{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,31]],"date-time":"2026-08-31T23:26:04Z","timestamp":1788218764089,"version":"build-2803163510"},"reference-count":33,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2026,6,18]],"date-time":"2026-06-18T00:00:00Z","timestamp":1781740800000},"content-version":"vor","delay-in-days":17,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2026,6,1]],"date-time":"2026-06-01T00:00:00Z","timestamp":1780272000000},"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":[[2026,6]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>Primary ciliary dyskinesia (PCD) is a genetic disorder characterized by impaired mucociliary clearance, leading to recurrent airway infections and bronchiectasis. Monitoring disease progression requires both functional and structural assessments of the lungs. This study aims to investigate the relationship between lung function, structural abnormalities, and quality of life in patients with PCD.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Materials and Methods<\/jats:title>\n                    <jats:p>The study included 30 genetically confirmed PCD patients under routine follow\u2010up and had no active infection at the time of evaluation. Demographic and clinical data were recorded. Pulmonary function tests (PFT), including spirometry, plethysmography, and diffusion capacity for carbon monoxide, performed within the past year, were analyzed. Chest computed tomography (CT) scans were evaluated using the modified Bhalla scoring system. The St. George's Respiratory Questionnaire (SGRQ) was administered during the most recent follow\u2010up.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      The median age of the 30 PCD patients (male\u2010to\u2010female ratio: 17:13) was 11.6 years. The median FEV\n                      <jats:sub>1<\/jats:sub>\n                      z\u2010score was \u22121.6, with mild, moderate, and severe obstruction observed in 48%, 24%, and 20% of the patients, respectively. FEV\n                      <jats:sub>1<\/jats:sub>\n                      , FVC, and FEF\n                      <jats:sub>25\u201075<\/jats:sub>\n                      values decreased with age, while TLC was increased. High\u2010resolution computed tomography (HRCT) revealed mild lung damage in 80% and moderate damage in 20% of the cohort. SGRQ scores showed a positive correlation with exacerbations and a negative correlation with spirometric parameters.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Pulmonary functional impairment and reduced health\u2010related quality of life in children with PCD are closely related to the perceived burden of disease. Although structural abnormalities on HRCT do not directly affect quality\u2010of\u2010life scores, they correlate with functional impairment.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ppul.71705","type":"journal-article","created":{"date-parts":[[2026,6,18]],"date-time":"2026-06-18T17:16:39Z","timestamp":1781802999000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["The Relationship Between Structural and Functional Pulmonary Status and Quality of Life in Children With Primary Ciliary Dyskinesia"],"prefix":"10.1002","volume":"61","author":[{"ORCID":"https:\/\/orcid.org\/0009-0007-8946-0239","authenticated-orcid":false,"given":"Nesibe Hatun","family":"Sar\u0131han","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Faculty of Medicine Istanbul Medeniyet University Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9801-3181","authenticated-orcid":false,"given":"Sinem Can","family":"Oksay","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Pediatric Pulmonology, Faculty of Medicine Istanbul Medeniyet University Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1593-4351","authenticated-orcid":false,"given":"Sabriye G\u00fcl\u00e7in","family":"Bozbeyo\u011flu","sequence":"additional","affiliation":[{"name":"Department of Radiology, Faculty of Medicine Istanbul Medeniyet University Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7556-6568","authenticated-orcid":false,"given":"Saniye","family":"Girit","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Pediatric Pulmonology, Faculty of Medicine Istanbul Medeniyet University Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,6,18]]},"reference":[{"key":"e_1_2_11_2_1","article-title":"Primer Siliyer Diskinezide G\u00fcncel Tedaviler. 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