{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,7]],"date-time":"2026-08-07T15:25:05Z","timestamp":1786116305627,"version":"build-2736575974"},"reference-count":71,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2026,7,1]],"date-time":"2026-07-01T00:00:00Z","timestamp":1782864000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2026,7,1]],"date-time":"2026-07-01T00:00:00Z","timestamp":1782864000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2026,7]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>Primary ciliary dyskinesia (PCD) is a rare genetic disorder that causes chronic lung disease. Patients with PCD often experience pulmonary exacerbations with worsening symptoms and lung function decline. This review provides an overview of the existing knowledge regarding the PEx in PCD and reveals understudied topics that should be addressed in the future. We describe the different PCD\u2010specific PEx definitions that exist in the literature, and provide data on the frequency of PEx. Few studies targeted the pathophysiology of PEx, with a focus on the most common microorganisms in the airways and on inflammatory markers that are increased in the sputum (total cells, neutrophils, neutrophil elastase, interleukin\u20108). Several risk factors for PEx have been identified, among them female sex, age, and airway infection with Pseudomonas aeruginosa. Lung clearance index appears to be a good prognostic factor for future PEx. Current treatment and prevention strategies are described. Further research is needed to enhance our knowledge on PEx mechanisms, on appropriate treatment therapies, and on the long\u2010term consequences of this condition.<\/jats:p>","DOI":"10.1002\/ppul.71710","type":"journal-article","created":{"date-parts":[[2026,7,1]],"date-time":"2026-07-01T12:01:14Z","timestamp":1782907274000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Pulmonary Exacerbations and Treatment in PCD: A Narrative Review"],"prefix":"10.1002","volume":"61","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2597-8016","authenticated-orcid":false,"given":"Pinelopi","family":"Anagnostopoulou","sequence":"first","affiliation":[{"name":"Medical School University of Cyprus Nicosia Cyprus"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephanie","family":"Thee","sequence":"additional","affiliation":[{"name":"Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine Charit\u00e9 \u2010 Universit\u00e4tsmedizin Berlin Berlin Germany"},{"name":"Berlin Institute of Health at Charit\u00e9 \u2010 Universit\u00e4tsmedizin Berlin Berlin Germany"},{"name":"German Centre for Child and Adolescent Health (DZKJ) partner site Berlin Berlin Germany"},{"name":"German Centre for Lung Research (DZL) associated partner Berlin Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0511-5352","authenticated-orcid":false,"given":"Panayiotis","family":"Kouis","sequence":"additional","affiliation":[{"name":"Medical School University of Cyprus Nicosia Cyprus"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8339-9285","authenticated-orcid":false,"given":"Panayiotis","family":"Yiallouros","sequence":"additional","affiliation":[{"name":"Medical School University of Cyprus Nicosia Cyprus"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,7,1]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.23304"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(21)00453-7"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra1813799"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01090-2016"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00736-2016"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1569-1993(11)60012-X"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(20)30058-8"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01390-2015"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.23479"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.26599"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1136\/thoraxjnl-2020-215504"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmjresp-2022-001236"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01770-2016"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1183\/23120541.00147-2018"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1183\/23120541.00686-2025"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.3389\/fmolb.2026.1767533"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.25099"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12887-020-02062-4"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.3390\/ijerph17218099"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.05.00142704"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1136\/thx.2010.136119"},{"key":"e_1_2_8_23_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00051-2017"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01769-2023"},{"key":"e_1_2_8_25_1","doi-asserted-by":"publisher","DOI":"10.15326\/jcopdf.6.2.2018.0156"},{"key":"e_1_2_8_26_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.chest.2024.05.023"},{"key":"e_1_2_8_27_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.c.31967"},{"key":"e_1_2_8_28_1","doi-asserted-by":"publisher","DOI":"10.3389\/fped.2025.1564156"},{"key":"e_1_2_8_29_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cmi.2015.08.020"},{"key":"e_1_2_8_30_1","doi-asserted-by":"publisher","DOI":"10.1136\/thoraxjnl-2015-207891"},{"key":"e_1_2_8_31_1","doi-asserted-by":"publisher","DOI":"10.1128\/spectrum02213-23"},{"key":"e_1_2_8_32_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.25945"},{"key":"e_1_2_8_33_1","doi-asserted-by":"publisher","DOI":"10.1136\/bcr-2015-211493"},{"key":"e_1_2_8_34_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.chest.2024.06.3843"},{"key":"e_1_2_8_35_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmed.2017.02.001"},{"key":"e_1_2_8_36_1","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.202204-314OC"},{"key":"e_1_2_8_37_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2022.01.040"},{"key":"e_1_2_8_38_1","doi-asserted-by":"publisher","DOI":"10.1002\/lipd.12230"},{"key":"e_1_2_8_39_1","doi-asserted-by":"publisher","DOI":"10.1136\/thx.2011.161117"},{"key":"e_1_2_8_40_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13293-024-00633-z"},{"key":"e_1_2_8_41_1","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.202403-230OC"},{"key":"e_1_2_8_42_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01659-2017"},{"key":"e_1_2_8_43_1","doi-asserted-by":"publisher","DOI":"10.1183\/23120541.00253-2024"},{"key":"e_1_2_8_44_1","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.202404-340OC"},{"key":"e_1_2_8_45_1","doi-asserted-by":"publisher","DOI":"10.1183\/23120541.00218-2024"},{"key":"e_1_2_8_46_1","doi-asserted-by":"publisher","DOI":"10.1183\/16000617.0002-2025"},{"key":"e_1_2_8_47_1","doi-asserted-by":"publisher","DOI":"10.3390\/jcm13123372"},{"key":"e_1_2_8_48_1","doi-asserted-by":"publisher","DOI":"10.1186\/s40248-018-0139-2"},{"key":"e_1_2_8_49_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00300-2022"},{"key":"e_1_2_8_50_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199409083311003"},{"key":"e_1_2_8_51_1","doi-asserted-by":"publisher","DOI":"10.3389\/fped.2022.1084313"},{"key":"e_1_2_8_52_1","doi-asserted-by":"publisher","DOI":"10.1183\/23120541.01105-2025"},{"key":"e_1_2_8_53_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00431-026-06923-5"},{"key":"e_1_2_8_54_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00176608"},{"key":"e_1_2_8_55_1","doi-asserted-by":"publisher","DOI":"10.3390\/medsci14010133"},{"key":"e_1_2_8_56_1","doi-asserted-by":"publisher","DOI":"10.1080\/17476348.2017.1360770"},{"key":"e_1_2_8_57_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200812-1845PP"},{"key":"e_1_2_8_58_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa2510095"},{"key":"e_1_2_8_59_1","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.154.2.8756815"},{"key":"e_1_2_8_60_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.113.5.1329"},{"key":"e_1_2_8_61_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2018.02.006"},{"key":"e_1_2_8_62_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01126-2025"},{"key":"e_1_2_8_63_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.202102-0461OC"},{"key":"e_1_2_8_64_1","doi-asserted-by":"publisher","DOI":"10.1177\/1479972318787919"},{"key":"e_1_2_8_65_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.71018"},{"key":"e_1_2_8_66_1","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.202407-771OC"},{"key":"e_1_2_8_67_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.02278-2023"},{"key":"e_1_2_8_68_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11136-017-1564-y"},{"key":"e_1_2_8_69_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa2411664"},{"key":"e_1_2_8_70_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01551-2024"},{"key":"e_1_2_8_71_1","doi-asserted-by":"publisher","DOI":"10.1002\/alr.21873"},{"key":"e_1_2_8_72_1","doi-asserted-by":"publisher","DOI":"10.1183\/23120541.00026-2024"}],"container-title":["Pediatric Pulmonology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.71710","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/ppul.71710","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.71710","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,6]],"date-time":"2026-08-06T09:23:56Z","timestamp":1786008236000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ppul.71710"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,7]]},"references-count":71,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2026,7]]}},"alternative-id":["10.1002\/ppul.71710"],"URL":"https:\/\/doi.org\/10.1002\/ppul.71710","archive":["Portico"],"relation":{},"ISSN":["8755-6863","1099-0496"],"issn-type":[{"value":"8755-6863","type":"print"},{"value":"1099-0496","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,7]]},"assertion":[{"value":"2025-12-09","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-06-13","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-07-01","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e71710"}}