The CFTR modulator combination elexacaftor/tezacaftor/ivacaftor (ETI) is a genetic mutation-targeted treatment in cystic fibrosis that results in profound improvements in clinical outcomes. Each of the compounds are substrates of CYP3A4/5, the cytochrome P450 enzyme family for which tacrolimus is also a substrate. The use of these compounds in an individual with a solid organ transplant has not been previously studied and there is potential for a drug interaction. In this report, we describe a pediatric liver transplant recipient with clinical decline related to cystic fibrosis who improved substantially with ETI, without significant impact on the systemic exposure of either ETI or tacrolimus.

CFTR 调节剂组合 elexacaftor/tezacaftor/ivacaftor (ETI) 是一种基因突变靶向治疗囊性纤维化，可显着改善临床结果。每种化合物都是 CYP3A4/5 的底物，CYP3A4/5 是细胞色素 P450 酶家族，他克莫司也是其底物。这些化合物在进行实体器官移植的个体中的使用之前尚未进行过研究，并且存在药物相互作用的可能性。在这份报告中，我们描述了一名患有与囊性纤维化相关的临床衰退的儿科肝移植受者，他在接受 ETI 后显着改善，而对 ETI 或他克莫司的全身暴露没有显着影响。