Ciliary Motility and Ultrastructure in Bronchial Epithelium of Lung Transplant Recipients with Primary Ciliary Dyskinesia.

Autores de IIS La Fe

• Miguel Armengot Carceller

  Autor

• María Del Lidón Carretero Vilarroig

  Autor

• Rosana Blanco Mañez

  Autor

• Noelia Múñoz Fernández

  Autor

• Enrique Cases Viedma

  Autor

• 

  José María Millán Salvador

  Autor

• Santiago Almanzo

  Autor

• Teresa Jaijo Sanchís

  Autor

Participantes ajenos a IIS La Fe

• Bancalari C

Grupos

• 

  Biomedicina Molecular, Celular y Genómica

• Infecciones Respiratorias

• Neumología

Abstract

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.

Keywords

• Kartagener syndrome; bronchiectasis; ciliary motility; ciliary ultrastructure; ciliopathies; lung transplantation