眼科 ›› 2015, Vol. 24 ›› Issue (3): 177-181.doi: 10.13281/j.cnki.issn.1004-4469.2015.03.010

• 论著 • 上一篇    下一篇

视网膜色素变性393例临床特征分析  

蓓 朱晓青   

  1. 100730 首都医科大学附属北京同仁医院 北京同仁眼科中心 眼科学与视觉科学北京市重点实验室

  • 收稿日期:2014-05-26 出版日期:2015-05-25 发布日期:2015-06-04
  • 通讯作者: 朱晓青,Email:zhuxiaoqing2006@126.com E-mail:zhuxiaoqing2006@126.com

Clinical features of 393 cases of retinitis pigmentosa

TIAN Bei, ZHU Xiao-qing   

  1.  Beijing Tongren Eye Center, Beijing Key Laboratory of Ophthalmology and Visual Science, Beijing Tongren Hospital, Capital Medical University, Beijing 100730, China

  • Received:2014-05-26 Online:2015-05-25 Published:2015-06-04
  • Contact: ZHU Xiao-qing, Email: zhuxiaoqing2006@126.com E-mail:zhuxiaoqing2006@126.com

摘要: 目的 探讨视网膜色素变性(RP)患者的临床表型特点。设计 回顾性病例系列。研究对象 2007年1月至2014年5月就诊于北京同仁眼科中心的RP患者393例。方法 回顾并总结该393例RP患者的基本信息、病史特点及临床表现特点。主要指标 RP的发病年龄、眼前后段常见临床表现和并发症。结果 RP患者多为双眼发病(98.0%),无明显性别偏向(男:女=1:0.98)。视力下降(78.4%)和夜盲(86.5%)是常见且典型的主诉。大多患者为近视(52.2%),最佳矫正视力多处于0.1~0.6之间(55.5%)。后囊下型并发性白内障(16.5%)是RP常见的眼前段并发症,眼底主要表现为视盘颜色偏淡(40.9%)、动静脉变细(分别为57.1%和56.9%)及色素沉着,以骨细胞样色素(63.6%)为主且大多弥漫分布(64.5%),部分患者同时合并脉络膜萎缩(32.9%)和黄斑前膜(30.3%)。典型性RP(58.9%)是该病的主要类型,其次为无色素性RP(12.6%)。结论 RP的临床表型多样,除典型的视神经蜡样萎缩、血管变细和骨细胞样色素沉着三联征外,尚有多种眼前段及后段的变异表现和并发症。

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 , 视网膜色素变性, 骨细胞样色素沉着

Abstract: Objective To analyze clinical features of patients with retinitis pigmentosa (RP). Design Retrospective case series. Participants 393 patients with RP who consulted in Beijing Tongren Eye Center between January 2007 and May 2014. Methods Basic characterization, clinical history and clinical manifestations of 393 RP patients were reviewed. Main Outcome Measures Age of onset, clinical features of anterior and posterior segment of the eye and complications. Results RP generally affects bilaterally (98.0%) without gender preference (M:F=1:0.98). Blurred vision (78.4%) and night blindness (86.5%) were typical complaints. Most patients were myopia (52.2%) with best corrected visual acuity (Snellen) ranged from 0.1 to 0.6 (55.5%). Posterior subcapsular cataract (16.5%) was a common complication. Optic disc pale (40.9%), generally narrowing of retinal artery (57.1%) and vein (56.9%), and pigment disposition were main fundus findings. Osteoblast like pigment (63.6%) was the most common type and located diffusely (64.5%). Some patients also had choroid atrophy (32.9%) and macular epiretinal membrane (30.3%). Typical RP (58.9%) was the most common type with non-pigment RP (12.6%) secondly. Conclusion Clinical features of RP may vary greatly. Besides optic atrophy, narrowing of retinal vessels and pigmentation deposition, RP may have other manifestations and complications in both anterior and posterior segment of the eye.

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 retinitis pigmentosa, osteoblast like pigment