Long-term visual prognoses in patients with retinitis pigmentosa: The Ludwig von Sallmann lecture
Long-term visual prognoses in patients with retinitis pigmentosa: The Ludwig von Sallmann lecture
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DOI：
10.1016/j.exer.2007.03.001
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发表时间：
2007-07-01
影响因子：
3.4
通讯作者：
Berson, Eliot L.
中科院分区：
文献类型：
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作者：
Berson, Eliot L.
视网膜色素变性可以通过窄带通计算机平均锥形视网膜电信号(ERG)在几乎整个过程中得到跟踪。这些反应的长期递减率可以用指数函数来描述。基于1039名患者和6553次来访的视锥ERG精算表，显示了给定30赫兹视锥ERG幅度下降到0.05微伏(即虚拟失明)的平均患者的估计年数。该表是基于未接受治疗的患者和接受维生素A棕榈酸酯治疗的患者每年剩余视锥ERG幅度的10%和8.3%的预测丢失率。该表可以用来提供一次就诊的平均长期视力预后的估计；对特定患者的更准确的估计需要在2至3年的间隔内额外就诊几次。有证据支持这一观点，即40岁时视锥振幅为3.5-V或更大的患者(约占典型视网膜色素变性患者的25%)平均有望在不接受治疗的情况下在其一生中保留一些有用的视力。了解ERG中剩余锥体功能的数量通常会减少患者的焦虑，并帮助患者规划未来。(C)2007爱思唯尔有限公司。保留所有权利。
Retinitis pigmentosa can be followed over almost its entire course with narrow bandpassed, computer averaged cone electroretinograms (ERGs). The long-term rate of decline of these responses can be described by an exponential function. A cone ERG actuarial table based on 1039 patients and 6553 visits is presented to show the estimated number of years for an average patient with a given 30-Hz cone ERG amplitude to decline to 0.05 mu V (i.e. virtual blindness). The table is based on a projected rate of loss of 10% of remaining cone ERG amplitude per year for those not on treatment and 8.3% per year for those on treatment with vitamin A palmitate 15,000 IU/day. The table can be used to provide an estimate of the average long-term visual prognosis from a single visit; more precise estimates for a specific patient require several additional visits over 2- to 3-year intervals. Evidence is presented to support the idea that patients with a projected cone amplitude of 3.5 mu V or greater at age 40 (about 25 % of our patient population with typical retinitis pigmentosa) would be expected, on average, to retain some useful vision for their entire lives without treatment. Knowledge of the amount of remaining cone function in the ERG often reduces patient anxiety and helps patients plan for their future. (c) 2007 Elsevier Ltd. All rights reserved.