Spatial Characteristics of Peripheral Visual Islands in Retinitis Pigmentosa.
Spatial Characteristics of Peripheral Visual Islands in Retinitis Pigmentosa.
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DOI：
10.1167/iovs.63.2.26
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发表时间：
2022-02-01
影响因子：
4.4
通讯作者：
Singh MS
中科院分区：
文献类型：
--
作者：
Patel TP;Vongsachang H;Schilling A;Kong X;Singh MS
视网膜色素变性(RP)是以进行性周围视野(PVF)丧失为特征的，其模式因人而异。对PVF保存的更多了解可能有助于对治疗靶点的研究。然而，留置性PVF的特征尚不完全清楚。我们的目的是评价RP中残留的PVF的空间特征。我们开发了一个计算平台，使用Goldmann V4e等视线生成保留的PVF基因座空间分布的概率图。RP受试者被分成横截面数据集和纵向数据集。根据症状性疾病持续时间(SDD)类别生成PVF滞留概率图。我们应用一个数学模型来确定保留的PVF的解剖相关性。本研究共纳入152名受试者。平均年龄46.7岁。SDD年龄为20岁(47.4%)、20~40岁(39.5%)或40岁(13.2%)。有65名受试者获得了纵向数据(3.2-5.7年的随访)。在横断面数据集中，保留的PVF位点最有可能位于50°~80°等偏心经线之间和30°~50°放射轴之间。在纵向数据集中，颞下PVF位点最有可能随着时间的推移而保存下来。PVF滞留的区域在解剖学上与赤道前鼻上视网膜相对应。室性心动过速的半自动定量分析可能是分析室性心动过速的空间特征的有用工具。鼻上区的视网膜细胞可能对RP相关的功能衰退有弹性。了解视网膜PVF弹性的细胞和分子基础可能有助于开发RP的治疗方法。
Retinitis pigmentosa (RP) is typified by progressive peripheral visual field (pVF) loss in patterns that can vary between individuals. Greater understanding of pVF preservation may inform research on therapeutic targets. However, characteristics of retained pVF are incompletely understood. We aimed to evaluate the spatial characteristics of retained pVF in RP. We developed a computational platform to generate a probability map of the spatial distribution of retained pVF loci using the Goldmann V4e isopter. RP subjects were grouped into cross-sectional and longitudinal datasets. Probability maps of retained pVF were generated for categories of symptomatic disease duration (SDD). We applied a mathematical model to determine the anatomical correlate of the retained pVF. A total of 152 subjects were included. The mean age was 46.7 years. SDD was <20 years (47.4%), 20 to 40 years (39.5%), or >40 years (13.2%). Longitudinal data (3.2–5.7 years of follow up) were available for 65 subjects. In the cross-sectional dataset, retained pVF loci were most likely to be located between the 50° and 80° isoeccentric meridians and between the 30° to 50° radial axes. In the longitudinal dataset, inferotemporal pVF loci were the most likely to be preserved over time. The area of pVF retention corresponded anatomically to the pre-equatorial superonasal retina. Semiautomated quantitation of pVF may be a useful tool to analyze spatial characteristics of VF in RP. Retinal cells in the superonasal periphery may be resilient to RP-related functional decline. Understanding the cellular and molecular basis of pVF resilience in the retina may inform efforts to develop treatment modalities for RP.
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影响因子：
4.2
作者：
WELEBER, RG;TOBLER, WR
通讯作者：
TOBLER, WR
影响因子：
4.2
作者：
BERSON, EL;SANDBERG, MA;HANSON, AH
通讯作者：
HANSON, AH
影响因子：
5.3
作者：
Odaka, Tomohiro;Fujisawa, Kimihiko;Nose, Yoshiaki
通讯作者：
Nose, Yoshiaki
影响因子：
13.7
作者：
Grover, S;Fishman, GA;Derlacki, DJ
通讯作者：
Derlacki, DJ
DOI：
10.1038/gim.2015.30
发表时间：
2015-05
期刊：
Genetics in medicine : official journal of the American College of Medical Genetics
影响因子：
--
作者：
Richards S;Aziz N;Bale S;Bick D;Das S;Gastier-Foster J;Grody WW;Hegde M;Lyon E;Spector E;Voelkerding K;Rehm HL;ACMG Laboratory Quality Assurance Committee
通讯作者：
ACMG Laboratory Quality Assurance Committee