Clinical and histopathologic findings in clumped pigmentary retinal degeneration

被引：22

作者：

To, KW ^{[1
]}

Adamian, M ^{[1
]}

Jakobiec, FA ^{[1
]}

Berson, EL ^{[1
]}

机构：

[1] HARVARD UNIV,MASSACHUSETTS EYE & EAR INFIRM,SCH MED,DEPT OPHTHALMOL,OPHTHALM PATHOL SERV,BOSTON,MA

来源：

ARCHIVES OF OPHTHALMOLOGY | 1996年 / 114卷 / 08期

关键词：

D O I：

10.1001/archopht.1996.01100140158008

中图分类号：

R77 [眼科学];

学科分类号：

100212 ;

摘要：

Objective: To describe the clinical and histopathologic features of clumped pigmentary retinal degeneration (CPRD). Design: Retrospective case series. Setting: Tertiary referral center. Patients: Twenty-four-patients, aged 7 to 83 years, were identified from the medical record files of the Berman-Gund Laboratory, Boston, Mass, as having the clinical features of CPRD. The autopsy eye from a SG-year-old man with CPRD was studied with light and electron microscopy. Main Outcome Measures: Visual acuities, visual fields, dark-adaptation thresholds, and results of electroretinograms; histopathologic study of an autopsy eye. Results: The functional deficit of patients with CPRD seems to be similar to that of patients with typical retinitis pigmentosa. Different degrees of severity were observed among patients of similar age. The histopathologic data showed that the clinically distinct areas of clumped pigment are due to excessive accumulation of melanin granules in retinal pigment epithelial cells. Conclusion: Based on the distinct clinical and histopathologic appearance, CPRD should be considered as a separate form of retinal degeneration.

引用

页码：950 / 955

页数：6

共 15 条

[1] NATURAL COURSE OF RETINITIS PIGMENTOSA OVER A 3-YEAR INTERVAL [J].