PDF(Pubmed)
Abstract：
The purpose of this study was to establish spectral domain optical coherence tomography (SD-OCT) assessment data in well-established canine models of inherited retinal dystrophies: PDE6B-rod-cone dysplasia 1 (RCD1: early onset retinitis pigmentosa), PRCD-progressive rod-cone degeneration (PRCD: late onset retinitis pigmentosa), CNGB3-achromatopsia, and RPE65-Leber congenital amaurosis (LCA). High resolution SD-OCT images of the retina were acquired from both eyes in 5 planes: temporal; superotemporal; superior; nasal; and inferior in adult dogs with: RCD1 (n = 4 dogs, median age: 1.5 yrs); PRCD (n = 2, 4.3 yrs); LCA (n = 3, 5.2 yrs); achromatopsia (n = 3, 4.2 yrs); and wild types (wt, n = 6, 5.5 yrs). Total, inner and outer retinal thicknesses and ellipsoid zone were analyzed. In selected animals, histomorphometric evaluations were performed. In dogs with RCD1, PRCD, and LCA, the thickness of the outer retina was, compared to wt, significantly decreased (p ≤ 0.02) in all OCT imaging planes, and in superotemporal and inferior imaging planes in dogs with achromatopsia. No significant thinning was observed in inner retina thickness in any disease model except in the inferior imaging plane in dogs with RCD1. Dogs with RCD1, PRCD, and LCA had significantly more areas with disrupted ellipsoid zone in the presumed area centralis than wt (p ≤ 0.001). OCT findings provide baseline information for research of retinal dystrophies using these canine models.
摘要：
这项研究的目的是在已建立的遗传性视网膜营养不良的犬模型中建立光谱域光学相干断层扫描（SD-OCT）评估数据：PDE6B-杆-锥发育不良1（RCD1：早发性色素性视网膜炎），PRCD-进行性视锥细胞变性（PRCD：晚发性视网膜色素变性），CNGB3-色盲,和RPE65-Leber先天性黑蒙（LCA）。在5个平面上从两只眼睛获得视网膜的高分辨率SD-OCT图像：颞侧；超颞侧；上；鼻；成年犬的下位：RCD1（n=4犬，中位年龄：1.5岁）；PRCD（n=2，4.3岁）；LCA（n=3，5.2岁）；色盲（n=3，4.2岁）；和野生型（wt，n=6，5.5年）。总计,分析视网膜内外厚度和椭球区。在选定的动物中，进行了组织形态计量学评估.在RCD1，PRCD的狗中，和LCA,外视网膜的厚度是，与wt相比，在所有OCT成像平面中显著降低(p≤0.02)，以及患有色盲的狗的超颞叶和下叶成像平面。在任何疾病模型中，除了在具有RCD1的狗的下成像平面中，内部视网膜厚度均未观察到明显的变薄。带RCD1，PRCD的狗，LCA在假定的中心区域中具有椭圆形区域的面积明显多于wt（p≤0.001）。OCT研究结果为使用这些犬模型的视网膜营养不良研究提供了基线信息。
