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小口病患者正常的S视锥细胞视网膜电图b波。

Normal S cone electroretinogram b-wave in Oguchi's disease.

作者信息

Yamamoto S, Hayashi M, Takeuchi S, Shirao Y, Kita K, Kawasaki K

机构信息

Toho University Sakura Hospital, Japan.

出版信息

Br J Ophthalmol. 1997 Dec;81(12):1043-5. doi: 10.1136/bjo.81.12.1043.

DOI:10.1136/bjo.81.12.1043
PMID:9497461
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1722076/
Abstract

AIM

The short wavelength sensitive (S) cone electroretinograms (ERGs) were examined in two patients with Oguchi's disease to study S cone function.

METHODS

Ganzfeld colour flashes under bright white background illumination were used to elicit S cone, and mixed long (L) and middle (M) wavelength sensitive cone ERGs.

RESULTS

The S cone ERG b-wave was normal with short wavelength stimuli with normal L and M cone responses in both patients.

CONCLUSIONS

These ERG results indicate that the S cone system as well as the L and M cone system is not defective in Oguchi's disease, while the S cone responses are not detectable in the complete type of congenital stationary night blindness as reported previously. The results imply that there is a significant difference in the way the S cone system is affected in different forms of stationary night blindness, and that the S cone and rod do not share the same arrestin system.

摘要

目的

对两名小口氏病患者进行短波长敏感(S)视锥细胞视网膜电图(ERG)检查,以研究S视锥细胞功能。

方法

在明亮的白色背景照明下使用全视野彩色闪光来诱发S视锥细胞以及混合的长(L)和中(M)波长敏感视锥细胞ERG。

结果

两名患者在短波长刺激下S视锥细胞ERG的b波正常,L和M视锥细胞反应正常。

结论

这些ERG结果表明,小口氏病中S视锥细胞系统以及L和M视锥细胞系统均无缺陷,而如先前报道,在完全型先天性静止性夜盲中无法检测到S视锥细胞反应。结果表明,在不同形式的静止性夜盲中,S视锥细胞系统受影响的方式存在显著差异,并且S视锥细胞和视杆细胞不共享相同的抑制蛋白系统。

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OGUCHI'S DISEASE.小口氏病
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Undetectable S cone electroretinogram b-wave in complete congenital stationary night blindness.完全性先天性静止性夜盲中无法检测到的S波视网膜电图b波。
Br J Ophthalmol. 1996 Jul;80(7):637-9. doi: 10.1136/bjo.80.7.637.
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Paraneoplastic retinopathy associated with antiretinal bipolar cell antibodies in cutaneous malignant melanoma.皮肤恶性黑色素瘤中与抗视网膜双极细胞抗体相关的副肿瘤性视网膜病变。
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A homozygous 1-base pair deletion in the arrestin gene is a frequent cause of Oguchi disease in Japanese.抑制蛋白基因中纯合性1个碱基对的缺失是日本人 Oguchi 病的常见病因。
Nat Genet. 1995 Jul;10(3):360-2. doi: 10.1038/ng0795-360.
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Oguchi disease: suggestion of linkage to markers on chromosome 2q.小口病:与2号染色体q臂上标记物连锁的提示
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Electroretinography: Some basic principles.视网膜电图:一些基本原理。
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On- and off-responses in photopic electroretinogram in complete and incomplete types of congenital stationary night blindness.完全型和不完全型先天性静止性夜盲患者明视视网膜电图的开反应和关反应
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Paraneoplastic night blindness with malignant melanoma.副肿瘤性夜盲症伴恶性黑色素瘤
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