Fishman G A, Farbman J S, Alexander K R
Department of Ophthalmology, University of Illinois, College of Medicine, Chicago 60612.
Ophthalmology. 1991 Jun;98(6):957-62. doi: 10.1016/s0161-6420(91)32196-1.
Twelve patients with Stargardt's dystrophy were each found to have a prolongation in rod dark adaptation. All had a normal rate of recovery during the early portion of rod dark adaptation but a selective prolongation of the later segment of rod recovery. This observation was apparent in patients with limited fundus flecks and those with extensive fundus flecks, whether or not a dark choroid was observed and independent of the presence or absence of an atrophic-appearing macular lesion. A defect within retinal pigment epithelial cells of an enzyme or intracellular transport mechanism involved in the visual pigment regeneration cycle could account for these findings.
研究发现,12例患有斯塔加特营养不良症的患者均存在视杆细胞暗适应延长的情况。在视杆细胞暗适应的早期阶段,所有患者的恢复速率均正常,但视杆细胞恢复的后期阶段出现了选择性延长。这一观察结果在眼底斑点有限的患者和眼底斑点广泛的患者中均很明显,无论是否观察到脉络膜暗区,且与萎缩性黄斑病变的有无无关。视色素再生循环中涉及的酶或细胞内转运机制在视网膜色素上皮细胞内出现缺陷可能是这些发现的原因。