Using Goldmann Visual Field Volume to Track Disease Progression in Choroideremia.
Using Goldmann Visual Field Volume to Track Disease Progression in Choroideremia.
复制标题
使用 Goldmann 视野体积追踪无脉络膜症的疾病进展。
DOI：
10.1016/j.xops.2023.100397
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发表时间：
2023-12
影响因子：
--
通讯作者：
Scheetz, Todd E.
中科院分区：
文献类型：
--
作者：
Deluca, Adam P.;Whitmore, S. Scott;Tatro, Nicole J.;Andorf, Jeaneen L.;Faga, Ben P.;Faga, Laurel A.;Colins, Malia M.;Luse, Meagan A.;Fenner, Beau J.;Stone, Edwin M.;Scheetz, Todd E.
无脉络膜症是由CHM基因中的致病性变体引起的X连锁脉络膜病变。其特征是周边视野中早期出现多个暗点，这些暗点扩散并合并，通常直到疾病晚期才保留中央视觉。这些特征使得视觉衰退的定量监测特别具有挑战性。在这里，我们描述了一种新的计算方法，将戈德曼视野（GVF）的数据转化为定量体积测量。通过这种方法，我们分析了无脉络膜患者纵向回顾性队列的视野丧失。单中心、回顾性、队列研究。我们分析了来自41个家庭的56例经分子学证实的男性无脉络膜患者的238次临床访视的数据（范围，每例患者1-27次访视）。患者的中位随访时间为4年（范围：0-56岁），访视时年龄范围为5 - 76岁。包括GVF数据在内的经分子学证实的无脉络膜患者的临床数据被纳入分析。使用基于平板电脑的应用程序追踪Goldmann视野记录，并为每个轨迹内插三维视觉山。该程序允许从几十年来使用不同协议收集的数据中量化视野损失，包括不同或不完整的等深器。收集视力（VA）数据并转换为最小分辨角值的对数。延迟指数混合效应模型用于评估视野体积随时间的损失。视力和GVF体积。发病时的估计平均年龄为12.6岁（标准差，9.1岁; 95%分位数区间，6.5-36.4岁）。基于指数模型，平均射野体积损失为每年6.8%（标准差，4.5%; 95%分位数区间，1.9%-18.8%）。视野容积与双眼之间的相关性（r2 = 0.935）比最佳矫正视力（r2 = 0.285）更强。GVF数据的体积分析能够量化无脉络膜患者的外周视觉功能并评估疾病进展。这里提出的方法可能有助于分析遗传性视网膜疾病和其他与视野丧失相关的疾病患者的历史GVF数据。这项工作为在无脉络膜治疗试验中建立适当的结局指标提供了信息，特别是在试验设计中。专有或商业披露可以在本文末尾的脚注和披露中找到。
Choroideremia is an X-linked choroidopathy caused by pathogenic variants in the CHM gene. It is characterized by the early appearance of multiple scotomas in the peripheral visual field that spread and coalesce, usually sparing central vision until late in the disease. These features make quantitative monitoring of visual decline particularly challenging. Here, we describe a novel computational approach to convert Goldmann visual field (GVF) data into quantitative volumetric measurements. With this approach, we analyzed visual field loss in a longitudinal, retrospective cohort of patients with choroideremia. Single-center, retrospective, cohort study. We analyzed data from 238 clinic visits of 56 molecularly-confirmed male patients with choroideremia from 41 families (range, 1–27 visits per patient). Patients had a median follow up of 4 years (range, 0–56 years) with an age range of 5 to 76 years at the time of their visits. Clinical data from molecularly-confirmed patients with choroideremia, including GVF data, were included for analysis. Goldmann visual field records were traced using a tablet-based application, and the 3-dimensional hill of vision was interpolated for each trace. This procedure allowed quantification of visual field loss from data collected over decades with differing protocols, including different or incomplete isopters. Visual acuity (VA) data were collected and converted to logarithm of the minimum angle of resolution values. A delayed exponential mixed-effects model was used to evaluate the loss of visual field volume over time. Visual acuity and GVF volume. The estimated mean age at disease onset was 12.6 years (standard deviation, 9.1 years; 95% quantile interval, 6.5–36.4 years). The mean field volume loss was 6.8% per year (standard deviation, 4.5%; 95% quantile interval, 1.9%–18.8%) based on exponential modeling. Field volume was more strongly correlated between eyes (r2 = 0.935) than best-corrected VA (r2 = 0.285). Volumetric analysis of GVF data enabled quantification of peripheral visual function in patients with choroideremia and evaluation of disease progression. The methods presented here may facilitate the analysis of historical GVF data from patients with inherited retinal disease and other diseases associated with visual field loss. This work informs the creation of appropriate outcome measures in choroideremia therapeutic trials, particularly in trial designs. Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.
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DOI：
10.1007/978-3-030-27378-1_23
发表时间：
2019-01-01
期刊：
RETINAL DEGENERATIVE DISEASES: MECHANISMS AND EXPERIMENTAL THERAPY
影响因子：
--
作者：
Foote, Katharina G.;Roorda, Austin;Duncan, Jacque L.
通讯作者：
Duncan, Jacque L.
影响因子：
1.7
作者：
Bagdonaite-Bejarano, Laura;Hansen, Ronald M.;Fulton, Anne B.
通讯作者：
Fulton, Anne B.
DOI：
10.1073/pnas.86.19.7510
发表时间：
1989-10-01
影响因子：
11.1
作者：
CREMERS, FPM;VANDEPOL, DJR;ROPERS, HH
通讯作者：
ROPERS, HH
影响因子：
4.4
作者：
Dimopoulos, Ioannis S.;Tseng, Calvin;MacDonald, Ian M.
通讯作者：
MacDonald, Ian M.
影响因子：
13.7
作者：
Aleman TS;Han G;Serrano LW;Fuerst NM;Charlson ES;Pearson DJ;Chung DC;Traband A;Pan W;Ying GS;Bennett J;Maguire AM;Morgan JI
通讯作者：
Morgan JI