Armengot Miguel, Bancalari Catalina, Carretero-Vilarroig Lidón, Blanco-Máñez Rosana, Muñoz-Fernández Noelia, Cases Enrique, Millán José M, Almanzo Santiago, Jaijo Teresa
Department of Otorhinolaryngology, Hospital Universitari i Politècnic La Fe, 46026 Valencia, Spain.
Department of Surgery, Faculty of Medicine and Dentistry, University of Valencia, 46010 Valencia, Spain.
J Clin Med. 2025 May 14;14(10):3439. doi: 10.3390/jcm14103439.
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. 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. 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. 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.
原发性纤毛运动障碍（PCD）是一种罕见的遗传性疾病，会影响黏液纤毛系统，导致肺部进行性损伤。这种恶化可导致支气管扩张、肺不张和呼吸衰竭，严重时需要进行肺移植。本研究旨在评估PCD患者移植肺支气管上皮中的纤毛运动和超微结构，以确定移植后黏液纤毛功能是否得以保留。这些发现旨在增进科学认识，并为这些患者提供预后见解。对两名患有PCD和晚期肺部疾病并接受双侧肺移植的患者进行了一项前瞻性观察研究。使用高速视频显微镜和透射电子显微镜对鼻和支气管纤毛样本进行了分析。随访评估包括纤毛功能分析、肺排斥监测和生活质量评估，随访期长达移植后30个月。移植后评估显示，在整个研究期间（长达30个月），移植肺中的纤毛运动和超微结构正常，表明黏液纤毛功能得以长期保留。PCD患者的移植肺长期维持正常的支气管纤毛运动和结构，提示移植物和受者的预后良好。这些发现支持了PCD患者肺移植的可行性和长期有效性。
