The Progression of Stargardt Disease as Determined by Spectral-Domain Optical Coherence Tomography over a 24-Month Period (ProgStar Report No. 18).

IF 2 4区医学 Q2 OPHTHALMOLOGY

Ophthalmic Research Pub Date : 2024-01-01 Epub Date: 2024-07-18 DOI:10.1159/000540028

Rupert Wolfgang Strauss, Lang Lang, Alexander Ho, Anamika Jha, Michael Ip, Paul S Bernstein, David G Birch, Artur V Cideciyan, Michel Michaelides, Isabelle Audo, Janet S Sunness, Elias I Traboulsi, Eberhart Zrenner, SriniVas R Sadda, Lucas Janeschitz-Kriegl, Sheila West, Xiangrong Kong, Hendrik P N Scholl

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引用次数: 0

Abstract

Introduction: The aim of this study was to evaluate the progression of atrophy as determined by spectral-domain optical coherence tomography (SD-OCT) in patients with molecularly confirmed ABCA4-associated Stargardt disease type 1 (STGD1) over a 24-month period in a multicenter prospective cohort study.

Methods: SD-OCT images from 428 eyes of 236 patients were analyzed. Change of mean thickness (MT) and intact area were estimated after semiautomated segmentation for the following individual layers in the central subfield (CS), inner ring (IR), and outer ring (OR) of the ETDRS grid: retinal pigment epithelium (RPE), outer segments (OSs), inner segments (IS), outer nuclear layer (ONL) inner retina (IR), and total retina.

Results: Statistically significant decreases of all outer retinal layers (RPE, OS, IS, and ONL) could be observed over a 24-month period both in decline of mean retinal thickness and intact area (p < 0.0001, respectively), whereas the IR showed an increase of retinal thickness in the CS and IR and remained unchanged in the OR.

Conclusions: Significant loss could be detected in outer retinal layers by SD-OCT over a 24-month period in patients with STGD1. Loss of thickness and/or intact area of such layers may serve as potential endpoints for clinical trials that aim to slow down the disease progression of STGD1.

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通过光谱域光学相干断层扫描测定的为期 24 个月的斯塔加特病（ProgStar）进展情况（ProgStar 第 19 号报告）。

简介在一项多中心前瞻性队列研究中，通过光谱域光学相干断层扫描（SD-OCT）评估分子确诊的 ABCA4 相关性斯塔加特病 1 型（STGD1）患者在 24 个月内眼球萎缩的进展情况：分析了 236 名患者 428 只眼睛的 SD-OCT 图像。在对 ETDRS 网格的中央子场 (CS)、内环 (IR) 和外环 (OR) 的以下各层进行半自动分割后，估算了平均厚度 (MT) 和完整面积的变化：视网膜色素上皮 (RPE)、外节段 (OS)、内节段 (IS)、外核层 (ONL) 视网膜内层 (IR) 和视网膜总层 (TR)：结果：在 24 个月的时间里，所有视网膜外层（RPE、OS、IS 和 ONL）的平均视网膜厚度和完整面积都出现了统计学意义上的明显下降（分别为 p<.0001）；而视网膜内层在中央子场和内环的视网膜厚度有所增加，在外环则保持不变：结论：SD-OCT 可以检测到 STGD1 患者视网膜外层在 24 个月的时间内有明显的缺失。这些视网膜外层的厚度和/或完整面积的损失可作为旨在减缓 STGD1 疾病进展的临床试验的潜在终点。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Ophthalmic Research 医学-眼科学

CiteScore

3.80

自引率

4.80%

发文量

审稿时长

6-12 weeks

期刊介绍： ''Ophthalmic Research'' features original papers and reviews reporting on translational and clinical studies. Authors from throughout the world cover research topics on every field in connection with physical, physiologic, pharmacological, biochemical and molecular biological aspects of ophthalmology. This journal also aims to provide a record of international clinical research for both researchers and clinicians in ophthalmology. Finally, the transfer of information from fundamental research to clinical research and clinical practice is particularly welcome.