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遗传性视网膜营养不良的早期视觉症状模式。

Early visual symptom patterns in inherited retinal dystrophies.

机构信息

Institute for Ophthalmic Research, Centre for Ophthalmology, University of Tuebingen, Tuebingen, Germany.

出版信息

Ophthalmologica. 2011;226(3):151-6. doi: 10.1159/000330381. Epub 2011 Aug 10.

Abstract

The present retrospective study compared initial visual symptom patterns in inherited retinal dystrophies (IRD) on the basis of records of 544 patients diagnosed with a wide variety of IRD at the Tuebingen University Eye Hospital from 2005 to 2008. Age at first onset of symptoms was noted, and the following clinical data were analyzed: visual acuity (VA), night vision disturbances, photophobia, onset of visual field defects, best corrected VA, and types of visual field defects. Median age at visual symptom onset was defined with 25th and 75th percentiles and compared in 15 IRD types. The main trends in VA changes in retinitis pigmentosa and cone-rod dystrophies were identified. This study was the first to combine disease history and clinical data analysis in such a wide variety of IRD. It showed that patterns of initial symptoms in IRD can provide extra clues for early differential diagnosis and inclusion of IRD patients in clinical trials.

摘要

本回顾性研究比较了 2005 年至 2008 年在图宾根大学眼科医院诊断的 544 例各种遗传性视网膜疾病(IRD)患者的初始视觉症状模式。记录了症状首次出现的年龄,并分析了以下临床数据:视力(VA)、夜间视力障碍、畏光、视野缺损的出现、最佳矫正视力和视野缺损的类型。通过 25%和 75%分位数定义了中位年龄,并在 15 种 IRD 类型中进行了比较。确定了色素性视网膜炎和锥-杆营养不良中 VA 变化的主要趋势。这项研究首次将病史和临床数据分析结合在如此广泛的 IRD 中。它表明,IRD 初始症状模式可为早期鉴别诊断和将 IRD 患者纳入临床试验提供额外线索。

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