{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,7]],"date-time":"2026-09-07T22:31:44Z","timestamp":1788820304763,"version":"build-2803163510"},"reference-count":34,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2026,5,11]],"date-time":"2026-05-11T00:00:00Z","timestamp":1778457600000},"content-version":"vor","delay-in-days":10,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/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"}],"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:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Primary ciliary dyskinesia (PCD) is a genetically heterogeneous disorder causing chronic oto\u2010sino\u2010pulmonary disease from birth. Since diagnosis is often delayed into childhood or adulthood, early\u2010life disease burdens remain poorly described.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>This retrospective study analyzed disease burdens until 2 years of age and outcomes at 6\u20107 years of age in children diagnosed with PCD near birth at two Canadian PCD centers.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Thirty\u2010five infants (median diagnostic age 34 days) were included. All received daily chest physiotherapy, while 52% received daily inhaled hypertonic saline therapy (IHS) from diagnosis. By 2 years of age, 66% experienced outpatient respiratory exacerbations, 29% had inpatient respiratory exacerbations, 60% required emergency room visits for respiratory illness, and 14% had\n                      <jats:italic>Pseudomonas aeruginosa<\/jats:italic>\n                      in sputum cultures. Abnormal audiology occurred in 91% screened, and 49% required tympanostomy tubes. Despite early diagnosis and therapy, spirometry showed a trend towards obstruction (mean FEV\u2081\/FVC ratio Z\u2010score \u20101.43) by 7 years of age. Infants on daily IHS from birth (n\u2009=\u200918, mostly at one center) had significantly fewer\n                      <jats:italic>Pseudomonas aeruginosa<\/jats:italic>\n                      sputum isolates (0%\n                      <jats:italic>vs.<\/jats:italic>\n                      29%,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.02) but significantly increased inpatient respiratory exacerbations (44%\n                      <jats:italic>vs.<\/jats:italic>\n                      12%,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.04) compared to infants not on regular IHS (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200917).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>\n                      Despite early diagnosis, ongoing respiratory surveillance, and regular airway clearance, infants with PCD develop frequent respiratory infections and have high clinical care burdens. Infants have markedly increased risk of hearing loss, warranting universal screening and close follow\u2010up. Inhaled hypertonic saline may influence early\n                      <jats:italic>Pseudomonas<\/jats:italic>\n                      acquisition and hospitalization risk, warranting prospective study of this commonly prescribed therapy.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ppul.71658","type":"journal-article","created":{"date-parts":[[2026,5,11]],"date-time":"2026-05-11T12:59:56Z","timestamp":1778504396000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Early Life Disease Burden and Outcomes in Children Diagnosed With Primary Ciliary Dyskinesia in Infancy"],"prefix":"10.1002","volume":"61","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0435-8902","authenticated-orcid":false,"given":"Madhan","family":"Kumar","sequence":"first","affiliation":[{"name":"Montreal Children's Hospital McGill University Health Centre Montreal Quebec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8666-5115","authenticated-orcid":false,"given":"Dvir","family":"Gatt","sequence":"additional","affiliation":[{"name":"Pediatric Pulmonary Unit Soroka University Medical Center Be'er Sheva Israel"},{"name":"The Hospital for Sick Children University of Toronto Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zofia","family":"Zysman\u2010Colman","sequence":"additional","affiliation":[{"name":"Montreal Children's Hospital McGill University Health Centre Montreal Quebec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jos\u00e9","family":"Fontoura\u2010Matias","sequence":"additional","affiliation":[{"name":"The Hospital for Sick Children University of Toronto Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deborah","family":"Fertuck","sequence":"additional","affiliation":[{"name":"Montreal Children's Hospital McGill University Health Centre Montreal Quebec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sharon D.","family":"Dell","sequence":"additional","affiliation":[{"name":"BC Children's Hospital, Department of Pediatrics University of British Columbia Vancouver British Columbia Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0004-0155-7377","authenticated-orcid":false,"given":"Fiona E.","family":"Kritzinger","sequence":"additional","affiliation":[{"name":"The Hospital for Sick Children University of Toronto Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adam J.","family":"Shapiro","sequence":"additional","affiliation":[{"name":"Montreal Children's Hospital McGill University Health Centre Montreal Quebec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,5,11]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2020.11.040"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.13-1704"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.25533"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1183\/23120541.00005-2020"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00001010"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.202403-230OC"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01659-2017"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.20792"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/jpc.15818"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2014-0808"},{"key":"e_1_2_10_12_1","doi-asserted-by":"crossref","unstructured":"B.Rubbo S.Best R. 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[cited 2025 Oct 15] https:\/\/www.who.int\/tools\/child-growth-standards\/standards."},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamanetworkopen.2023.16174"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.23304"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2005-0249"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201803-0548OC"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.27412"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.21378"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/0895-4356(88)90063-7"},{"key":"e_1_2_10_22_1","unstructured":"P69\u2005A Retrospective Study Exploring Nutritional Status of Children With Bronchiectasis and Primary Ciliary Dyskinesia | Thorax [Internet]. 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