• 文献检索
  • 文档翻译
  • 深度研究
  • 学术资讯
  • Suppr Zotero 插件Zotero 插件
  • 邀请有礼
  • 套餐&价格
  • 历史记录
应用&插件
Suppr Zotero 插件Zotero 插件浏览器插件Mac 客户端Windows 客户端微信小程序
定价
高级版会员购买积分包购买API积分包
服务
文献检索文档翻译深度研究API 文档MCP 服务
关于我们
关于 Suppr公司介绍联系我们用户协议隐私条款
关注我们

Suppr 超能文献

核心技术专利:CN118964589B侵权必究
粤ICP备2023148730 号-1Suppr @ 2026

文献检索

告别复杂PubMed语法,用中文像聊天一样搜索,搜遍4000万医学文献。AI智能推荐,让科研检索更轻松。

立即免费搜索

文件翻译

保留排版,准确专业,支持PDF/Word/PPT等文件格式,支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述,25分钟生成高质量综述,智能提取关键信息,辅助科研写作。

立即免费体验

斯塔加特病患者的延迟性视杆细胞暗适应

Delayed rod dark adaptation in patients with Stargardt's disease.

作者信息

Fishman G A, Farbman J S, Alexander K R

机构信息

Department of Ophthalmology, University of Illinois, College of Medicine, Chicago 60612.

出版信息

Ophthalmology. 1991 Jun;98(6):957-62. doi: 10.1016/s0161-6420(91)32196-1.

DOI:10.1016/s0161-6420(91)32196-1
PMID:1866151
Abstract

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.

摘要

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。

相似文献

1
Delayed rod dark adaptation in patients with Stargardt's disease.斯塔加特病患者的延迟性视杆细胞暗适应
Ophthalmology. 1991 Jun;98(6):957-62. doi: 10.1016/s0161-6420(91)32196-1.
2
Effect of vitamin A treatment on the prolongation of dark adaptation in Stargardt's dystrophy.
Retina. 1994;14(1):27-30. doi: 10.1097/00006982-199401000-00006.
3
Fundus flavimaculatus with severely reduced cone electroretinogram.
Jpn J Ophthalmol. 1992;36(3):249-56.
4
A clinical review of Stargardt's disease and/or fundus flavimaculatus with follow-up.
Int Ophthalmol. 1985 Nov;8(4):225-35. doi: 10.1007/BF00137651.
5
Stargardt's disease. Family studies.
Doc Ophthalmol. 1992;79(1):79-89. doi: 10.1007/BF00160134.
6
Comparison of visual acuity loss in patients with different stages of Stargardt's disease.不同阶段Stargardt病患者视力丧失情况的比较。
Ophthalmology. 2006 Oct;113(10):1748-51. doi: 10.1016/j.ophtha.2006.04.027. Epub 2006 Jul 26.
7
The electroretinogram in Stargardt's disease and fundus flavimaculatus.
Doc Ophthalmol. 1989 Dec;73(4):395-404. doi: 10.1007/BF00154495.
8
Occult macular dystrophy.隐匿性黄斑营养不良。
Am J Ophthalmol. 1996 Nov;122(5):644-53. doi: 10.1016/s0002-9394(14)70482-9.
9
Bull's-eye macular dystrophy associated with peripheral involvement.伴有周边部受累的靶心样黄斑营养不良
Ophthalmologica. 1998;212(4):260-7. doi: 10.1159/000027304.
10
Dark adaptation in patients with Best vitelliform macular dystrophy.Best卵黄样黄斑营养不良患者的暗适应
Br J Ophthalmol. 1994 Jun;78(6):430-2. doi: 10.1136/bjo.78.6.430.

引用本文的文献

1
Dark Adaptometry as a Diagnostic Tool in Retinal Diseases: Mechanisms and Clinical Utility.暗适应测量作为视网膜疾病的诊断工具:机制与临床应用
J Clin Med. 2025 May 27;14(11):3742. doi: 10.3390/jcm14113742.
2
Advances and therapeutic opportunities in visual cycle modulation.视觉循环调节的进展与治疗机遇
Prog Retin Eye Res. 2025 May;106:101360. doi: 10.1016/j.preteyeres.2025.101360. Epub 2025 Apr 23.
3
Retinoic acid related orphan receptor α is a genetic modifier that rescues retinal degeneration in a mouse model of Stargardt disease and Dry AMD.
维甲酸相关孤儿受体 α 是一种遗传修饰因子,可挽救 Stargardt 病和干性 AMD 小鼠模型中的视网膜变性。
Gene Ther. 2024 Jul;31(7-8):413-421. doi: 10.1038/s41434-024-00455-z. Epub 2024 May 16.
4
Primary versus Secondary Elevations in Fundus Autofluorescence.眼底自发荧光的原发性与继发性抬高。
Int J Mol Sci. 2023 Aug 2;24(15):12327. doi: 10.3390/ijms241512327.
5
Three-Year Safety Results of SAR422459 (EIAV-ABCA4) Gene Therapy in Patients With ABCA4-Associated Stargardt Disease: An Open-Label Dose-Escalation Phase I/IIa Clinical Trial, Cohorts 1-5.SAR422459(EIAV-ABCA4)基因治疗在 ABCA4 相关性 Stargardt 病患者中的 3 年安全性结果:一项开放标签剂量递增 I/IIa 临床试验,队列 1-5。
Am J Ophthalmol. 2022 Aug;240:285-301. doi: 10.1016/j.ajo.2022.02.013. Epub 2022 Mar 4.
6
Perifoveal Cone- and Rod-Mediated Temporal Contrast Sensitivities in Stargardt Disease/Fundus Flavimaculatus.Stargardt病/黄斑营养不良患者的中心凹周围视锥和视杆介导的时间对比敏感度
Invest Ophthalmol Vis Sci. 2021 Nov 1;62(14):24. doi: 10.1167/iovs.62.14.24.
7
Findings from a Genotyping Study of Over 1000 People with Inherited Retinal Disorders in Ireland.爱尔兰一项针对 1000 多名遗传性视网膜疾病患者的基因分型研究结果。
Genes (Basel). 2020 Jan 16;11(1):105. doi: 10.3390/genes11010105.
8
The Retinoid and Non-Retinoid Ligands of the Rod Visual G Protein-Coupled Receptor.视杆细胞视觉 G 蛋白偶联受体的视黄酸和非视黄酸配体。
Int J Mol Sci. 2019 Dec 10;20(24):6218. doi: 10.3390/ijms20246218.
9
Identification of splice defects due to noncanonical splice site or deep-intronic variants in ABCA4.鉴定 ABCA4 中由于非规范剪接位点或深内含子变异引起的剪接缺陷。
Hum Mutat. 2019 Dec;40(12):2365-2376. doi: 10.1002/humu.23890. Epub 2019 Sep 3.
10
Correlating the Expression and Functional Activity of ABCA4 Disease Variants With the Phenotype of Patients With Stargardt Disease.关联 ABCA4 病变更替与斯塔加特病患者表型的表达和功能活性。
Invest Ophthalmol Vis Sci. 2018 May 1;59(6):2305-2315. doi: 10.1167/iovs.17-23364.