Study on the correlation between rate of visual field loss and gene abnormality in retinitis pigmentosa

视网膜色素变性是由多种基因异常引起的。由于该病的基础治疗尚未建立，有关疾病进展和视力预后的信息可能对患者很重要。在本研究中，我们提出了使用自动静态视野检查来定量评估疾病进展的方法，研究了RP患者的视野检查结果，并使用HFA文件VER分析了Humphrey视野检查程序30-2和10-2的平均偏差(MD)进展率。5(东京直达办公公司)。在65例(126只眼)接受Humphrey视野检查5年或以上的RP患者中，68只眼(54%)的MD在随访期内有统计学意义的下降。30-2方案的MD进展率为0.11~3.1分贝/年，平均0.93分贝/年。10-2程序的视力损失范围为0.19~2.05，平均0.64分贝/年。然后研究视力损失与视野进展的关系。大多数眼在Humphrey视野检查中心10-2程序的MD为-15db时仍保持良好的视力，但在此之后，部分病例开始出现视力下降的现象。视力与矫正图样标准差(CPSD)有良好的相关性。CPSD值高的眼，即视野受损不均匀时，在视野的慢径处表现为近乎正常的敏感度，在中心视野10度的周边出现绝对暗点。

Retinitis pigmentosa is caused by various gene abnormality. Because the fundamental treatment for the disease has not been established, the information on the disease progression and visual prognosis may be important for the patients. In the present study, we proposed the method of quantitative evaluation for the disease progression using the automated static perimetry.We studied the perimetry results of patients with RP and analyzed the progression rate of the mean deviation (MD) of Humphrey perimetry program 30-2 and 10-2 using HFA files ver. 5 (BeeLine Office Co. Tokyo). Out of 126 eyes of 65 patients with RP who had been examined using Humphrey perimetry for 5 years or more, 68 eyes (54%) demonstrated statistically significant reduction of MD during the follow-up period. The progression rate of MD in 30-2 program ranged between 0.11 and 3.1 dB/year with the average of 0.93 dB/year in 26 eyes. That for 10-2 program ranged between 0.19 and 2.05 with the average of 0.64 dB/year in 51 eyes.Then we studied the relationship between visual loss and the perimetric progression. Most eyes maintain good visual acuity until the MD of Humphrey perimetry central 10-2 program is -15dB, while after that, some cases start to show decreased visual acuity. Eyes with MD of -15 dB of worse, the visual acuity and corrected pattern standard deviation (CPSD) showed good correlation. Eyes with high value of CPSD, which means unevenly damaged visual field showing almost normal sensitivity at the canter of the field and absolute scotoma in the periphery of the 10-degree central field.