Qian T W, Xu X
Department of Ophthalmology, Affiliated First People's Hospital, Colloge of Medicine, Shanghai Jiaotong University, Shanghai 200080, China.
Zhonghua Yan Ke Za Zhi. 2017 Feb 11;53(2):148-153. doi: 10.3760/cma.j.issn.0412-4081.2017.02.015.
Retinitis pigmentosa (RP) is a genetically heterogeneous group of hereditary retinal disorders characterized by photoreceptor cell death, associated with night blindness, vision loss, progressive peripheral visual field loss and abnormalities in the electroretinogram. A number of gene defects have so far been associated with RP, which cause a progressive loss of rod photoreceptor function, followed by cone photoreceptor dysfunction and eventually complete blindness. The rate of blindness related to RP is high. At present there is no effective therapeutic strategy for RP. In recent years, with the progress of molecular biology technique, many new therapeutic approaches have become promising. This article summarizes the pathogenesis of RP and gives a brief overview of related research progress of RP therapeutic strategies. .
视网膜色素变性（RP）是一组遗传性视网膜疾病，具有遗传异质性，其特征是光感受器细胞死亡，伴有夜盲、视力丧失、进行性周边视野缺损以及视网膜电图异常。迄今为止，许多基因缺陷与RP相关，这些缺陷导致视杆光感受器功能逐渐丧失，随后视锥光感受器功能障碍，最终导致完全失明。与RP相关的失明率很高。目前尚无针对RP的有效治疗策略。近年来，随着分子生物学技术的进步，许多新的治疗方法展现出了前景。本文总结了RP的发病机制，并简要概述了RP治疗策略的相关研究进展。
