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Retinal pathology in the Kearns-Sayre syndrome.

Publication ,  Journal Article
McKechnie, NM; King, M; Lee, WR
Published in: The British journal of ophthalmology
January 1985

Examination of the retinal tissues obtained at necropsy from a 14-year-old boy with Kearns-Sayre syndrome showed marked photoreceptor and pigment epithelial cell loss in the retinal periphery and around the optic nerve head. Electron microscopy of surviving retinal pigment epithelial (RPE) cells indicated a loss of apical microvilli and basal infoldings. The RPE was unusually devoid of melanosomes and showed no evidence of phagocytosis of photoreceptor debris. The cytoplasm of the RPE contained numerous, often enlarged, mitochondria. These structural changes suggested that a breakdown in the energy dependent interrelationships between the RPE and the photoreceptor layer was responsible for the outer retinal degeneration. The finding of numerous macrophages in the subretinal space suggests a secondary inflammatory component in the retinal degeneration.

Published In

The British journal of ophthalmology

ISSN

0007-1161

Publication Date

January 1985

Volume

69

Issue

1

Start / End Page

63 / 75

Location

england

Related Subject Headings

  • Retina
  • Pigment Epithelium of Eye
  • Photoreceptor Cells
  • Ophthalmoplegia
  • Ophthalmology & Optometry
  • Microscopy, Electron, Scanning
  • Microscopy, Electron
  • Male
  • Macrophages
  • Kearns-Sayre Syndrome
 

Citation

APA
Chicago
ICMJE
MLA
NLM
McKechnie, N. M., King, M., & Lee, W. R. (1985). Retinal pathology in the Kearns-Sayre syndrome. The British Journal of Ophthalmology, 69(1), 63–75.
McKechnie, N. M., M. King, and W. R. Lee. “Retinal pathology in the Kearns-Sayre syndrome.The British Journal of Ophthalmology 69, no. 1 (January 1985): 63–75.
McKechnie NM, King M, Lee WR. Retinal pathology in the Kearns-Sayre syndrome. The British journal of ophthalmology. 1985 Jan;69(1):63–75.
McKechnie, N. M., et al. “Retinal pathology in the Kearns-Sayre syndrome.The British Journal of Ophthalmology, vol. 69, no. 1, Jan. 1985, pp. 63–75.
McKechnie NM, King M, Lee WR. Retinal pathology in the Kearns-Sayre syndrome. The British journal of ophthalmology. 1985 Jan;69(1):63–75.

Published In

The British journal of ophthalmology

ISSN

0007-1161

Publication Date

January 1985

Volume

69

Issue

1

Start / End Page

63 / 75

Location

england

Related Subject Headings

  • Retina
  • Pigment Epithelium of Eye
  • Photoreceptor Cells
  • Ophthalmoplegia
  • Ophthalmology & Optometry
  • Microscopy, Electron, Scanning
  • Microscopy, Electron
  • Male
  • Macrophages
  • Kearns-Sayre Syndrome