{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,2]],"date-time":"2026-06-02T00:53:20Z","timestamp":1780361600163,"version":"3.54.1"},"reference-count":25,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2023,4,25]],"date-time":"2023-04-25T00:00:00Z","timestamp":1682380800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2023,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>PCD is a genetic disease leading to a decline in pulmonary function. There is only little knowledge of factors determining the long\u2010term pulmonary outcome. Especially adherence has not been addressed yet although being an independent risk factor for an increased loss of lung capacity in other chronic respiratory diseases.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objective<\/jats:title><jats:p>Assessing the impact of bacterial airway colonization and adherence on long\u2010term lung function in patients with PCD.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Data on colonization and lung function parameters like forced expiratory volume in the first second (FEV<jats:sub>1,<\/jats:sub> <jats:italic>Z\u2010<\/jats:italic>score) and lung clearance index (LCI<jats:sub>2,5%<\/jats:sub>) were collected for 7.01\u2009\u00b1\u20092.2 years (893 quarters) in 44 PCD patients. Adherence was classified as good, moderate or poor. The impact of both adherence and colonization was assessed for the long\u2010term course of FEV1, the association of colonization with lung function also quarterly.<\/jats:p><\/jats:sec><jats:sec><jats:title>Statistics<\/jats:title><jats:p>Kruskall\u2013Wallis test, <jats:italic>T<\/jats:italic> test, ANOVA, linear regression, linear mixed model.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Chronic colonization did not show any impact on the for long\u2010term course of FEV<jats:sub>1<\/jats:sub>, but adherence was a significant factor: patients with good adherence showed better FEV<jats:sub>1<\/jats:sub> at the end of the observation period than children with poor adherence (\u20130.15\u2009\u00b1\u20090.88 vs. \u20132.63\u2009\u00b1\u20091.79, <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.01).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Adherence has not yet been investigated for PCD. However, we found it to be a major significant factor affecting long\u2010term FEV<jats:sub>1<\/jats:sub> in PCD. Thus, it should be taken into consideration in the treatment protocols for PCD.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.26417","type":"journal-article","created":{"date-parts":[[2023,4,25]],"date-time":"2023-04-25T10:44:51Z","timestamp":1682419491000},"page":"1950-1958","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Risk factors for the deterioration of pulmonary function in primary ciliary dyskinesia"],"prefix":"10.1002","volume":"58","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3752-0985","authenticated-orcid":false,"given":"Vanessa","family":"Fein","sequence":"first","affiliation":[{"name":"Department of Paediatric Pneumology, University Children's Hospital Ruhr\u2010University Bochum Bochum Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christoph","family":"Maier","sequence":"additional","affiliation":[{"name":"University Children's Hospital Ruhr\u2010University Bochum 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