• 文献检索
  • 文档翻译
  • 深度研究
  • 学术资讯
  • Suppr Zotero 插件Zotero 插件
  • 邀请有礼
  • 套餐&价格
  • 历史记录
应用&插件
Suppr Zotero 插件Zotero 插件浏览器插件Mac 客户端Windows 客户端微信小程序
定价
高级版会员购买积分包购买API积分包
服务
文献检索文档翻译深度研究API 文档MCP 服务
关于我们
关于 Suppr公司介绍联系我们用户协议隐私条款
关注我们

Suppr 超能文献

核心技术专利:CN118964589B侵权必究
粤ICP备2023148730 号-1Suppr @ 2026

文献检索

告别复杂PubMed语法,用中文像聊天一样搜索,搜遍4000万医学文献。AI智能推荐,让科研检索更轻松。

立即免费搜索

文件翻译

保留排版,准确专业,支持PDF/Word/PPT等文件格式,支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述,25分钟生成高质量综述,智能提取关键信息,辅助科研写作。

立即免费体验

依列卡福妥-替扎卡福妥-依伐卡福妥疗法对囊性纤维化和晚期肺部疾病患者磁共振成像的早期影响

Early Effects of Elexacaftor-Tezacaftor-Ivacaftor Therapy on Magnetic Resonance Imaging in Patients with Cystic Fibrosis and Advanced Lung Disease.

作者信息

Macconi Letizia, Galici Valeria, Di Maurizio Marco, Rossi Enrica, Taccetti Giovanni, Terlizzi Vito

机构信息

Department of Radiology, Meyer Children's Hospital, 50139 Florence, Italy.

Department of Paediatric Medicine, Cystic Fibrosis Regional Reference Center, Meyer Children's Hospital, 50139 Florence, Italy.

出版信息

J Clin Med. 2022 Jul 22;11(15):4277. doi: 10.3390/jcm11154277.

DOI:10.3390/jcm11154277
PMID:35893365
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9331995/
Abstract

This is a prospective, observational study involving three Cystic Fibrosis (CF) adult patients, evaluating the changes in chest magnetic resonance imaging (MRI) three months after the start of elexacaftor/tezacaftor and ivacaftor therapy. MRI showed a drastic reduction in mucus plugging and bronchial wall thickening, with an improvement in the diffusion-weighted MRI score. Similarly, a marked improvement in spirometric parameters, nutritional status, and sweat chloride was observed. Our preliminary data confirm that chest MRI could be a useful tool to assess disease progression in CF patients on modulatory drug therapy.

摘要

这是一项前瞻性观察性研究,涉及三名成年囊性纤维化(CF)患者,评估在开始使用依列卡福/替扎卡福和依伐卡托治疗三个月后胸部磁共振成像(MRI)的变化。MRI显示黏液阻塞和支气管壁增厚明显减轻,扩散加权MRI评分有所改善。同样,观察到肺功能参数、营养状况和汗液氯化物水平有显著改善。我们的初步数据证实,胸部MRI可能是评估接受调节药物治疗的CF患者疾病进展的有用工具。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/14c0/9331995/eade35b1b4b6/jcm-11-04277-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/14c0/9331995/eade35b1b4b6/jcm-11-04277-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/14c0/9331995/eade35b1b4b6/jcm-11-04277-g001.jpg

相似文献

1
Early Effects of Elexacaftor-Tezacaftor-Ivacaftor Therapy on Magnetic Resonance Imaging in Patients with Cystic Fibrosis and Advanced Lung Disease.依列卡福妥-替扎卡福妥-依伐卡福妥疗法对囊性纤维化和晚期肺部疾病患者磁共振成像的早期影响
J Clin Med. 2022 Jul 22;11(15):4277. doi: 10.3390/jcm11154277.
2
Structural changes in lung morphology detected by MRI after modulating therapy with elexacaftor/tezacaftor/ivacaftor in adolescent and adult patients with cystic fibrosis.MRI 检测调制治疗后青少年和成年囊性纤维化患者肺形态结构的变化:依利卓艾卡福特/替扎卡福特/伊伐卡福特。
Respir Med. 2023 Sep;216:107328. doi: 10.1016/j.rmed.2023.107328. Epub 2023 Jun 13.
3
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
Lancet Respir Med. 2022 Mar;10(3):267-277. doi: 10.1016/S2213-2600(21)00454-9. Epub 2021 Dec 20.
4
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.
5
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.
6
Elexacaftor/Tezacaftor/Ivacaftor Improves Bronchial Artery Dilatation Detected by Magnetic Resonance Imaging in Patients with Cystic Fibrosis.依列卡福妥/替扎卡福妥/依伐卡福妥可改善囊性纤维化患者磁共振成像检测到的支气管动脉扩张。
Ann Am Thorac Soc. 2023 Nov;20(11):1595-1604. doi: 10.1513/AnnalsATS.202302-168OC.
7
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.
8
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.
9
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.
10
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.

引用本文的文献

1
Tracheal Diverticula in People with Cystic Fibrosis on Elexacaftor/Tezacaftor/Ivacaftor: An Italian Multicenter Retrospective Study.接受依列卡福妥/替扎卡福妥/依伐卡福妥治疗的囊性纤维化患者的气管憩室:一项意大利多中心回顾性研究
J Clin Med. 2025 Mar 28;14(7):2320. doi: 10.3390/jcm14072320.
2
Liver biochemical indexes and cholesterol metabolism in cystic fibrosis patients with F508del/CFTR variant genotype after elexacaftor/tezacaftor/ivacaftor treatment.囊性纤维化 F508del/CFTR 变异基因型患者接受依伐卡托/泰他卡托/艾氟卡托治疗后的肝生化指标和胆固醇代谢。
Sci Rep. 2024 Jul 29;14(1):17422. doi: 10.1038/s41598-024-68511-7.
3
One year of treatment with elexacaftor/tezacaftor/ivacaftor in patients with cystic fibrosis homozygous for the F508del mutation causes a significant increase in liver biochemical indexes.

本文引用的文献

1
Effects of Elexacaftor/Tezacaftor/Ivacaftor Therapy on Lung Clearance Index and Magnetic Resonance Imaging in Patients with Cystic Fibrosis and One or Two Alleles.依伐卡托/泰他卡托/艾维雷司他钠治疗对具有一个或两个等位基因突变的囊性纤维化患者肺清除指数和磁共振成像的影响。
Am J Respir Crit Care Med. 2022 Aug 1;206(3):311-320. doi: 10.1164/rccm.202201-0219OC.
2
Magnetic resonance imaging detects improvements of pulmonary and paranasal sinus abnormalities in response to elexacaftor/tezacaftor/ivacaftor therapy in adults with cystic fibrosis.磁共振成像检测到,在接受依列卡福/替扎卡福/依伐卡福治疗的成年囊性纤维化患者中,肺部和鼻窦异常情况有所改善。
J Cyst Fibros. 2022 Nov;21(6):1053-1060. doi: 10.1016/j.jcf.2022.03.011. Epub 2022 Apr 8.
3
在携带F508del突变纯合子的囊性纤维化患者中,使用依列卡福/替扎卡福/艾伐卡福治疗一年会导致肝脏生化指标显著升高。
Front Mol Biosci. 2024 Jan 8;10:1327958. doi: 10.3389/fmolb.2023.1327958. eCollection 2023.
4
Elexacaftor-Tezacaftor-Ivacaftor: A Life-Changing Triple Combination of CFTR Modulator Drugs for Cystic Fibrosis.依列卡福妥-替扎卡福妥-依伐卡托:用于囊性纤维化的改变人生的三联组合CFTR调节剂药物。
Pharmaceuticals (Basel). 2023 Mar 8;16(3):410. doi: 10.3390/ph16030410.
5
Effects of Lumacaftor/Ivacaftor on Cystic Fibrosis Disease Progression in Children 2 through 5 Years of Age Homozygous for : A Phase 2 Placebo-controlled Clinical Trial.Lumacaftor/Ivacaftor 对 2 至 5 岁纯合子囊性纤维化患儿疾病进展的影响:一项 2 期安慰剂对照临床试验。
Ann Am Thorac Soc. 2023 Aug;20(8):1144-1155. doi: 10.1513/AnnalsATS.202208-684OC.
Magnetic resonance imaging of cystic fibrosis: Multi-organ imaging in the age of CFTR modulator therapies.
囊性纤维化的磁共振成像:CFTR调节剂疗法时代的多器官成像。
J Cyst Fibros. 2022 Mar;21(2):e148-e157. doi: 10.1016/j.jcf.2021.11.006. Epub 2021 Dec 5.
4
Effectiveness and safety of elexacaftor/tezacaftor/ivacaftor in patients with cystic fibrosis and advanced lung disease with the Phe508del/minimal function genotype.依洛尤单抗治疗家族性高胆固醇血症的有效性和安全性:一项随机、双盲、安慰剂对照研究
Respir Med. 2021 Nov-Dec;189:106646. doi: 10.1016/j.rmed.2021.106646. Epub 2021 Oct 12.
5
Effects of Lumacaftor-Ivacaftor on Lung Clearance Index, Magnetic Resonance Imaging, and Airway Microbiome in Phe508del Homozygous Patients with Cystic Fibrosis.Lumacaftor-Ivacaftor 对肺清除指数、磁共振成像和 Phe508del 纯合子囊性纤维化患者气道微生物组的影响。
Ann Am Thorac Soc. 2021 Jun;18(6):971-980. doi: 10.1513/AnnalsATS.202008-1054OC.
6
Detection and monitoring of lung inflammation in cystic fibrosis during respiratory tract exacerbation using diffusion-weighted magnetic resonance imaging.使用扩散加权磁共振成像检测和监测囊性纤维化呼吸道加重期间的肺部炎症。
Eur Respir J. 2017 Jul 20;50(1). doi: 10.1183/13993003.01437-2016. Print 2017 Jul.
7
Use of hyperpolarized helium-3 MRI to assess response to ivacaftor treatment in patients with cystic fibrosis.使用超极化氦-3磁共振成像评估囊性纤维化患者对依伐卡托治疗的反应。
J Cyst Fibros. 2017 Mar;16(2):267-274. doi: 10.1016/j.jcf.2016.12.004. Epub 2017 Jan 26.
8
Usefulness of diffusion-weighted MR imaging in the evaluation of pulmonary lesions.弥散加权磁共振成像在肺部病变评估中的应用。
Eur Radiol. 2010 Apr;20(4):807-15. doi: 10.1007/s00330-009-1629-6. Epub 2009 Oct 28.
9
Changes in airway dimensions on computed tomography scans of children with cystic fibrosis.囊性纤维化患儿计算机断层扫描气道尺寸的变化
Am J Respir Crit Care Med. 2005 Jul 15;172(2):218-24. doi: 10.1164/rccm.200410-1311OC. Epub 2005 Apr 14.