This article is not an overview of all facets of lung transplantation in cystic fibrosis (CF), but rather it is intended as a review of current allocation controversies, as well as of trends in diagnostics and management in lung transplant recipients and in patients with end-stage lung disease. Despite changes in donor and recipient selection, long-term survival in pediatric lung transplant has continued to be limited by chronic lung allograft dysfunction (CLAD). Due to, in part, this short survival benefit, transplant continues to be an appropriate option for only a subset of pediatric patients with CF. The feasibility of transplant as a therapeutic option is also affected by the limited pediatric organ supply, which has moreover contributed to controversy over lung allocation. Debates over the allocation of this scarce resource, however, may also help to drive innovation in the field of lung transplant. Longer pretransplant survival-as aided by new lung bypass technologies, for example-could help to alleviate organ shortages, as well as facilitate the transport of organs to suitable pediatric recipients. Improved diagnosis and treatment for CLAD and for antibody-mediated rejection have the potential to extend survival in pediatric lung transplant. Regardless, the relative rarity of transplant could pose future challenges for pediatric lung transplant programs, which require adequate numbers of patients to maintain proper expertise.

中文翻译：

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囊性纤维化的肺移植：趋势和争议。

本文并非概述囊性纤维化（CF）中肺移植的所有方面，而是旨在回顾当前的分配争议，以及肺移植接受者和终末期患者的诊断和治疗趋势阶段的肺部疾病。尽管捐助者和接受者的选择发生了变化，但小儿肺移植的长期生存仍然受到慢性同种异体移植功能障碍（CLAD）的限制。在某种程度上，由于这种短暂的生存期收益，移植仍然仅是一部分小儿CF患者的合适选择。移植作为治疗选择的可行性还受到小儿器官供应有限的影响，此外，这还引发了关于肺分配的争议。但是，关于这种稀缺资源的分配问题，也可能有助于推动肺移植领域的创新。例如，在新的肺旁路技术的帮助下，更长的移植前生存期可以帮助减轻器官短缺，并促进器官向合适的儿科接受者的运输。改良的CLAD诊断和治疗以及抗体介导的排斥反应有可能延长小儿肺移植的生存期。无论如何，相对罕见的移植可能会给小儿肺移植计划带来未来的挑战，而这需要足够数量的患者来保持适当的专业知识。以及促进器官向合适的儿科接受者的运输。改良的CLAD诊断和治疗以及抗体介导的排斥反应有可能延长小儿肺移植的生存期。无论如何，相对罕见的移植可能会给小儿肺移植计划带来未来的挑战，而这需要足够数量的患者来保持适当的专业知识。以及促进器官向合适的儿科接受者的运输。改良的CLAD诊断和治疗以及抗体介导的排斥反应有可能延长小儿肺移植的生存期。无论如何，相对罕见的移植可能会给小儿肺移植计划带来未来的挑战，而这需要足够数量的患者来保持适当的专业知识。