Holz F G
Augenklinik der Ruprecht-Karls-Universität Heidelberg.
Ophthalmologe. 2001 Jan;98(1):10-8. doi: 10.1007/s003470170194.
Visualization of the retinal pigment epithelium (RPE) in vivo has proven difficult for various reasons, including the optical properties of the eye and the small size of the cellular elements, forming a single layer between the neurosensory retina and Bruch's membrane. With the advent of scanning laser ophthalmoscopy it is now possible to image topographic distribution and intensity fundus autofluorescence derived from accumulations of lipofuscin granules in RPE cells. Excessive lipofuscin storage in the RPE cytoplasm occurs not only in association with age but also with many hereditary and degenerative retinal diseases including age-related macular degeneration, Stargardt disease, Best disease, and pattern dystrophies. Lipofuscin in the RPE derives mainly from incomplete degradation of phagocytosed distal segments of photoreceptor outer segments, and is composed of various biomolecules including lipids, protein, and retinoids. Some of its constituents have recently been shown to possess toxic properties in vitro and may play a pathophysiological role in diseases such as age-related macular degeneration.Visualizing lipofuscin in vivo may help to better understand the significance of these metabolic alterations in the pathogenesis of retinal disorders. In addition,fundus autofluorescence imaging can be useful in the preclinical diagnosis of hereditary retinal disease. Dynamic alterations of intrinsic RPE fluorescence change may be applicable for monitoring effects at the level of the RPE of novel therapeutic modalities. Furthermore, identification of high-risk characteristics in patients with age-related macular degeneration with this technique may be helpful for indicating and adjusting future therapies.
由于多种原因,包括眼睛的光学特性以及细胞成分的微小尺寸,视网膜色素上皮(RPE)在体内的可视化一直颇具难度,RPE在神经感觉视网膜和布鲁赫膜之间形成单层结构。随着扫描激光检眼镜的出现,现在能够对源自RPE细胞中脂褐素颗粒积聚的眼底自发荧光的地形分布和强度进行成像。RPE细胞质中脂褐素的过度蓄积不仅与年龄相关,还与许多遗传性和退行性视网膜疾病有关,包括年龄相关性黄斑变性、斯塔加特病、贝斯特病和图案性营养不良。RPE中的脂褐素主要源自光感受器外段吞噬远端节段的不完全降解,由包括脂质、蛋白质和类视黄醇在内的各种生物分子组成。其一些成分最近已被证明在体外具有毒性特性,可能在诸如年龄相关性黄斑变性等疾病中发挥病理生理作用。在体内可视化脂褐素可能有助于更好地理解这些代谢改变在视网膜疾病发病机制中的意义。此外,眼底自发荧光成像在遗传性视网膜疾病的临床前诊断中可能有用。RPE固有荧光变化的动态改变可能适用于监测新型治疗方式在RPE水平的效果。此外,用该技术识别年龄相关性黄斑变性患者的高危特征可能有助于指导和调整未来的治疗。