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[视网膜色素上皮功能障碍的临床表现]

[Clinical manifestations of functional disturbances of the retinal pigment epithelium].

作者信息

Gamulescu M A, Renner A B, Helbig H

机构信息

Augenklinik, Universitätsklinikum Regensburg, Regensburg, Deutschland.

出版信息

Ophthalmologe. 2009 Apr;106(4):305-10. doi: 10.1007/s00347-008-1870-4.

DOI:10.1007/s00347-008-1870-4
PMID:19340431
Abstract

The retinal pigment epithelium (RPE) serves a variety of different functions and impairment of these functions can lead to a multitude of different diseases of the posterior segment of the eye. The RPE plays an important role as an ion and fluid pump for the reabsorption of subretinal fluid in retinal detachment. On the other hand, defects in this pump function and in the outer blood-retinal barrier formed by the RPE, lead to fluid retention in inflammatory diseases. Metaplasia of RPE cells to myofibroblasts can lead to proliferative vitreoretinopathy and tractive retinal detachment. Early age-related maculopathy is caused by disturbances of phagocytosis and metabolism of the RPE. Imbalance of the physiological equilibrium between vasoproliferative and vasoinhibitory factors secreted by the RPE is probably involved in the development of atrophic or neovascular forms of advanced age-related macular degeneration. Mutations in the different steps involved in regeneration of the visual pigment (visual cycle) may lead to retinal dystrophy. Finally, immunoregulatory properties of the RPE are responsible for the phenomenon of immunological privilege, which may facilitate clinical interventions such as gene therapy and RPE transplantation.

摘要

视网膜色素上皮(RPE)具有多种不同功能,这些功能的损害可导致多种不同的眼后段疾病。RPE作为离子和液体泵,在视网膜脱离时对视网膜下液的重吸收起着重要作用。另一方面,该泵功能以及由RPE形成的外血视网膜屏障的缺陷,会导致炎症性疾病中的液体潴留。RPE细胞向肌成纤维细胞的化生可导致增殖性玻璃体视网膜病变和牵拉性视网膜脱离。早期年龄相关性黄斑病变是由RPE吞噬作用和代谢紊乱引起的。RPE分泌的血管增殖因子和血管抑制因子之间的生理平衡失衡可能与萎缩性或新生血管性晚期年龄相关性黄斑变性的发生有关。视觉色素再生(视觉循环)所涉及的不同步骤中的突变可能导致视网膜营养不良。最后,RPE的免疫调节特性导致了免疫赦免现象,这可能有助于基因治疗和RPE移植等临床干预。

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本文引用的文献

1
[The role of retinal pigment epithelium in visual functions].[视网膜色素上皮在视觉功能中的作用]
Ophthalmologe. 2009 Apr;106(4):299-304. doi: 10.1007/s00347-008-1869-x.
2
[Genetic diseases of the retinal pigment epithelium].[视网膜色素上皮的遗传性疾病]
Ophthalmologe. 2009 Apr;106(4):311-9. doi: 10.1007/s00347-008-1871-3.
3
Effect of gene therapy on visual function in Leber's congenital amaurosis.基因治疗对莱伯先天性黑蒙视觉功能的影响。
N Engl J Med. 2008 May 22;358(21):2231-9. doi: 10.1056/NEJMoa0802268. Epub 2008 Apr 27.
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Hereditary retinal disease.遗传性视网膜疾病。
Curr Opin Ophthalmol. 2008 May;19(3):255-62. doi: 10.1097/ICU.0b013e3282fc27fc.
5
Transplantation of the RPE in AMD.年龄相关性黄斑变性中视网膜色素上皮的移植
Prog Retin Eye Res. 2007 Sep;26(5):516-54. doi: 10.1016/j.preteyeres.2007.02.002. Epub 2007 Mar 6.
6
An update on the genetics of age-related macular degeneration.年龄相关性黄斑变性的遗传学最新进展。
Mol Vis. 2007 Feb 7;13:196-205.
7
Immunosuppressive properties of the pigmented epithelial cells and the subretinal space.色素上皮细胞和视网膜下间隙的免疫抑制特性。
Chem Immunol Allergy. 2007;92:86-93. doi: 10.1159/000099259.
8
AAV-mediated gene transfer for retinal diseases.腺相关病毒介导的视网膜疾病基因转移
Expert Opin Biol Ther. 2006 Dec;6(12):1279-94. doi: 10.1517/14712598.6.12.1279.
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Ranibizumab for neovascular age-related macular degeneration.雷珠单抗用于治疗新生血管性年龄相关性黄斑变性。
N Engl J Med. 2006 Oct 5;355(14):1419-31. doi: 10.1056/NEJMoa054481.
10
Diseases caused by defects in the visual cycle: retinoids as potential therapeutic agents.视觉循环缺陷导致的疾病:类视黄醇作为潜在治疗药物。
Annu Rev Pharmacol Toxicol. 2007;47:469-512. doi: 10.1146/annurev.pharmtox.47.120505.105225.