{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,7]],"date-time":"2026-09-07T22:31:48Z","timestamp":1788820308613,"version":"build-2803163510"},"reference-count":30,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2024,11,22]],"date-time":"2024-11-22T00:00:00Z","timestamp":1732233600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2024,11,22]],"date-time":"2024-11-22T00:00:00Z","timestamp":1732233600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/100006108","name":"National Center for Advancing Translational Sciences","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100006108","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2025,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>Primary ciliary dyskinesia (PCD) management has not been systematically evaluated and is largely empirical.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Pediatric participants with PCD were enrolled in a prospective, longitudinal, multicenter, observational study. Therapies were recorded at annual visits and categorized by type. Age\u2010related trends in prevalence of therapies were described by serial cross\u2010sectional analyses. Generalized estimating equations analyzed covariates affecting prevalence of certain therapies and whether these covariates impacted oral antibiotic courses.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      A total of 137 participants completed 897 visits over 13 years. All but one received \u2265\u20091 antibiotic courses during study participation, most often cephalosporins (74%) or amoxicillin\u2010clavulanate (73%). Thirty\u2010one percent reported chronic azithromycin use. Per participant, there was an average of 2.3 (SD\u2009=\u20092.2) oral antibiotic courses annually. The rate of reported antibiotic courses at the 6 United States sites was 2.6 times higher compared to the Canadian site (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001). As patients got older, they were more likely to report use of amoxicillin\u2010clavulanate (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001), chronic azithromycin (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001), fluroquinolones (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001), inhaled steroids with long\u2010acting beta\u2010agonists (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.010), and hypertonic saline (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001). Compared to outer dynein arm defects, those with inner dynein arm\/microtubular disorganization defects reported increased use of chronic azithromycin (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.011) and inhaled steroids (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.015).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Discussion<\/jats:title>\n                    <jats:p>Older participants and those with inner dynein arm\/microtubular disorganization defects reported more therapies likely due to disease progression and more severe phenotypes, respectively. We report that a wide range of therapies are used in PCD without disease\u2010specific studies defining benefits and risks.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ppul.27412","type":"journal-article","created":{"date-parts":[[2024,11,22]],"date-time":"2024-11-22T05:41:12Z","timestamp":1732254072000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Therapies Used by Children With Primary Ciliary Dyskinesia: A Natural History Study"],"prefix":"10.1002","volume":"60","author":[{"given":"Robert A.","family":"Gardner","sequence":"first","affiliation":[{"name":"Department of Pediatrics Levine Children's Hospital, Wake Forest University School of Medicine Charlotte North Carolina USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas W.","family":"Ferkol","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of North Carolina at Chapel Hill Chapel Hill North Carolina USA"},{"name":"Marsico Lung Institute University of North Carolina at Chapel Hill Chapel Hill North Carolina USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephanie D.","family":"Davis","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of North Carolina at Chapel Hill Chapel Hill North Carolina USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margaret","family":"Rosenfeld","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Washington Seattle Washington State USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Scott D.","family":"Sagel","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Colorado Aurora Colorado USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sharon D.","family":"Dell","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of British Columbia Vancouver British Columbia Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlos E.","family":"Milla","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Stanford University Palo Alto California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lang","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Biostatistics University of North Carolina at Chapel Hill Chapel Hill North Carolina USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Feng\u2010Chang","family":"Lin","sequence":"additional","affiliation":[{"name":"Department of Biostatistics University of North Carolina at Chapel Hill Chapel Hill North Carolina USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kelli M.","family":"Sullivan","sequence":"additional","affiliation":[{"name":"Marsico Lung Institute University of North Carolina at Chapel Hill Chapel Hill North Carolina USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maimoona A.","family":"Zariwala","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of North Carolina at Chapel Hill Chapel Hill North Carolina USA"},{"name":"Marsico Lung Institute University of North Carolina at Chapel Hill Chapel Hill North Carolina USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael R.","family":"Knowles","sequence":"additional","affiliation":[{"name":"Marsico Lung Institute University of North Carolina at Chapel Hill Chapel Hill North Carolina USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margaret W.","family":"Leigh","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of North Carolina at Chapel Hill Chapel Hill North Carolina USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"for the Genetic Disorders of Mucociliary Clearance Consortium","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,11,22]]},"reference":[{"key":"e_1_2_10_2_1","first-page":"1993","volume-title":"Gene Reviews\u00ae","author":"Zariawala M. A."},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.1982P3"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201301-0059CI"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(20)30058-8"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01770-2016"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(23)00226-6"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.23304"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ccm.2016.04.008"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201803-0548OC"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201409-1672OC"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201805-0819ST"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00073911"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(12)60953-2"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1001\/jama.2013.1937"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1001\/jama.2010.563"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1001\/jama.290.13.1749"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.50.4727"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(13)70185-1"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.4187\/respcare.04086"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.prrv.2017.03.011"},{"key":"e_1_2_10_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmed.2007.05.014"},{"key":"e_1_2_10_23_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.1950200509"},{"key":"e_1_2_10_24_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1099-0496(199901)27:1<59::AID-PPUL11>3.0.CO;2-D"},{"key":"e_1_2_10_25_1","doi-asserted-by":"publisher","DOI":"10.3390\/ijms22189834"},{"issue":"29","key":"e_1_2_10_26_1","doi-asserted-by":"crossref","first-page":"1","DOI":"10.4193\/Rhin20.600","article-title":"European Position Paper on Rhinosinusitis and Nasal Polyps 2020","volume":"58","author":"Fokkens W. J.","year":"2020","journal-title":"Rhinology Journal"},{"key":"e_1_2_10_27_1","doi-asserted-by":"publisher","DOI":"10.1177\/0194599819874842"},{"key":"e_1_2_10_28_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00405-010-1341-9"},{"key":"e_1_2_10_29_1","doi-asserted-by":"publisher","DOI":"10.1590\/1984-0462\/2020\/38\/2018338"},{"key":"e_1_2_10_30_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12890-015-0038-7"},{"key":"e_1_2_10_31_1","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.202206-524OC"}],"container-title":["Pediatric Pulmonology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.27412","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/ppul.27412","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.27412","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,2,18]],"date-time":"2026-02-18T15:29:14Z","timestamp":1771428554000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ppul.27412"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,11,22]]},"references-count":30,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2025,1]]}},"alternative-id":["10.1002\/ppul.27412"],"URL":"https:\/\/doi.org\/10.1002\/ppul.27412","archive":["Portico"],"relation":{},"ISSN":["8755-6863","1099-0496"],"issn-type":[{"value":"8755-6863","type":"print"},{"value":"1099-0496","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,11,22]]},"assertion":[{"value":"2024-05-17","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-11-10","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-11-22","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e27412"}}