使用微视野衍生指标对 RPGR 相关性视网膜病变的成人和儿童的视网膜功能进行特征描述。
Characterization of Retinal Function Using Microperimetry-Derived Metrics in Both Adults and Children With RPGR-Associated Retinopathy.
机构信息
Moorfields Eye Hospital (E.A., M.G., J.T., A.R.B., M.M.), London, United Kingdom; From the University College London Institute of Ophthalmology (E.A., M.G., J.T., A.R.B., M.M.), University College London, London, United Kingdom.
Moorfields Eye Hospital (E.A., M.G., J.T., A.R.B., M.M.), London, United Kingdom; From the University College London Institute of Ophthalmology (E.A., M.G., J.T., A.R.B., M.M.), University College London, London, United Kingdom; Department of Ophthalmology, University of Arkansas Medical Sciences, Little Rock, Arkansas, USA.
出版信息
Am J Ophthalmol. 2022 Feb;234:81-90. doi: 10.1016/j.ajo.2021.07.018. Epub 2021 Jul 22.
PURPOSE
To investigate microperimetry testing of retinitis pigmentosa GTPase regulator gene (RPGR)-associated retinopathy in a cohort of children and adults.
DESIGN
Prospective observational case series.
METHODS
The coefficient of repeatability and intraclass correlation coefficient (ICC) of mean sensitivity (MS) were calculated for mesopic microperimetry. Best-corrected visual acuity (BCVA), contrast sensitivity (CS), MS, total volume (V), and central 3-degree field volume (V) from volumetric and topographic analyses were acquired.
RESULTS
The study recruited 76 individuals with RPGR (53 adults, 23 children). The mean follow-up period was 2.8 years. The ICC values for MS, V, and V were 0.982 dB (95% CI, 0.969-0.989 dB), 0.970 dB-steradian (sr) (95% CI, -0.02658 to 0.03691 dB-sr), and 0.986 dB-sr (95% CI, 0.978-0.991), respectively. The r values for interocular MS, V, and V were 0.97 (P < .01), 0.97 (P < .01), and 0.98 (P < .01), respectively, indicating strong interocular correlation. The interocular correlation of progression for MS, V, and V was 0.81 (P < .01), 0.64 (P < .01), and 0.81 (P < .01), respectively. There was no statistically significant difference in the interocular progression rates for MS or V. V did show a statistically significant difference. Most patients lost retinal sensitivity rapidly during their second and third decades of life.
CONCLUSIONS
The high degree of reproducibility of results and the good interocular correlation lends this method to accurately monitoring disease progression, as well as supporting validation of the use of MP in assessing the outcomes of gene therapy clinical treatment trials.
目的
研究微视野计检测视蛋白相关视网膜色素变性 GTP 酶调节基因（RPGR）相关视网膜病变在一组儿童和成人中的应用。
设计
前瞻性观察性病例系列。
方法
计算中值微视野计的重复性系数和组内相关系数（ICC）。获取最佳矫正视力（BCVA）、对比敏感度（CS）、平均敏感度（MS）、总体积（V）和容积及拓扑分析的中心 3 度视野体积（V）。
结果
本研究共纳入 76 名 RPGR 患者（53 名成人，23 名儿童）。平均随访时间为 2.8 年。MS、V 和 V 的 ICC 值分别为 0.982dB（95%CI：0.969-0.989dB）、0.970dB-立体弧度（sr）（95%CI：-0.02658 至 0.03691dB-sr）和 0.986dB-sr（95%CI：0.978-0.991）。双眼间 MS、V 和 V 的 r 值分别为 0.97（P<0.01）、0.97（P<0.01）和 0.98（P<0.01），表明双眼间具有很强的相关性。MS、V 和 V 的双眼间进展相关性分别为 0.81（P<0.01）、0.64（P<0.01）和 0.81（P<0.01）。MS 或 V 的双眼间进展率无统计学差异。V 确实存在统计学差异。大多数患者在生命的第二和第三个十年快速丧失视网膜敏感性。
结论
结果具有很高的可重复性，双眼间相关性良好，这使得该方法能够准确监测疾病进展，并支持使用 MP 评估基因治疗临床试验的结果。
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