{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,5]],"date-time":"2026-06-05T10:59:28Z","timestamp":1780657168572,"version":"3.54.1"},"reference-count":21,"publisher":"MDPI AG","issue":"10","license":[{"start":{"date-parts":[[2025,5,14]],"date-time":"2025-05-14T00:00:00Z","timestamp":1747180800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Instituto de Salud Carlos III","award":["PI19\/00949"],"award-info":[{"award-number":["PI19\/00949"]}]},{"name":"Instituto de Salud Carlos III","award":["PI22\/01010"],"award-info":[{"award-number":["PI22\/01010"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Background and Objective: Primary ciliary dyskinesia (PCD) is a rare genetic disorder that affects the mucociliary system, leading to progressive lung damage. This deterioration can result in bronchiectasis, atelectasis, and respiratory failure, necessitating lung transplantation in severe cases. This study aims to assess ciliary motility and ultrastructure in the bronchial epithelium of transplanted lungs in patients with PCD to determine whether mucociliary function is preserved post-transplantation. The findings seek to enhance scientific understanding and provide prognostic insights for these patients. Materials and Methods: A prospective observational study was conducted on two patients with PCD and advanced lung disease who underwent bilateral lung transplantation. Nasal and bronchial cilia samples were analyzed using high-speed videomicroscopy and transmission electron microscopy. Follow-up assessments included ciliary function analysis, lung rejection monitoring, and quality-of-life evaluations, with follow-up extending up to 30 months post-transplant. Results: Post-transplant evaluations demonstrated normal ciliary motility and ultrastructure in the transplanted lungs throughout the study period (up to 30 months), indicating the long-term preservation of mucociliary function. Conclusions: Transplanted lungs in patients with PCD maintain normal bronchial ciliary motility and structure in the long term, suggesting a favorable prognosis for both the graft and the recipient. These findings support the feasibility and long-term effectiveness of lung transplantation in patients with PCD.<\/jats:p>","DOI":"10.3390\/jcm14103439","type":"journal-article","created":{"date-parts":[[2025,5,14]],"date-time":"2025-05-14T12:22:17Z","timestamp":1747225337000},"page":"3439","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Ciliary Motility and Ultrastructure in Bronchial Epithelium of Lung Transplant Recipients with Primary Ciliary Dyskinesia"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8258-6292","authenticated-orcid":false,"given":"Miguel","family":"Armengot","sequence":"first","affiliation":[{"name":"Department of Otorhinolaryngology, Hospital Universitari i Polit\u00e8cnic La Fe, 46026 Valencia, Spain"},{"name":"Department of Surgery, Faculty of Medicine and Dentistry, University of Valencia, 46010 Valencia, Spain"},{"name":"Center for Biomedical Network Research on Rare Diseases CIBERER), Carlos III Health Institute, 46026 Valencia, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0556-6583","authenticated-orcid":false,"given":"Catalina","family":"Bancalari","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology, Hospital Universitari i Polit\u00e8cnic La Fe, 46026 Valencia, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lid\u00f3n","family":"Carretero-Vilarroig","sequence":"additional","affiliation":[{"name":"Molecular, Cellular and Genomic Biomedicine Group (BMCG), IIS La Fe, 46026 Valencia, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rosana","family":"Blanco-M\u00e1\u00f1ez","sequence":"additional","affiliation":[{"name":"Department of Pathology, University and Polytechnic Hospital La Fe, 46026 Valencia, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Noelia","family":"Mu\u00f1oz-Fern\u00e1ndez","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology, Hospital Universitari i Polit\u00e8cnic La Fe, 46026 Valencia, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Enrique","family":"Cases","sequence":"additional","affiliation":[{"name":"Department of Pulmonology, University and Polytechnic Hospital La Fe, 46026 Valencia, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7211-9129","authenticated-orcid":false,"given":"Jos\u00e9 M.","family":"Mill\u00e1n","sequence":"additional","affiliation":[{"name":"Molecular, Cellular and Genomic Biomedicine Group (BMCG), IIS La Fe, 46026 Valencia, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8316-3010","authenticated-orcid":false,"given":"Santiago","family":"Almanzo","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology, Hospital Universitari i Polit\u00e8cnic La Fe, 46026 Valencia, Spain"},{"name":"Department of Surgery, Faculty of Medicine and Dentistry, University of Valencia, 46010 Valencia, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0973-9146","authenticated-orcid":false,"given":"Teresa","family":"Jaijo","sequence":"additional","affiliation":[{"name":"Molecular, Cellular and Genomic Biomedicine Group (BMCG), IIS La Fe, 46026 Valencia, Spain"},{"name":"Genetics Unit, University and Polytechnic Hospital La Fe, 46026 Valencia, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,5,14]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","unstructured":"Werner, C., Onnebrink, J.G., and Omran, H. 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