Case reportLiving-donor lobar lung transplantation for primary ciliary dyskinesia
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Comment
There has been growing appreciation of the significant role played by the mucociliary transport system in the body. The mucociliary transport system is an important defense mechanism by which the human body usually maintains its homeostasis by protecting the body against invading particles, including bacteria. This system includes two major functional mechanisms, mucous secretional and ciliary transport systems, each of which is usually complementary and cooperative. Cystic fibrosis (CF) is
Acknowledgements
We acknowledge Professor Yuji Ohtsuki, Department of Pathology II, Kochi Medical University, for his electron micrograph courtesy.
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Living-donor lobar lung transplantation
2024, Journal of Heart and Lung TransplantationTechnical Challenges in Lung Transplantation of Kartagener Syndrome Recipients: A Unique Team Experience With 12 Patients
2020, Transplantation ProceedingsCitation Excerpt :Considering that the majority of these patients do not have cardiac abnormalities in conjunction with lung disease, except patients with concomitant cardiac malformations, en-bloc double LTx and sequential bilateral LTx with or without cardiopulmonary bypass are feasible [9–12]. Recent reports in the literature described the possibility of managing KS recipients with bilateral lobar LTx from living donors supported by cardiopulmonary bypass and even unilateral LTx with contralateral pneumonectomy and central venous-arterial extracorporeal membrane oxygenation [2,13]. However, some publications do not consider any technical modification of the classical sequential bilateral LTx, and other centers even consider this disease a contraindication for LTx.
Clinical and genetic aspects of primary ciliary dyskinesia/kartagener syndrome
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2006, Seminars in PerinatologyCitation Excerpt :Few, limited, longitudinal studies of PCD suggest that the onset of airway disease occurs early in childhood,23-25 yet with appropriate diagnosis and therapy, the progression of lung disease can be slowed. For end-stage lung disease in PCD, lung transplantation (including heart–lung, bilateral lung, and living related bilateral lung transplantation) is an option and has been performed successfully with surgical modifications for patients with situs inversus.87-89 The extrapulmonary manifestations of PCD can contribute to morbidity.
Long-term management and outcome of lung transplantation in Japan
2023, Journal of Thoracic Disease