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视网膜色素变性患者的视野进展模式

Patterns of visual field progression in patients with retinitis pigmentosa.

作者信息

Grover S, Fishman G A, Brown J

机构信息

Department of Ophthalmology and Visual Sciences, University of Illinois at Chicago, Illinois 60612, USA.

出版信息

Ophthalmology. 1998 Jun;105(6):1069-75. doi: 10.1016/S0161-6420(98)96009-2.

Abstract

OBJECTIVE

The purpose of the study was to determine whether distinct patterns of visual field progression are present in patients with retinitis pigmentosa (RP) and to evaluate the correlation between these patterns, if present, and different genetic subtypes of RP.

DESIGN

A retrospective analysis of patterns of visual field progression in RP was performed.

PARTICIPANTS

Visual fields of 162 patients with RP, including 55 with type 2 Usher syndrome, who had at least 3 Goldmann visual field examinations during a period of at least 3 years were reviewed.

MAIN OUTCOME MEASURES

Goldmann visual fields.

RESULTS

Visual fields of 86 patients could be classified into one of three specific patterns of visual field progression. Pattern I included those patients with a progressive concentric loss of visual fields; pattern II included those with visual field loss that began superiorly and subsequently developed an arcuate scotoma that progressed either from the nasal (IIA) or the temporal (IIB and IIC) side; and pattern III included patients whose visual field loss was characterized initially by a complete or incomplete midperipheral "ring scotoma" that broke through into the periphery. The end stage of all these patterns was a residual central visual field, sometimes also associated with a small peripheral island. In 53 of the 162 patients, the pattern of visual field loss could not be categorized because of an advanced stage of field loss at the time of the initial examination.

CONCLUSIONS

Distinctive patterns of visual field progression can be observed in patients with retinitis pigmentosa and type 2 Usher syndrome. There were no intrafamilial variations in the pattern of visual field loss in our data on 24 patients from 11 families. Within certain genetic subtypes, there was a predilection for a preponderance of a specific pattern of visual field progression. Future studies may be able to correlate these patterns of visual field loss with different genetic mutations. A greater understanding as to why certain patterns of field loss exist could potentially provide greater insight into the various pathogenetic mechanism(s) by which photoreceptor cells degenerate in this group of patients.

摘要

目的

本研究旨在确定视网膜色素变性(RP)患者是否存在不同的视野进展模式,并评估这些模式(若存在)与RP不同基因亚型之间的相关性。

设计

对RP患者的视野进展模式进行回顾性分析。

参与者

回顾了162例RP患者的视野情况,其中包括55例2型Usher综合征患者,这些患者在至少3年的时间里至少进行了3次Goldmann视野检查。

主要观察指标

Goldmann视野。

结果

86例患者的视野可分为三种特定的视野进展模式之一。模式I包括那些视野呈进行性同心性丧失的患者;模式II包括那些视野丧失始于上方,随后发展为弓形暗点,且该暗点从鼻侧(IIA)或颞侧(IIB和IIC)进展的患者;模式III包括那些视野丧失最初表现为完全或不完全的中周部“环形暗点”并突破至周边部的患者。所有这些模式的末期均为残余的中心视野,有时还伴有小的周边视野岛。在162例患者中,有53例由于初次检查时视野丧失已处于晚期而无法对视野丧失模式进行分类。

结论

在视网膜色素变性和2型Usher综合征患者中可观察到独特的视野进展模式。在我们对来自11个家庭的24例患者的数据中,视野丧失模式在家族内没有差异。在某些基因亚型中,存在特定视野进展模式占优势的倾向。未来的研究或许能够将这些视野丧失模式与不同的基因突变联系起来。对某些视野丧失模式为何存在有更深入的了解,可能会为这组患者中光感受器细胞退化的各种致病机制提供更多的见解。

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