Ocular clinicopathologic study of gyrate atrophy

D. J. Wilson; R. G. Weleber; W. R. Green

doi:10.1016/S0002-9394(14)76892-8

Ocular clinicopathologic study of gyrate atrophy

D. J. Wilson, R. G. Weleber, W. R. Green

Ophthalmology

Research output: Contribution to journal › Article › peer-review

39 Scopus citations

Abstract

We performed a histopathologic study of whole globes obtained post mortem from a patient with well-documented, vitamin B6-responsive gyrate atrophy. The retina in the posterior pole had focal areas of photoreceptor atrophy with adjacent retinal pigment epithelial hyperplasia. An abrupt transition from the near normal retina to a zone of near total atrophy of the retina, retinal pigment epithelium, and choroid was present in the fundus midperiphery. Electron microscopic examination disclosed abnormalities of the mitochondria of the corneal endothelium and the non-pigmented ciliary epithelium. Similar, but less severe, mitochondrial abnormalities were present in the photoreceptors.

Original language	English (US)
Pages (from-to)	24-33
Number of pages	10
Journal	American journal of ophthalmology
Volume	111
Issue number	1
DOIs	https://doi.org/10.1016/S0002-9394(14)76892-8
State	Published - 1991

ASJC Scopus subject areas

Ophthalmology

Access to Document

10.1016/S0002-9394(14)76892-8

Cite this

@article{8c16ea8fb5df4b578634886f7204bcec,

title = "Ocular clinicopathologic study of gyrate atrophy",

abstract = "We performed a histopathologic study of whole globes obtained post mortem from a patient with well-documented, vitamin B6-responsive gyrate atrophy. The retina in the posterior pole had focal areas of photoreceptor atrophy with adjacent retinal pigment epithelial hyperplasia. An abrupt transition from the near normal retina to a zone of near total atrophy of the retina, retinal pigment epithelium, and choroid was present in the fundus midperiphery. Electron microscopic examination disclosed abnormalities of the mitochondria of the corneal endothelium and the non-pigmented ciliary epithelium. Similar, but less severe, mitochondrial abnormalities were present in the photoreceptors.",

author = "Wilson, {D. J.} and Weleber, {R. G.} and Green, {W. R.}",

note = "Funding Information: From the Departments of Ophthalmology (Drs. Wilson and Weleber) and Medical Genetics (Dr. Weleber), Oregon Health Sciences University, Portland, Oregon; and Departments of Ophthalmology and Pathology, Johns Hopkins University and Hospital, Baltimore, Maryland (Dr. Green). This study was supported by Medical Research Foundation of Oregon grant 1-2-328-1540 (Dr. Wilson), and a center grant from the National Retinitis Pigmentosa Foundation (Dr. Weleber).",

year = "1991",

doi = "10.1016/S0002-9394(14)76892-8",

language = "English (US)",

volume = "111",

pages = "24--33",

journal = "American journal of ophthalmology",

issn = "0002-9394",

publisher = "Elsevier USA",

number = "1",

}

TY - JOUR

T1 - Ocular clinicopathologic study of gyrate atrophy

AU - Wilson, D. J.

AU - Weleber, R. G.

AU - Green, W. R.

N1 - Funding Information: From the Departments of Ophthalmology (Drs. Wilson and Weleber) and Medical Genetics (Dr. Weleber), Oregon Health Sciences University, Portland, Oregon; and Departments of Ophthalmology and Pathology, Johns Hopkins University and Hospital, Baltimore, Maryland (Dr. Green). This study was supported by Medical Research Foundation of Oregon grant 1-2-328-1540 (Dr. Wilson), and a center grant from the National Retinitis Pigmentosa Foundation (Dr. Weleber).

PY - 1991

Y1 - 1991

N2 - We performed a histopathologic study of whole globes obtained post mortem from a patient with well-documented, vitamin B6-responsive gyrate atrophy. The retina in the posterior pole had focal areas of photoreceptor atrophy with adjacent retinal pigment epithelial hyperplasia. An abrupt transition from the near normal retina to a zone of near total atrophy of the retina, retinal pigment epithelium, and choroid was present in the fundus midperiphery. Electron microscopic examination disclosed abnormalities of the mitochondria of the corneal endothelium and the non-pigmented ciliary epithelium. Similar, but less severe, mitochondrial abnormalities were present in the photoreceptors.

AB - We performed a histopathologic study of whole globes obtained post mortem from a patient with well-documented, vitamin B6-responsive gyrate atrophy. The retina in the posterior pole had focal areas of photoreceptor atrophy with adjacent retinal pigment epithelial hyperplasia. An abrupt transition from the near normal retina to a zone of near total atrophy of the retina, retinal pigment epithelium, and choroid was present in the fundus midperiphery. Electron microscopic examination disclosed abnormalities of the mitochondria of the corneal endothelium and the non-pigmented ciliary epithelium. Similar, but less severe, mitochondrial abnormalities were present in the photoreceptors.

UR - http://www.scopus.com/inward/record.url?scp=0025962538&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0025962538&partnerID=8YFLogxK

U2 - 10.1016/S0002-9394(14)76892-8

DO - 10.1016/S0002-9394(14)76892-8

M3 - Article

C2 - 1985486

AN - SCOPUS:0025962538

SN - 0002-9394

VL - 111

SP - 24

EP - 33

JO - American journal of ophthalmology

JF - American journal of ophthalmology

IS - 1

ER -

Ocular clinicopathologic study of gyrate atrophy

Abstract

ASJC Scopus subject areas

Access to Document

Other files and links

Fingerprint

Cite this