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Investigative Ophthalmology & Visual Science, Vol 27, 702-707, Copyright © 1986 by Association for Research in Vision and Ophthalmology

ARTICLES AND REPORTS

Age-related changes of the retinal pigment epithelium of cats with Chediak-Higashi syndrome

LL Collier, EJ King and DJ Prieur

The eyes of 7, 9, and 11-year-old Chediak-Higashi syndrome (CHS)- affected cats were examined by light, fluorescence, and electron microscopy. Numerous round to oval bodies of various sizes were associated with the retinal pigment epithelium (RPE). These bodies stained positively with periodic acid-Schiff. They also displayed a bright yellow autofluorescence and stained positively with a prolonged Ziehl-Neelsen acid-fast method for demonstration of lipofuscin, suggesting that they contained lipofuscin or a lipofuscin-like material. Ultrastructural examination disclosed the bodies to be secondary lysosomes and large to giant-sized residual bodies. Many of the residual bodies were extracellular and formed drusen-like mounds, covered by deposits of basal lamina, beneath the RPE. Also evident were scattered degenerated RPE cells and other RPE cells that had detached and migrated into the interphotoreceptor space. The presence of drusenoid bodies, and the loss of cells from the RPE monolayer in CHS eyes have not been reported previously. Many of the changes in the CHS cat eyes resemble those in non-CHS aging eyes of man and other species.




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Copyright © 1986 by the Association for Research in Vision and Ophthalmology