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评估视网膜色素变性患者的残余视锥细胞功能。

Assessing Residual Cone Function in Retinitis Pigmentosa Patients.

作者信息

Arsiwalla Tasneem A, Cornish Elisa E, Nguyen Phuc Vuong, Korsakova Maria, Ali Haipha, Saakova Nonna, Fraser Clare L, Jamieson Robyn V, Grigg John R

机构信息

Save Sight Institute, The Discipline of Clinical Ophthalmology and Eye Health, Faculty of Medicine and Health, The University of Sydney, Sydney, New South Wales, Australia.

Sydney Eye Hospital Foundation, University of Sydney, Sydney, New South Wales, Australia.

出版信息

Transl Vis Sci Technol. 2020 Dec 17;9(13):29. doi: 10.1167/tvst.9.13.29. eCollection 2020 Dec.

DOI:10.1167/tvst.9.13.29
PMID:33364083
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7746956/
Abstract

PURPOSE

The purpose of this study was to investigate cone function deterioration in patients with retinitis pigmentosa (RP) using full field electroretinogram (ffERG), pattern electroretinogram (pERG), and optical coherence tomography (OCT) and their correlation with visual acuity (VA).

METHODS

Clinical records (2008-2018) of patients with RP undergoing repeat electrophysiology were reviewed. Results of ffERG (30 Hz flicker and fused flicker amplitude [FFAmp]), pERG [p50 and n95], and macular OCT (ellipsoid zone [EZ] and outer segment thickness) were collected.

RESULTS

One hundred twenty-six eyes from 63 patients (33 women, mean age 35 years) were included. The mean decline in VA was 0.11 ± 0.14 logarithm of minimum angle of resolution (logMAR). The FFAmp decreased by 3.01 ± 5.9 µV with global cone function deteriorating by 18.7% annually. The percentage change in FFAmp (RE [ = 0.553], LE [ = 0.531]), and 30 Hz flicker amplitude (RE [ = 0.615], LE [ = 0.529]) strongly correlated with VA ( < 0.00001). The pERG p50 (15 and 30 degrees) change analyzed in 34 patients showed reduction by 23% and 23.4%, respectively. The percentage change in p50 30 degrees ( = 0.397) correlated with VA and EZ layer ( < 0.05). The EZ layer change was calculated in 45 patients and the shortening and thinning rate was 4.3% and 4.4% annually, respectively. The EZ length percentage change correlated with VA (RE [ = 0.34] and LE [ = 0.466; < 0.05).

CONCLUSIONS

We quantified the decline in cone function in patients with RP utilizing ffERG and FFAmp measures of residual cone function. These parameters correlated with VA and OCT when measurable. These objective measures may assist in monitoring disease progression.

TRANSLATIONAL RELEVANCE

Residual cone function provides an objective estimate of residual visual function, which aids in counselling patients regarding prognosis.

摘要

目的

本研究旨在使用全视野视网膜电图(ffERG)、图形视网膜电图(pERG)和光学相干断层扫描(OCT)研究视网膜色素变性(RP)患者的视锥细胞功能衰退情况及其与视力(VA)的相关性。

方法

回顾了2008年至2018年接受重复电生理检查的RP患者的临床记录。收集了ffERG(30Hz闪烁和融合闪烁振幅[FFAmp])、pERG[p50和n95]以及黄斑OCT(椭圆体带[EZ]和外段厚度)的结果。

结果

纳入了63例患者(33名女性,平均年龄35岁)的126只眼。视力的平均下降为0.11±0.14最小分辨角对数(logMAR)。FFAmp下降了3.01±5.9µV,整体视锥细胞功能每年衰退18.7%。FFAmp的百分比变化(右眼[=0.553],左眼[=0.531])以及30Hz闪烁振幅(右眼[=0.615],左眼[=0.529])与视力密切相关(<0.00001)。对34例患者分析的pERG p50(15度和30度)变化分别显示下降了23%和23.4%。30度p50的百分比变化(=0.397)与视力和EZ层相关(<0.05)。计算了45例患者的EZ层变化,每年缩短和变薄率分别为4.3%和4.4%。EZ长度百分比变化与视力相关(右眼[=0.34],左眼[=0.466;<0.05])。

结论

我们利用ffERG和残余视锥细胞功能的FFAmp测量方法对RP患者的视锥细胞功能衰退进行了量化。这些参数在可测量时与视力和OCT相关。这些客观测量方法可能有助于监测疾病进展。

转化相关性

残余视锥细胞功能提供了残余视觉功能的客观估计,有助于向患者提供预后咨询。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/094d/7746956/4a4b68ee85d5/tvst-9-13-29-f007.jpg
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2
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3
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4
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5
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6
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10
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