依列卡福妥/替扎卡福妥/依伐卡福妥在小鼠胎儿肺外植体模型中破坏呼吸道发育。 - Suppr

Elexacaftor/Tezacaftor/Ivacaftor Disrupts Respiratory Tract Development in a Murine Fetal Lung Explant Model.

作者信息

Lhuillier Mickaël, Aoust Laura, Dreano Elise, Franco-Montoya Marie-Laure, Landry-Truchon Kim, Houde Nicolas, Chhun Stéphanie, Hinzpeter Alexandre, Edelman Aleksander, Delacourt Christophe, Jeannotte Lucie, Sermet-Gaudelus Isabelle, Hadchouel Alice

机构信息

Institut Necker Enfants Malades Paris, France.

Assistance Publique-Hôpitaux de Paris (AP-HP)-Centre Paris, France.

出版信息

Am J Respir Cell Mol Biol. 2022 Dec;67(6):723-726. doi: 10.1165/rcmb.2022-0175LE.

DOI:10.1165/rcmb.2022-0175LE

PMID:36454086

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9743190/

Abstract

摘要

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c6db/9743190/de7348c87f74/rcmb.2022-0175LEf1.jpg

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Elexacaftor/Tezacaftor/Ivacaftor Disrupts Respiratory Tract Development in a Murine Fetal Lung Explant Model.依列卡福妥/替扎卡福妥/依伐卡福妥在小鼠胎儿肺外植体模型中破坏呼吸道发育。

Am J Respir Cell Mol Biol. 2022 Dec;67(6):723-726. doi: 10.1165/rcmb.2022-0175LE.

Efficacy and safety of elexacaftor plus tezacaftor plus ivacaftor versus tezacaftor plus ivacaftor in people with cystic fibrosis homozygous for F508del-CFTR: a 24-week, multicentre, randomised, double-blind, active-controlled, phase 3b trial.依列卡福妥联合替扎卡福妥和依伐卡托对比替扎卡福妥联合依伐卡托治疗F508del-CFTR纯合子囊性纤维化患者的疗效和安全性：一项为期24周的多中心、随机、双盲、活性对照3b期试验

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Patient perspectives following initiation of elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis and advanced lung disease.接受依利尤单抗治疗的囊性纤维化患者肺功能进展的临床预测因素分析。

Respir Med Res. 2021 Nov;80:100829. doi: 10.1016/j.resmer.2021.100829. Epub 2021 May 17.

Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele.依伐卡托与泰比卡托和艾克卡托三联复方药物治疗携带单个 F508del 突变的囊性纤维化

N Engl J Med. 2019 Nov 7;381(19):1809-1819. doi: 10.1056/NEJMoa1908639. Epub 2019 Oct 31.

Efficacy and safety of the elexacaftor plus tezacaftor plus ivacaftor combination regimen in people with cystic fibrosis homozygous for the F508del mutation: a double-blind, randomised, phase 3 trial.在纯合子 F508del 突变的囊性纤维化患者中，elexacaftor 加 tezacaftor 加 ivacaftor 联合治疗方案的疗效和安全性：一项双盲、随机、3 期临床试验。

Lancet. 2019 Nov 23;394(10212):1940-1948. doi: 10.1016/S0140-6736(19)32597-8. Epub 2019 Oct 31.

Triple Therapy for Cystic Fibrosis -Gating and -Residual Function Genotypes.囊性纤维化-门控和-残留功能基因型的三联疗法。

N Engl J Med. 2021 Aug 26;385(9):815-825. doi: 10.1056/NEJMoa2100665.

Projecting the impact of delayed access to elexacaftor/tezacaftor/ivacaftor for people with Cystic Fibrosis.预测延迟接受依伐卡托/泰它卡托/艾氟卡托治疗囊性纤维化患者的影响。

J Cyst Fibros. 2021 Mar;20(2):243-249. doi: 10.1016/j.jcf.2020.07.017. Epub 2020 Aug 24.

Elexacaftor/Ivacaftor/Tezacaftor: First Approval.依伐卡托/埃乐卡福特/泰泽卡福特：首次批准。

Drugs. 2019 Dec;79(18):2001-2007. doi: 10.1007/s40265-019-01233-7.

The rescue of F508del-CFTR by elexacaftor/tezacaftor/ivacaftor (Trikafta) in human airway epithelial cells is underestimated due to the presence of ivacaftor.由于依伐卡托的存在，在人气道上皮细胞中，艾列卡托/替扎卡托/依伐卡托（三联疗法）对F508del-CFTR的挽救作用被低估了。

Eur Respir J. 2022 Feb 24;59(2). doi: 10.1183/13993003.00671-2021. Print 2022 Feb.

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.

本文引用的文献

Drug exposure to infants born to mothers taking Elexacaftor, Tezacaftor, and Ivacaftor.母亲在服用 Elexacaftor、Tezacaftor 和 Ivacaftor 时，婴儿接触药物的情况。

J Cyst Fibros. 2022 Jul;21(4):725-727. doi: 10.1016/j.jcf.2021.12.004. Epub 2021 Dec 22.

Cystic Fibrosis Transmembrane Conductance Regulator Modulators During Pregnancy: A Case Series.孕期囊性纤维化跨膜传导调节因子调节剂：病例系列

Cureus. 2021 Aug 25;13(8):e17427. doi: 10.7759/cureus.17427. eCollection 2021 Aug.

Fertility, Pregnancy and Lactation Considerations for Women with CF in the CFTR Modulator Era.CFTR调节剂时代CF女性患者的生育、妊娠和哺乳注意事项

J Pers Med. 2021 May 15;11(5):418. doi: 10.3390/jpm11050418.

Elexacafator/tezacaftor/ivacaftor resolves subfertility in females with CF: A two center case series.Elexacaftor/tezacaftor/ivacaftor 可解决 CF 女性的生育能力低下问题：两项中心病例系列研究。

J Cyst Fibros. 2021 May;20(3):399-401. doi: 10.1016/j.jcf.2020.12.011. Epub 2021 Jan 19.

The Balance between the Safety of Mother, Fetus, and Newborn Undergoing Cystic Fibrosis Transmembrane Conductance Regulator Treatments during Pregnancy.孕期接受囊性纤维化跨膜传导调节因子治疗的母亲、胎儿和新生儿的安全平衡。

ACS Pharmacol Transl Sci. 2020 Aug 19;3(5):835-843. doi: 10.1021/acsptsci.0c00098. eCollection 2020 Oct 9.

Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele.依伐卡托与泰比卡托和艾克卡托三联复方药物治疗携带单个 F508del 突变的囊性纤维化

N Engl J Med. 2019 Nov 7;381(19):1809-1819. doi: 10.1056/NEJMoa1908639. Epub 2019 Oct 31.

Clinical development of triple-combination CFTR modulators for cystic fibrosis patients with one or two alleles.用于患有一个或两个等位基因的囊性纤维化患者的三联CFTR调节剂的临床开发。

ERJ Open Res. 2019 Jun 17;5(2). doi: 10.1183/23120541.00082-2019. eCollection 2019 Apr.

Measured fetal and neonatal exposure to Lumacaftor and Ivacaftor during pregnancy and while breastfeeding.测量妊娠期和哺乳期胎儿和新生儿暴露于 Lumacaftor 和 Ivacaftor 的情况。

J Cyst Fibros. 2018 Nov;17(6):779-782. doi: 10.1016/j.jcf.2018.05.009. Epub 2018 Jun 1.

Lack of cystic fibrosis transmembrane conductance regulator disrupts fetal airway development in pigs.囊性纤维化跨膜电导调节因子缺失破坏猪胎儿气道发育。

Lab Invest. 2018 Jun;98(6):825-838. doi: 10.1038/s41374-018-0026-7. Epub 2018 Feb 21.

Crucial requirement of ERK/MAPK signaling in respiratory tract development.ERK/MAPK信号通路在呼吸道发育中的关键作用。

Development. 2015 Nov 1;142(21):3801. doi: 10.1242/dev.131821.

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Elexacaftor/Tezacaftor/Ivacaftor Disrupts Respiratory Tract Development in a Murine Fetal Lung Explant Model.

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