Visual field mean deviation and relevant factors in 928 Chinese retinitis pigmentosa patients

AIM: To investigate the associations between demographic and clinical factors with the rate of visual field mean derivation (MD) decline in retinitis pigmentosa (RP) patients. METHODS: Correlations of MDs with the visual acuity and retinal pigmentation were analyzed in 928 RP patients. MD decreasing rate in 10y and potential influences of gender, age, family history and retinal pigmentation on the rate were explored in 201 RP patients. RESULTS: In the 928 patients, average MD and visual acuity were -14.44±8.61 dB and 0.79±0.35 respectively and when MD was lower than -9.18 dB the visual acuity would be below 1.0 (20/20). The average MD medium between eyes with or without retinal pigmentation was -14.82 dB. In 123 non-pigmented eyes, the average MD were lower than the medium but in 153 pigmented eyes it was higher than that. In the 201 patients, the average decreasing value of MD in 10 years’ period was -8.01±3.66 dB and the value were correlated to retinal pigmentation but not to gender, age or RP family history. CONCLUSION: The rate of MD decline in RP eyes is significantly related to retinal pigmentation. Our study demonstrates the quantitative rate of MD decline in RP patients and the value of MD could well reflect the severity of RP.     

视网膜色素变性的多焦视网膜电图

目的研究视网膜色素变性（ＲＰ）的多焦视网膜电图（ＥＲＧ）特性和评价多焦ＥＲＧ应用价值。方法检测了 １２例（２４眼）ＲＰ患者的多焦 ＥＲＧ,测试野为２６．６°×２２．１°,记录 １０３个部位的反应,同时检测全视野 ＥＲＧ。结果ＲＰ眼在各个环的ａ波和ｂ波潜伏期、ｂ波振幅密度和振幅之和均较正常值有显著延长和降低（Ｐ＜０．０５或Ｐ＜０．０１）。５０％患眼在第１环可记录到潜伏期正常、振幅密度和总和均降低的ｂ波,而７５％患眼的全视野ＥＲＧ呈平坦反应。结论 多焦ＥＲＧ能更有效地评价ＲＰ的黄斑区功能。     

视网膜色素变性的研究进展

视网膜色素变性(retinitis pigmentosa,RP)是常见的与遗传相关的致盲性眼病之一,随着人类基因图谱的成功建立,它成为近几年国内外基础医学和临床医学共同的研究热点。本文回顾十余年来国内外特别是国外相关文献,对视网膜色素变性的发病机制、治疗方法的研究进展作一综述。     

Red glasses and visual function in retinitis pigmentosa

As a consequence of animal studies reduction of the light regimes have been tried on patients with retinitis pigmentosa. The trials have been very limited and have not given reason for hope that such reduced light therapy may be beneficial. However, RP patients trying red glasses have reported acute subjective improvement of their visual function. It was the purpose of this study to try to document the reports more objectively. Five visual functions were tested with and without red glasses with the following results: 1. Visual acuity and contrast sensitivity. For one volunteer a small reproducible improvement was found. 2. Color vision. In most cases deterioration was found of already deficient color vision. 3. Visual fields. For volunteers with relatively preserved vision no difference or slight deterioration was found. For the other volunteers slight improvements were found. 4. Intraocular light scatter. No differences were found. 5. Dark adaptation. Improvements were found when the glasses were used as adaptation aid according to the method of Trendelenburg (rod function). For cone function no difference was found upon continuous wearing of red glasses.In conclusion: use of red glasses does not seem to be of great benefit as a rule. On the other hand, apart from the reduction in color discrimination no serious disadvantages seem to be inherent in their wear by RP patients.     

超声乳化白内障吸出联合人工晶状体植入治疗视网膜色素变性合并白内障

目的 探讨超声乳化白内障吸出联合人工晶状体植入治疗视网膜色素变性合并白内障的疗效.方法 回顾性研究.对2012年1月至2016年12月在乐山市人民医院眼科确诊为原发性视网膜色素变性合并白内障患者给予超声乳化白内障吸出联合人工晶状体植入术52例(101眼)患者的临床资料进行回顾性分析.所有患者治疗前后均进行最佳矫正视力、眼压、裂隙灯前节检查、睫状肌麻痹下眼底检查以及光学相干断层扫描检查.白内障手术均顺利完成,术后随访3 ～ 12(5.09±2.20)个月.患者手术前、手术后各指标的比较用配对t检验,视力提高幅度与黄斑厚度的相关关系采用Pearson相关检验.结果 101眼患眼最佳矫正视力由术前0.12±0.09提高至术后0.21±0.16,差异有统计学意义(t=-8.016,P=0.000).术后视力与黄斑厚度有明显相关性:对于黄斑囊样水肿及黄斑前膜,术后视力提高行数和黄斑厚度呈负相关(r=-0.866,P=0.001),对于黄斑萎缩,两者呈正相关(r=0.928,P=0.000).2眼在白内障术后出现前囊膜收缩,2眼出现继发性高眼压,给予前囊膜放射状切开、药物控制眼压平稳后,视力提高且长期趋于稳定.结论 超声乳化白内障吸出联合人工晶状体植入术可以提高患者视力,是治疗视网膜色素变性合并白内障安全有效的方法.     

视网膜色素变性1基因在常染色体显性遗传视网膜色素变性家系中的突变分析

目的:研究常染色体显性遗传视网膜色素变性(autosomal dominant retinitis pigmentosa,ADRP)家系中视网膜色素变性1(retinitis pigmentosa-1,RP1)基因的突变特征及其在RP发病机制中的作用。方法:运用聚合酶链反应和直接测序方法,对6个ADRP家系的47例成员和50例对照者进行了RP1基因全编码区和邻近剪切位点的内含子区域序列突变的筛选与检测。运用单因素分析、多因素Logistic回归分析研究RP1基因点突变在RP发病中的作用。结果:ADRP家系成员和对照组RP1基因第4外显子上检测出2个变异位点。在1691和1725密码子存在杂合的两种类型的密码子(S1691P,Ser-Pro,TCT→CCT;Q1725Q,Gln-Gln,CAA→CAG)。ADRP家系成员中Ser-1691-Pro及Gln-1725-Gln位点突变率显著高于正常对照组(χ2=11.202,P<0.05)。结论:RP1基因Ser-1691-Pro及Gln-1725-Gln位点多态性可增高RP的危险性,具有潜在的致病性,考虑为ADRP家系的易感基因。     

视网膜色素变性合并白内障患者人工晶状体植入术

目的 探讨白内障人工晶体状体植入术对视网膜色素变性合并白内障患者治疗的手术技巧及效果。方法 对15例18眼视网膜色素变性合并白内障患者作囊外摘出联合后房型人工晶状体植入或超声乳化吸出晶状体并植入软性人工晶状体。对手术前后视力及并发症进行观察,并追踪观察视功能改变。结果 术后16眼视力较术前提高,视野部分有所改善,ERG无明显改变。结论 白内障人工晶状体植入术对大部分视网膜色素变性并发白内障患者的视力提高有帮助,是一种较为安全有效的治疗方法。     

Visual field expansion in patients with retinitis pigmentosa

BACKGROUND: To determine the effectiveness of using spectacle-mounted prisms for field expansion in patients with retinitis pigmentosa (RP). METHODS: Vision-related activities of daily living (V-ADL) questionnaire scores and functional visual field score (FFS) measurements were conducted before and after a one-month trial of spectacle-mounted prisms in those patients with RP who had residual central visual fields of less than 10 degrees. RESULTS: 16 patients were recruited who met study inclusion criteria. Mean V-ADL and FFS at baseline were 67.6 (73%) and 22.9 (46%), respectively. After a 1-month trial using spectacle-mounted prisms, V-ADL and FFS demonstrated significant improvement to 73.4 (80%, p < 0.05) and 27.0 (54%, p < 0.001), respectively. INTERPRETATION: Spectacle-mounted prisms effectively create visual field expansion and noticeable spatial orientation benefits in patients with RP. This may provide an adjunctive tool in low vision rehabilitation and should be considered in all cases with RP with less than 10 degrees of visual field.     

Mobility performance with retinitis pigmentosa

Purpose : Reduced mobility can have a serious impact on quality of life. Though previous studies have demonstrated that some vision measures relate to the mobility of subjects with simulated and true low vision, the relationship between residual vision and mobility is not clear. We investigated the relationship between clinical vision measures and mobility performance under different illumination levels for subjects with retinitis pigmentosa (RP). Methods : Binocular visual acuities, letter contrast sensitivities and static central threshold visual fields were measured on 10 subjects with RP and nine age-matched control subjects. Mobility performance was measured on an indoor mobility course at high and low illuminances and was assessed by percentage preferred walking speed (PPWS) and number of errors. Results : The RP group showed significantly reduced PPWS and greater numbers of errors than the control group. The reduction in illumination resulted in significantly worse error and PPWS scores. Unlike the control group, the presence of a glare source did not reduce the PPWS of the RP group under high illumination. Multiple regression analyses showed that the average visual field extent was the most significant predictor of mobility; letter contrast sensitivity and visual acuity added to the regression models for the low illumination measures to account for up to 75 per cent of the variation in mobility performance. Conclusions : People with RP have worse mobility than people with normal vision, more so under reduced illumination levels. Visual field extent was the strongest predictor of mobility performance. (Clin Exp Optom 1996; 80: 1: 1–12)     

Posterior chamber intraocular lens implantation in patients with retinitis pigmentosa

20 eyes of 12 Retinitis Pigmentosa patients underwent extracapsular cataract extraction followed by I.O.L. implantation. No unusual intra or post operative complications were encountered. All pseudophakic eyes showed improvement in visual acuity whereas the V.F. remained unaltered. Subjectively all 12 patients reported a benefit from surgery despite the Visual Field impairment.     

视网膜色素变性眼肌深层巩膜移植术患者图形视觉诱发电位分析

目的探讨视网膜色素变性(RP)患者的P-VEP特征及对RP患者眼肌深层巩膜移植术进行治疗前、后P-VEP变化加以比较,为跟踪疗效提供客观依据。方法采用MVT-3型多功能和多导程视觉电生理仪,分别测定棋盘格翻转刺激全视野、中央6°视野,以及遮盖中央、刺激6°以外周边视野时的P-VEP。结果在202例RP患者中有110例(54.5%),用上述3种刺激方式均记录不到P-VEP。全视野刺激能诱发P100波的为92例(45.5%),刺激中央视野可记录到P-VEP的为83例(41.1%),而刺激周边视野能记录到P-VEP的仅为48例(23.8%)。全视野刺激记录到的P100波平均振幅,RP组明显低于正常组的相应值,有非常显著的统计学意义(P<0.001)。用上述3种刺激方式从RP患者记录到的P100波峰潜伏期,与正常组的相应值相比,都明显延长,且都有显著的统计学意义(P<0.01)。施行了眼肌深层巩膜移植术的RP患者中,有42例在术后3～22(平均10.9)月,来院进行了P-VEP复查,手术前刺激中央视野能记录到P-VEP的为20例(47.6%),术后复查时已增加到26例(61.9%)(配对χ2检验,χ2=4.17,P<0.05)。不仅人数增加,而且振幅也明显增大。结论眼肌深层巩膜移植术使患者视力有明显好转,P-VEP也有明显改善。因此P-VEP可以作为对RP患者进行疗效评估的客观指标之一。     

Colour vision in retinitis pigmentosa

In retinitis pigmentosa patients the effect of cystoid macular edema on colour vision was studied. The occurrence of cystoid macular edema decreases with increasing colour vision defect. The mutual proportion of the main types of colour vision defects remains stable until visual acuity has dropped to 0.5; at lower VA levels the number of red-green defects increases. Neither the finding of a blue-yellow colour vision defect in FM 100 Hue testing nor the appearance of anomaloscopic pseudoprotanomaly is influenced by cystoid macular edema. The authors conclude that cystoid macular edema in retinitis pigmentosa patients mainly affects visual acuity and not colour vision. They also noted a familial occurrence of cystoid macular edema.     

后巩膜加固术联合超声乳化人工晶状体植入术治疗视网膜色素变性合并白内障

目的探讨后巩膜加固术联合超声乳化人工晶状体植入术治疗视网膜色素变性(retinitispigmentosa,RP)合并白内障的临床疗效。方法 对RP合并白内障患者16例(30眼)进行回顾性分析,所有患者先行后巩膜加固术,再行白内障超声乳化人工晶状体植入术,术后1周、1个月、3个月、6个月分别复查视力,定期复查视野、视网膜电图。结果 患者术后视力提高≥2行者24眼,<2行者4眼,无提高者2眼;术前、术后1周、1个月、3个月、6个月视力差异有统计学意义(P<0.05或0.01)。术后第1天角膜内皮轻微水肿3眼,前房Tyndall征阳性5眼,经皮质类固醇类激素滴眼液局部滴眼后,均于2～7d内消退。术前、术后眼压均正常,无一过性高眼压,未发生人工晶状体移位、角膜内皮失代偿、视网膜脱离等严重并发症。与术前比较,所有患眼术后视野范围扩大或光敏感度提高。视网膜电图与术前无明显差异。结论 后巩膜加固术联合超声乳化人工晶状体植入术是治疗RP合并白内障的一种安全、有效的方法。     

光学相干断层扫描（OCT）在视网膜色素变性患者白内障手术视力预后判断中的应用

目的　利用光学相干断层扫描（optical coherence tomography，OCT）判断视网膜色素变性（retinitis pigmentosa，RP）患者白内障手术后视力预后情况。方法　回顾性分析16例（24眼）RP合并白内障患者行白内障超声乳化吸出联合人工晶状体植入术的临床结果。观察患者术前合并黄斑病变和术后发生后囊膜混浊情况，分析白内障手术前后最佳矫正视力（best corrected visual acuity，BCVA）的变化和相关性。根据OCT的椭圆体带（ellipsoidal zone，EZ）结构完整性将RP患者分为EZ结构完全缺失组（Grade1组）、EZ结构异常或部分缺失组（Grade2组）和EZ结构基本正常组（Grade3组），观察各组术后BVCA。结果　术前有11眼RP患者合并有黄斑病变，其中黄斑囊样水肿2眼、黄斑前膜5眼、玻璃体黄斑牵拉4眼；术后发生后囊膜混浊5眼。患者术前及术后1周、1个月、3个月BCVA分别为（1.529±0.535）logMAR、(1.232±0.656）logMAR、(1.056±0.498）logMAR、(1.013±0.565）logMAR，术后最终BCVA较术前BCVA显著提高（t=3.252，P=0.002），其中19眼术后视力得到提高。术前BCVA与术后最终BCVA显著相关（r=0.683，P＜0.01）。Grade1组、Grade2组、Grade3组术后最终BCVA分别为（1.331±0.545）logMAR、（0.617±0.256）logMAR、（0.660±0.378）logMAR，Grade2组和Grade3组术后最终视力均好于Grade1组（F=6.764，P=0.005），Grade2组和Grade3组术后BCVA差异无统计学意义（P=0.878）。结论　大部分RP合并白内障患者在白内障手术后视力可得到改善，OCT可以判断RP患者白内障手术后视力预后，尤其是EZ结构完整性。     

Cataract and its surgery in Timor-Leste

PURPOSE: To determine the prevalence of visually significant and unoperated blinding cataract, and the coverage, characteristics and outcome of cataract surgery in Timor-Leste. METHODS: Based on the World Health Organization Rapid Assessment of Cataract Surgical Services protocol, a population-based cross-sectional survey was conducted in 2005. By two-stage cluster random sampling, 50 clusters of 30 people aged 40 years and older were selected. Each eye with a presenting visual acuity worse than 6/18 and/or a history of cataract surgery was examined. RESULTS: Of the 1470 people enumerated, 96.2% were examined. Of the eyes examined, 11.5% were blind (presenting vision less than 6/60). Cataract caused 66.3% of this, and 40.2% of vision impairment (presenting vision less than 6/18). For those people who were blind (7.7%; presenting vision less than 6/60 in the better eye), cataract was the most frequent cause (76.1%). Cataract surgery had occurred in 2% of participants. Gender and domicile were not associated with cataract blindness or surgery. Cataract Surgical Coverage (6/60) was 16.3% for Eyes and 20.2% for Persons. Surgical outcomes were poor, falling well short of World Health Organization guidelines. Astigmatism and posterior capsule opacity were important contributors to this. CONCLUSIONS: Until census data are available, age-gender-domicile correction of sample findings cannot be calculated. In the meantime, it is estimated there are 11 500 cataract blind in Timor-Leste. Long-term, indigenous cataract surgical services need to be capable of significantly increased output. Better surgical outcomes are required to ensure scarce resources are well used. Laser capsulotomy needs to be planned for as surgical numbers increase.     

视网膜色素变性的治疗进展

视网膜色素变性(retinitis pigmentosa,RP)是一类以进行性感光细胞和色素上皮细胞功能障碍为特征的遗传性疾病,是世界范围内常见的致盲性眼病。其发病机制和确切病因尚不明了,至今缺乏有效治疗办法。我们回顾近年来国内外特别是国外相关文献,对视网膜色素变性的治疗方法的研究进展作一综述。     

原发性视网膜色素变性的低视力患者配戴琥珀色滤光镜前后视觉功能变化

目的:对比观察原发性视网膜色素变性(RP)的低视力患者在配戴琥珀色滤光镜前后的视觉功能变化。方法:自身前后对照研究。收集2021-08/2022-03就诊于西安市第一医院眼科门诊诊断为原发性RP的低视力患者30例60眼。记录配戴琥珀色滤光镜前后裸眼远视力(UCDVA)、最佳矫正远视力(BCDVA)、裸眼近视力(UCNVA)、最佳矫正近视力(BCNVA)、视野和FM-100色觉,并分别在明室环境、暗室环境和暗室伴眩光环境进行对比敏感度检查并记录,分析配戴前后上述视功能参数的变化。结果:配戴琥珀色滤光镜后UCDVA和BCDVA均优于配戴前(t=-2.32,P<0.001;t=-6.77,P<0.001),而UCNVA和BCNVA在戴镜前后差异无统计学意义。戴镜后的视野指数(VFI)低于配戴前(t=8.62,P<0.001)、视野平均缺损值(MD)大于配戴前(t=7.73,P<0.001),FM100色棋检查显示TES和多个分区PES均高于戴镜前(P<0.001)。配戴后明室环境、暗室伴眩光环境下各频段CS值较配戴前提高(P<0.001),暗室不伴眩光环境...     

视网膜色素变性治疗的研究进展

视网膜色素变性(retinitis pigmentosa,RP)是由视网膜光感受器和视网膜色素上皮变性所引起的致盲性、遗传性眼病,目前尚无有效的治疗方法,相关治疗研究还处于探索阶段,如基因治疗、药物治疗、移植治疗、人工视网膜假体等。基因治疗RP是目前的研究热点,包括修复致病基因、核酸治疗、RNA干扰技术等。基因治疗、干细胞移植、人工视网膜假体治疗RP已经进入临床试验阶段,为治疗该病带来了新的希望。本文就RP治疗的研究进展做一综述。