Division of Pulmonary Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA.
Pediatr Transplant. 2021 Jun;25(4):e14007. doi: 10.1111/petr.14007. Epub 2021 Mar 20.
Evidence for the extrapulmonary benefits of (CFTR) modulators is rapidly expanding. The use of CFTR modulators in CF patients who have undergone lung transplantation is not clear without guidance published in the medical literature to assist clinicians in the care of these patients. We discuss the potential benefits of CFTR modulators and provide insight into their use based on our experience in a small cohort of CF LTx recipients. We present pros and cons of CFTR modulator therapy for LTx recipients with CF. CFTR modulators should be considered in CF patients after lung transplantation for the time being until further research defines how to best use these therapies in transplant recipients.
(CFTR)调节剂的肺外获益证据正在迅速增加。在医学文献中没有提供指导的情况下,接受过肺移植的 CF 患者使用 CFTR 调节剂的情况尚不清楚,无法帮助临床医生为这些患者提供护理。我们根据在一小部分 CF LTx 受者中的经验,讨论了 CFTR 调节剂的潜在益处,并提供了使用它们的见解。我们提出了 CFTR 调节剂治疗 CF 肺移植受者的优缺点。在进一步的研究确定如何在移植受者中最好地使用这些疗法之前,应考虑在肺移植后暂时为 CF 患者使用 CFTR 调节剂。