{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T17:09:12Z","timestamp":1791220152541,"version":"4.1.0"},"reference-count":31,"publisher":"European Respiratory Society (ERS)","issue":"2","content-domain":{"domain":["publications.ersnet.org"],"crossmark-restriction":true},"short-container-title":["Eur Respir J"],"accepted":{"date-parts":[[2016,4,13]]},"published-print":{"date-parts":[[2016,8]]},"abstract":"<jats:p>Primary ciliary dyskinesia (PCD) in adults has not been well described. In this retrospective observational study we aimed to characterise a large adult population and identify features associated with disease progression.<\/jats:p><jats:p>We retrospectively analysed 151 adult patients at a single tertiary centre at baseline and longitudinally for a median of 7\u2005years.<\/jats:p><jats:p>We found significant variation in age at diagnosis (median 23.5\u2005years; range &lt;1\u201372\u2005years). Older age at diagnosis was associated with impaired baseline forced expiratory volume in 1\u2005s (FEV<jats:sub>1<\/jats:sub>) (r=\u200a\u22120.30, p=0.01) and increased<jats:italic>Pseudomonas aeruginosa<\/jats:italic>colonisation (difference in medians 17\u2005years (95% CI 4.5\u201320\u2005years); p=0.002). Lung function decline was estimated at FEV<jats:sub>1<\/jats:sub>decline of 0.49% predicted per year. Lung function decline was associated with ciliary ultrastructure, with microtubular defect patients having the greatest decline (p=0.04). High-resolution computed tomography (HRCT) scores of severity of bronchial wall dilatation (p&lt;0.001) and extent of bronchiectasis (p=0.03) additionally showed evidence of modifying FEV<jats:sub>1<\/jats:sub>decline with age.<\/jats:p><jats:p>Our study reveals that a large proportion of adult PCD patients are diagnosed late, with impaired FEV<jats:sub>1<\/jats:sub>and increased<jats:italic>P. aeruginosa<\/jats:italic>colonisation. Increased disease burden on HRCT and ciliary ultrastructure may predict progressive lung function decline. This study characterises a large adult PCD population, identifies features associated with disease progression and highlights the need for prospective trials to determine whether early diagnosis of high-risk subgroups alongside optimal management can modify disease progression.<\/jats:p>","DOI":"10.1183\/13993003.00209-2016","type":"journal-article","created":{"date-parts":[[2016,6,11]],"date-time":"2016-06-11T04:26:57Z","timestamp":1465619217000},"page":"441-450","update-policy":"https:\/\/doi.org\/10.1183\/ers-crossmark-policy","source":"Crossref","is-referenced-by-count":145,"title":["A longitudinal study characterising a large adult primary ciliary dyskinesia population"],"prefix":"10.1183","volume":"48","author":[{"given":"Anand","family":"Shah","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amelia","family":"Shoemark","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephanie 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