抽象
介绍Xenon-129(129Xe) ventilation magnetic resonance imaging (MRI) is sensitive to detect early cystic fibrosis (CF) lung disease and response to treatment.129XE-MRI可以在临床试验和患者管理中发挥重要作用。在这里,我们提出了关于成像测量的可重复性的数据及其对纵向变化的敏感性。
方法29两次评估29名儿童和成人患有CF和一系列疾病严重程度,分别为16.0(14.4-19.5)个月的中位数(四分位数(IQR))。患者接受过129XE-MRI,肺部间隙指数(LCI),体积精制和肺活动量在两次访问中。11名患者重复129Xe-MRI in the same session to assess the within-visit repeatability. The ventilation defect percentage (VDP) was the primary metric calculated from129Xe-MRI.
结果在基线,平均值±sdage was 23.0±11.1 years and forced expiratory volume in 1 s (FEV1)Z分数为-2.2±2.0。中位数(IQR)VDP为9.5(3.4-31.6)%,LCI为9.0(7.7-13.7)。VDP的访问内和访问间可重复性高。16个月,没有单一的趋势129XE-MRI病进展。可见129XE-MRI通风变化是常见的,这反映了VDP的变化。根据访问内的重复性,VDP的显着短期变化为>±1.6%。对于长期随访,可以预期高达±7.7%的VDP的变化,或常规FEV的患者的±4.1%1。没有患者在FEV中有重大变化1; however, 59% had change in VDP >±1.6%. In patients with normal FEV1,通风和VDP中存在显着变化。
Conclusions129XE-MRI是评估CF患者纵向肺病的高效方法。VDP具有良好的肺功能敏感临床结果测量和临床试验的终点。
抽象
129CF中的XE-MRI是高度可重复的。在普通FEV的患者中1,129Xe-MRI is also sensitive to detect changes in longitudinal lung function and should be highly informative in an era of CFTR modulators and increasingly preserved FEV1https://bit.ly/2c0d8np.
脚注
本文提供了补充材料www.qdcxjkg.com
Conflict of interest: L.J. Smith reports grants from National Institute for Health Research, during the conduct of the study.
利益冲突:A.康斯利报告了国家卫生研究所的赠款,在这项研究期间;来自囊性纤维化纤维化纤维化纤维化基础的补助,来自Mylan Pharmaceuticals和Vertex Pharmaceuticals的个人费用,在提交的工作之外。
Conflict of interest: J. Bray has nothing to disclose.
Conflict of interest: P.J.C. Hughes reports grants from GlaxoSmithKline, outside the submitted work.
Conflict of interest: A. Biancardi has nothing to disclose.
Conflict of interest: G. Norquay has nothing to disclose.
利益冲突:M. Wildman没有什么可披露的。
Conflict of interest: N. West has nothing to disclose.
利益冲突:H. Marshall无需披露。
Conflict of interest: J.M. Wild has nothing to disclose.
Support statement: This report is independent research supported by the National Institute for Health Research (NIHR) and Health Education England and also the Medical Research Council (MRC). This work was supported by the NIHR grants ICA-CDRF-2015-01-027 (awarded to L.J. Smith) and NIHR-RP-R3-12-027 (awarded to J.M. Wild) and MRC grant MR/M008894/1 (awarded to J.M. Wild). A. Horsley was supported by an NIHR Clinician Scientist award (NIHR-CS012-13) and by the NIHR Manchester Biomedical Research Centre. The views expressed in this publication are those of the author(s) and not necessarily those of the NHS, the National Institute for Health Research, Health Education England or the Department of Health. Funding information for this article has been deposited with theCrossref Funder Registry。
- 收到February 26, 2020.
- AcceptedJune 29, 2020.
- 复制right ©ERS 2020