Sparrow Janet R, Duncker Tobias, Woods Russell, Delori François C
Department of Ophthalmology, Harkness Eye Institute, Columbia University Medical Center, 635 W. 165th Street, 10032, New York, NY, USA.
Department of Pathology and Cell Biology, Columbia University Medical Center, 635 W. 165th Street, 10032, New York, NY, USA.
Adv Exp Med Biol. 2016;854:285-90. doi: 10.1007/978-3-319-17121-0_38.
Since the lipofuscin of retinal pigment epithelial (RPE) cells has been implicated in the pathogenesis of Best vitelliform macular dystrophy, we quantified fundus autofluorescence (quantitative fundus autofluorescence, qAF) as an indirect measure of RPE lipofuscin levels. Mean non-lesion qAF was found to be within normal limits for age. By spectral domain optical coherence tomography (SD-OCT) vitelliform lesions presented as fluid-filled subretinal detachments containing reflective material. We discuss photoreceptor outer segment debris as the source of the intense fluorescence of these lesions and loss of anion channel functioning as an explanation for the bullous photoreceptor-RPE detachment. Unexplained is the propensity of the disease for central retina.
由于视网膜色素上皮（RPE）细胞的脂褐质与Best卵黄样黄斑营养不良的发病机制有关，我们对眼底自发荧光（定量眼底自发荧光，qAF）进行了量化，作为RPE脂褐质水平的间接测量指标。发现平均非病变qAF在年龄正常范围内。通过光谱域光学相干断层扫描（SD-OCT），卵黄样病变表现为含有反光物质的视网膜下液性脱离。我们讨论了光感受器外段碎片作为这些病变强烈荧光的来源，以及阴离子通道功能丧失作为大疱性光感受器-RPE脱离的一种解释。该疾病对视网膜中央区域的倾向尚无法解释。
