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Elexacaftor/Tezacaftor/Ivacaftor Improved Clinical Outcomes in a Patient with N1303K-CFTR Based on Experimental Evidence.基于实验证据，依列卡福妥/替扎卡福妥/依伐卡福妥改善了一名携带N1303K-CFTR突变的患者的临床结局。
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Elexacaftor/Tezacaftor/Ivacaftor Supports Treatment for CF with ΔI1023-V1024-CFTR.依列卡福妥/替扎卡福妥/依伐卡托支持对携带ΔI1023-V1024-CFTR的囊性纤维化患者进行治疗。
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Response to Elexacaftor/Tezacaftor/Ivacaftor in people with cystic fibrosis with the N1303K mutation: Case report and review of the literature.携带N1303K突变的囊性纤维化患者对依列卡福/替扎卡福/依伐卡福的反应：病例报告及文献综述
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Cystic fibrosis airway inflammation enables elexacaftor/tezacaftor/ivacaftor-mediated rescue of N1303K mutation.囊性纤维化气道炎症可实现依列卡福/替扎卡福/依伐卡福对N1303K突变的挽救作用。
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A personalized medicine approach to optimize care for a pediatric cystic fibrosis patient with atypical clinical symptoms.一种个性化医疗方法，用于优化对一名具有非典型临床症状的儿科囊性纤维化患者的护理。
Pediatr Pulmonol. 2024 Jan;59(1):229-232. doi: 10.1002/ppul.26719. Epub 2023 Oct 11.

本文引用的文献

1
Efficacy of Elexacaftor/Tezacaftor/Ivacaftor in Advanced Cystic Fibrosis Lung Disease.依列卡福妥/替扎卡福妥/依伐卡托治疗晚期囊性纤维化肺病的疗效。
Ann Am Thorac Soc. 2021 Nov;18(11):1924-1927. doi: 10.1513/AnnalsATS.202102-220RL.
2
A Phase 3 Open-Label Study of Elexacaftor/Tezacaftor/Ivacaftor in Children 6 through 11 Years of Age with Cystic Fibrosis and at Least One Allele.一项评估 elexacaftor/tezacaftor/ivacaftor 在至少携带一个突变等位基因的囊性纤维化 6 至 11 岁儿童中的疗效和安全性的 3 期、开放性标签研究。
Am J Respir Crit Care Med. 2021 Jun 15;203(12):1522-1532. doi: 10.1164/rccm.202102-0509OC.
3
Rapid Improvement after Starting Elexacaftor-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and Advanced Pulmonary Disease.在患有囊性纤维化和晚期肺部疾病的患者中开始使用 Elexacaftor-Tezacaftor-Ivacaftor 后的快速改善。
Am J Respir Crit Care Med. 2021 Jul 1;204(1):64-73. doi: 10.1164/rccm.202011-4153OC.
4
Rescue of multiple class II CFTR mutations by elexacaftor+tezacaftor+ivacaftor mediated in part by the dual activities of elexacaftor as both corrector and potentiator.依利卓（elexacaftor）作为矫正剂和增强剂的双重作用部分介导了依利卓（elexacaftor）联合泰比培南（tezacaftor）和 ivacaftor 对多种 II 类 CFTR 突变的挽救。
Eur Respir J. 2021 Jun 17;57(6). doi: 10.1183/13993003.02774-2020. Print 2021 Jun.
5
Efficacy of elexacaftor/tezacaftor/ivacaftor in patients with cystic fibrosis and advanced lung disease.依列卡福妥/替扎卡福妥/依伐卡托对患有囊性纤维化和晚期肺部疾病患者的疗效。
Eur Respir J. 2021 Feb 25;57(2). doi: 10.1183/13993003.03079-2020. Print 2021 Feb.
6
Allosteric folding correction of F508del and rare CFTR mutants by elexacaftor-tezacaftor-ivacaftor (Trikafta) combination.三药复方（Trikafta）依利卓艾他康唑/艾美拉唑/卡博替尼（elexacaftor-tezacaftor-ivacaftor）对 F508del 及罕见 CFTR 突变体的别构折叠校正。
JCI Insight. 2020 Sep 17;5(18):139983. doi: 10.1172/jci.insight.139983.
7
Clinical Effectiveness of Lumacaftor/Ivacaftor in Patients with Cystic Fibrosis Homozygous for F508del-CFTR. A Clinical Trial.鲁马卡托/依伐卡托对F508del-CFTR纯合子囊性纤维化患者的临床疗效。一项临床试验。
Ann Am Thorac Soc. 2021 Jan;18(1):75-83. doi: 10.1513/AnnalsATS.202002-144OC.
8
Combination potentiator ('co-potentiator') therapy for CF caused by CFTR mutants, including N1303K, that are poorly responsive to single potentiators.联合增强剂（“共增强剂”）疗法用于治疗 CFTR 突变体引起的 CF，包括对单增强剂反应不佳的 N1303K。
J Cyst Fibros. 2018 Sep;17(5):595-606. doi: 10.1016/j.jcf.2018.05.010. Epub 2018 Jun 12.
9
Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del.Tezacaftor-Ivacaftor 治疗纯合子 Phe508del 突变型囊性纤维化患者的疗效
N Engl J Med. 2017 Nov 23;377(21):2013-2023. doi: 10.1056/NEJMoa1709846. Epub 2017 Nov 3.
10
Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770.CFTR增强剂VX-770对体外CF气道上皮细胞功能的挽救作用
Proc Natl Acad Sci U S A. 2009 Nov 3;106(44):18825-30. doi: 10.1073/pnas.0904709106. Epub 2009 Oct 21.

Elexacaftor/Tezacaftor/Ivacaftor Improved Clinical Outcomes in a Patient with N1303K-CFTR Based on Experimental Evidence.

作者信息

Huang Yunjie, Paul Grace, Lee Jesun, Yarlagadda Sunitha, McCoy Karen, Naren Anjaparavanda P

机构信息

Cincinnati Children's Hospital Medical Center Cincinnati, Ohio.

Nationwide Children's Hospital Columbus, Ohio.

出版信息

Am J Respir Crit Care Med. 2021 Nov 15;204(10):1231-1235. doi: 10.1164/rccm.202101-0090LE.

DOI:10.1164/rccm.202101-0090LE
PMID:34379998
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8759307/
Abstract
摘要