Kinetics of visual field loss in Usher syndrome type II
Kinetics of visual field loss in Usher syndrome type II
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DOI：
10.1167/iovs.03-0906
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发表时间：
2004-03-01
影响因子：
4.4
通讯作者：
Somes, GW
中科院分区：
文献类型：
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作者：
Iannaccone, A;Kritchevsky, SB;Somes, GW
目的。为探讨Usher综合征II型患者视野衰退的动力学特征。方法对19例确诊为II型Usher综合征的患者，测量以I4e和V4e为靶点的137个Goldmann视野(GVF)的面积，计算各时间点的平均眼间GVF面积。平均随访5.58年。有症状的疾病持续时间被定义为首次发现症状后的几年。用随机系数混合模型分析数据集(We靶点n=67；V4e靶点n=70)，以确定描述视野大小随时间衰减的最佳模型。还计算了残留视野区域的半衰期(t(0.5))。结果：最能解释GVF区域衰退的变量是有症状的疾病持续时间。在指数模型中，对于每一年的症状性疾病，GVF地区自然对数的斜率估计对于We目标是-0.172，对于V4e目标是-0.136。因此，t(0.5)对于I4e目标约为4年，对于V4e目标约为5年。这些估计与以前对非综合征性视网膜色素变性(RP)的研究非常相似。结论：这项研究表明，平均而言，Usher综合征11型患者GVF下降的动力学与其他形式的RP非常相似，一旦疾病出现症状，GVF恶化遵循固定的动力学规律，即使是在晚发性视网膜疾病患者中也是如此。
PURPOSE. To characterize the kinetics of visual field decay in Usher syndrome type II.METHODS. The area of 137 Goldmann visual fields (GVFs) delimited with the I4e and V4e targets was measured in each eye of 19 patients with an established diagnosis of Usher syndrome type II, and the average interocular GVF area for each patient at each time point was calculated. The average follow-up was 5.58 years. Symptomatic disease duration was defined as years elapsed after symptoms were first noted. The data set (n = 67 for the We target; n = 70 for the V4e target) was analyzed with a random coefficient mixed model to identify the best-fit model describing the decay of visual field size over time. The half-life of the residual visual field area (t(0.5)) was also calculated.RESULTS. The variable that best explained the decay of the GVF area was the duration of symptomatic disease. In an exponential model, the slope estimate for the natural log of the GVF area was -0.172 for the We target and -0.136 for the V4e target for each year of symptomatic disease. Accordingly, t(0.5) was approximately 4 years for the I4e target and 5 years for the V4e target. These estimates are very similar to those in previous studies of nonsyndromic retinitis pigmentosa (RP).CONCLUSIONS. This study suggests that the kinetics of GVF decline in Usher syndrome type 11 are, on average, very similar to other forms of RP and that, once the disease becomes symptomatic, GVF deterioration follows stereotyped kinetics, even in patients with late-onset retinal disease.