA"},{"key":"2024101707153954000_5.1.00147-2018.30","doi-asserted-by":"crossref","first-page":"68","DOI":"10.1002\/ppul.21518","article-title":"Defining pulmonary exacerbation in children with non-cystic fibrosis bronchiectasis","volume":"47","author":"Kapur","year":"2012","journal-title":"Ped Pulm"},{"key":"2024101707153954000_5.1.00147-2018.31","doi-asserted-by":"crossref","first-page":"1144","DOI":"10.1164\/rccm.201610-2172OC","article-title":"Home monitoring of patients with cystic fibrosis to identify and treat acute pulmonary exacerbations. eICE study results","volume":"196","author":"Lechtzin","year":"2017","journal-title":"Am J Respir Crit Care Med"},{"key":"2024101707153954000_5.1.00147-2018.32","doi-asserted-by":"publisher","DOI":"10.1016\/j.cct.2013.09.004"},{"issue":"Suppl. 2","key":"2024101707153954000_5.1.00147-2018.33","doi-asserted-by":"crossref","first-page":"S200","DOI":"10.1513\/AnnalsATS.201502-098AW","article-title":"Pulmonary exacerbations in children with cystic fibrosis","volume":"12","author":"Waters","year":"2015","journal-title":"Ann Am Thorac Soc"},{"key":"2024101707153954000_5.1.00147-2018.34","doi-asserted-by":"publisher","DOI":"10.1186\/s12931-015-0167-9"},{"key":"2024101707153954000_5.1.00147-2018.35","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00051-2017"},{"key":"2024101707153954000_5.1.00147-2018.36","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00776-2015"},{"key":"2024101707153954000_5.1.00147-2018.37","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01181-2016"},{"key":"2024101707153954000_5.1.00147-2018.38","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01040-2018"},{"key":"2024101707153954000_5.1.00147-2018.39","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01659-2017"},{"key":"2024101707153954000_5.1.00147-2018.40","doi-asserted-by":"publisher","DOI":"10.1136\/thoraxjnl-2016-209356"},{"key":"2024101707153954000_5.1.00147-2018.41","first-page":"1726","article-title":"Primary ciliary dyskinesia: first health-related quality-of-life measures for pediatric patients","volume":"13","author":"Dell","year":"2016","journal-title":"Ann Am Thorac Soc"},{"key":"2024101707153954000_5.1.00147-2018.42","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00216214"},{"key":"2024101707153954000_5.1.00147-2018.43","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1186\/s12919-016-0067-0","article-title":"Proceedings of the COST action BM1407 inaugural conference BEAT-PCD: translational research in primary ciliary dyskinesia \u2013 bench, bedside, and population perspectives","volume":"10","author":"Rubbo","year":"2016","journal-title":"BMC Proc"}],"container-title":["ERJ Open Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1183\/23120541.00147-2018","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,2,24]],"date-time":"2025-02-24T04:57:06Z","timestamp":1740373026000},"score":1,"resource":{"primary":{"URL":"https:\/\/publications.ersnet.org\/lookup\/doi\/10.1183\/23120541.00147-2018"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,2]]},"references-count":43,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2019,2,1]]},"published-print":{"date-parts":[[2019,2]]}},"alternative-id":["10.1183\/23120541.00147-2018"],"URL":"https:\/\/doi.org\/10.1183\/23120541.00147-2018","relation":{},"ISSN":["2312-0541"],"issn-type":[{"value":"2312-0541","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,2]]}}}