{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,22]],"date-time":"2026-07-22T14:01:55Z","timestamp":1784728915130,"version":"3.55.0"},"reference-count":114,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2026,5,27]],"date-time":"2026-05-27T00:00:00Z","timestamp":1779840000000},"content-version":"vor","delay-in-days":26,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T00:00:00Z","timestamp":1777593600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100005188","name":"AAIR Charity","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100005188","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100012357","name":"LifeArc","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100012357","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2026,5]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>Primary ciliary dyskinesia (PCD) is a genetically heterogeneous disorder resulting in defective ciliary motility, which leads to a spectrum of clinical manifestations, including chronic upper and lower respiratory infections, middle ear disease, situs abnormalities, congenital heart defects, and infertility. The development of standardized management plans for pediatric patients with PCD is hindered by the lack of high\u2010quality evidence regarding treatment and follow\u2010up protocols, alongside significant inter\u2010individual variability in disease severity and presentation. Moreover, the diagnosis of PCD is often delayed or missed, limiting timely access to specialized care. This review provides an analysis of the current literature on the follow\u2010up of children with PCD. We reflect on the importance of integrated clinical services and consider the role of PCD services to drive research. We review how patients should be monitored during follow\u2010up, particularly for upper and lower airway disease. We then discuss the importance of educating patients, families, and schools about the condition. We emphasize emerging consensus guidelines for patient monitoring and treatment, and we discuss resources that can support clinical management to improve patient outcomes.<\/jats:p>","DOI":"10.1002\/ppul.71679","type":"journal-article","created":{"date-parts":[[2026,5,27]],"date-time":"2026-05-27T07:35:36Z","timestamp":1779867336000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Follow\u2010Up of Children With Primary Ciliary Dyskinesia"],"prefix":"10.1002","volume":"61","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1629-8601","authenticated-orcid":false,"given":"Bruna","family":"Rubbo","sequence":"first","affiliation":[{"name":"School of Clinical and Experimental Sciences, University of Southampton Faculty of Medicine Southampton UK"},{"name":"Primary Ciliary Dyskinesia Centre, NIHR Southampton Biomedical Research Centre, University Hospital Southampton NHS Foundation Trust Southampton UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathan Wen Yi","family":"Ong","sequence":"additional","affiliation":[{"name":"School of Clinical and Experimental Sciences, University of Southampton Faculty of Medicine Southampton UK"},{"name":"Primary Ciliary Dyskinesia Centre, NIHR Southampton Biomedical Research Centre, University Hospital Southampton NHS Foundation Trust Southampton UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William","family":"Tsang","sequence":"additional","affiliation":[{"name":"School of Clinical and Experimental Sciences, University of Southampton Faculty of Medicine Southampton UK"},{"name":"Primary Ciliary Dyskinesia Centre, NIHR Southampton Biomedical Research Centre, University Hospital Southampton NHS Foundation Trust Southampton UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lydia M.","family":"Newman","sequence":"additional","affiliation":[{"name":"School of Human Development and Health, University of Southampton Southampton UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8701-9975","authenticated-orcid":false,"given":"Jane S.","family":"Lucas","sequence":"additional","affiliation":[{"name":"School of Clinical and Experimental Sciences, University of Southampton Faculty of Medicine Southampton UK"},{"name":"Primary Ciliary Dyskinesia Centre, NIHR Southampton Biomedical Research Centre, University Hospital Southampton NHS Foundation Trust Southampton UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,5,27]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(19)30374-1"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41572-020-0209-6"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(21)00453-7"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00736-2016"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1093\/humupd\/dmad003"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1136\/thoraxjnl-2018-212104"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2019.09.022"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.02359-2020"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00073911"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.3390\/jcm11164774"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ccm.2021.03.013"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1136\/archdischild-2013-304831"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1183\/16000617.0023-2017"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.23304"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00176608"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1080\/17476348.2017.1360770"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1183\/23120541.00466-2025"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.prrv.2021.02.001"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12931-024-02735-z"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1183\/23120541.01115-2024"},{"key":"e_1_2_6_22_1","doi-asserted-by":"publisher","DOI":"10.1183\/16000617.0248-2022"},{"key":"e_1_2_6_23_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00085-2017"},{"key":"e_1_2_6_24_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.04601-2020"},{"key":"e_1_2_6_25_1","doi-asserted-by":"publisher","DOI":"10.1183\/23120541.00714-2022"},{"key":"e_1_2_6_26_1","doi-asserted-by":"publisher","DOI":"10.1183\/16000617.0058-2016"},{"key":"e_1_2_6_27_1","doi-asserted-by":"publisher","DOI":"10.4103\/lungindia.lungindia_417_25"},{"key":"e_1_2_6_28_1","doi-asserted-by":"publisher","DOI":"10.3389\/fmolb.2025.1547152"},{"key":"e_1_2_6_29_1","unstructured":"Department of Health and Social Care. The UK Rare Diseases Framework. 2021."},{"key":"e_1_2_6_30_1","doi-asserted-by":"publisher","DOI":"10.1136\/archdischild-2019-317687"},{"key":"e_1_2_6_31_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.26303"},{"key":"e_1_2_6_32_1","doi-asserted-by":"publisher","DOI":"10.1183\/23120541.00237-2019"},{"key":"e_1_2_6_33_1","doi-asserted-by":"publisher","DOI":"10.1183\/23120541.00843-2020"},{"key":"e_1_2_6_34_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01769-2023"},{"key":"e_1_2_6_35_1","doi-asserted-by":"publisher","DOI":"10.1183\/23120541.00301-2021"},{"key":"e_1_2_6_36_1","doi-asserted-by":"publisher","DOI":"10.1183\/23120541.01071-2024"},{"key":"e_1_2_6_37_1","doi-asserted-by":"publisher","DOI":"10.1183\/23120541.00115-2023"},{"key":"e_1_2_6_38_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11136-017-1564-y"},{"key":"e_1_2_6_39_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01918-2021"},{"key":"e_1_2_6_40_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.chest.2020.02.001"},{"key":"e_1_2_6_41_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201803-0548OC"},{"key":"e_1_2_6_42_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200811-1731OC"},{"key":"e_1_2_6_43_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.22577"},{"key":"e_1_2_6_44_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.healun.2016.08.025"},{"key":"e_1_2_6_45_1","doi-asserted-by":"publisher","DOI":"10.3389\/ti.2023.10819"},{"key":"e_1_2_6_46_1","doi-asserted-by":"publisher","DOI":"10.1183\/2312508X.10016717"},{"key":"e_1_2_6_47_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.02990-2020"},{"key":"e_1_2_6_48_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2023.12.002"},{"key":"e_1_2_6_49_1","doi-asserted-by":"publisher","DOI":"10.1183\/20734735.0021711"},{"key":"e_1_2_6_50_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201908-1590ST"},{"key":"e_1_2_6_51_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01499-2021"},{"key":"e_1_2_6_52_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmjresp-2020-000575"},{"key":"e_1_2_6_53_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.21569"},{"key":"e_1_2_6_54_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01040-2018"},{"key":"e_1_2_6_55_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201409-1672OC"},{"key":"e_1_2_6_56_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00209-2016"},{"key":"e_1_2_6_57_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.22984"},{"key":"e_1_2_6_58_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01659-2017"},{"key":"e_1_2_6_59_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.26213"},{"key":"e_1_2_6_60_1","doi-asserted-by":"publisher","DOI":"10.1183\/16000617.0002-2025"},{"key":"e_1_2_6_61_1","doi-asserted-by":"publisher","DOI":"10.1183\/23120541.00408-2020"},{"key":"e_1_2_6_62_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00524-2024"},{"key":"e_1_2_6_63_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.24249"},{"key":"e_1_2_6_64_1","doi-asserted-by":"publisher","DOI":"10.2214\/AJR.06.0965"},{"key":"e_1_2_6_65_1","doi-asserted-by":"publisher","DOI":"10.1136\/thoraxjnl-2014-206578"},{"key":"e_1_2_6_66_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.202402-0411OC"},{"key":"e_1_2_6_67_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.26709"},{"key":"e_1_2_6_68_1","doi-asserted-by":"publisher","DOI":"10.1159\/000514927"},{"key":"e_1_2_6_69_1","doi-asserted-by":"publisher","DOI":"10.1183\/23120541.00282-2024"},{"key":"e_1_2_6_70_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cmi.2015.08.020"},{"key":"e_1_2_6_71_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmed.2019.08.009"},{"key":"e_1_2_6_72_1","doi-asserted-by":"publisher","DOI":"10.1183\/23120541.00253-2024"},{"key":"e_1_2_6_73_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmed.2017.08.028"},{"key":"e_1_2_6_74_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12887-020-02062-4"},{"key":"e_1_2_6_75_1","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.202210-858OC"},{"key":"e_1_2_6_76_1","doi-asserted-by":"publisher","DOI":"10.1183\/23120541.00147-2018"},{"issue":"63","key":"e_1_2_6_77_1","article-title":"A Review of the Experiences of Children and Young Adults With Primary Ciliary Dyskinesia (PCD) Treated in a Specialist PCD Service in England","volume":"54","author":"Wilkins H.","year":"2019","journal-title":"ERJ"},{"key":"e_1_2_6_78_1","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.202407-771OC"},{"key":"e_1_2_6_79_1","doi-asserted-by":"publisher","DOI":"10.1089\/ped.2016.0718"},{"key":"e_1_2_6_80_1","doi-asserted-by":"publisher","DOI":"10.1183\/23120541.00701-2022"},{"key":"e_1_2_6_81_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01551-2015"},{"key":"e_1_2_6_82_1","doi-asserted-by":"publisher","DOI":"10.1111\/cea.14592"},{"key":"e_1_2_6_83_1","doi-asserted-by":"publisher","DOI":"10.1002\/alr.23180"},{"key":"e_1_2_6_84_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.otc.2022.09.010"},{"key":"e_1_2_6_85_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2004.10.041"},{"key":"e_1_2_6_86_1","doi-asserted-by":"publisher","DOI":"10.1183\/23120541.00932-2023"},{"key":"e_1_2_6_87_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00405-010-1341-9"},{"key":"e_1_2_6_88_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijporl.2016.08.011"},{"key":"e_1_2_6_89_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamaoto.2023.0841"},{"key":"e_1_2_6_90_1","doi-asserted-by":"publisher","DOI":"10.1097\/AUD.0000000000000219"},{"key":"e_1_2_6_91_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijporl.2023.111801"},{"key":"e_1_2_6_92_1","doi-asserted-by":"publisher","DOI":"10.3390\/jcm8091495"},{"key":"e_1_2_6_93_1","doi-asserted-by":"publisher","DOI":"10.1002\/lary.24654"},{"key":"e_1_2_6_94_1","doi-asserted-by":"publisher","DOI":"10.1002\/alr.21873"},{"key":"e_1_2_6_95_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.24507"},{"key":"e_1_2_6_96_1","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.201603-198OC"},{"key":"e_1_2_6_97_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.anai.2013.06.033"},{"key":"e_1_2_6_98_1","doi-asserted-by":"publisher","DOI":"10.1183\/23120541.00218-2024"},{"key":"e_1_2_6_99_1","doi-asserted-by":"publisher","DOI":"10.1177\/01945998211019320"},{"key":"e_1_2_6_100_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0042-1746193"},{"key":"e_1_2_6_101_1","doi-asserted-by":"publisher","DOI":"10.1053\/j.jvca.2023.01.033"},{"key":"e_1_2_6_102_1","doi-asserted-by":"publisher","DOI":"10.1002\/alr.23303"},{"key":"e_1_2_6_103_1","doi-asserted-by":"publisher","DOI":"10.1038\/ncomms5418"},{"key":"e_1_2_6_104_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng.2961"},{"key":"e_1_2_6_105_1","doi-asserted-by":"publisher","DOI":"10.1136\/thoraxjnl-2016-209356"},{"key":"e_1_2_6_106_1","doi-asserted-by":"publisher","DOI":"10.1093\/hropen\/hoae039"},{"key":"e_1_2_6_107_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00314-2017"},{"key":"e_1_2_6_108_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.02018-2015"},{"key":"e_1_2_6_109_1","doi-asserted-by":"publisher","DOI":"10.1177\/13591053231223912"},{"key":"e_1_2_6_110_1","doi-asserted-by":"publisher","DOI":"10.7748\/ncyp.2017.e936"},{"key":"e_1_2_6_111_1","doi-asserted-by":"publisher","DOI":"10.1136\/archdischild-2014-307423"},{"issue":"65","key":"e_1_2_6_112_1","article-title":"Service Delivery Models for Primary Ciliary Dyskinesia, an International Comparison","volume":"58","author":"Rubbo B.","year":"2021","journal-title":"European Respiratory Journal"},{"key":"e_1_2_6_113_1","doi-asserted-by":"publisher","DOI":"10.1183\/23120541.01624-2025"},{"key":"e_1_2_6_114_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pdig.0000401"},{"key":"e_1_2_6_115_1","doi-asserted-by":"publisher","DOI":"10.3389\/fpubh.2023.1109323"}],"container-title":["Pediatric Pulmonology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.71679","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/ppul.71679","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.71679","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,22]],"date-time":"2026-07-22T13:10:15Z","timestamp":1784725815000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ppul.71679"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,5]]},"references-count":114,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2026,5]]}},"alternative-id":["10.1002\/ppul.71679"],"URL":"https:\/\/doi.org\/10.1002\/ppul.71679","archive":["Portico"],"relation":{},"ISSN":["8755-6863","1099-0496"],"issn-type":[{"value":"8755-6863","type":"print"},{"value":"1099-0496","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,5]]},"assertion":[{"value":"2025-11-28","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-05-19","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-05-27","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e71679"}}