TERMIUM Plus®

The Government of Canada’s terminology and linguistic data bank.

FEHRS SPOTTED CORNEAL DYSTROPHY [1 record]

Record 1 1995-07-20

English

Subject field(s)
  • Visual Disorders
DEF

A recessive familial degeneration initially affecting Bowman's membrane and then the surface epithelium and stroma. It commences in the first decade of life as a diffuse subepithelial opacification, with patches of greater density, and gradually increases in intensity, especially centrally, until, about the age of 30, little vision remains.

French

Domaine(s)
  • Troubles de la vision
CONT

Cette dystrophie touche essentiellement le stroma qui est le siège d'opacités nuageuses floues, blanc sale, correspondant à des accumulations de mucopolysaccharides. Cependant, les études ultrastructurales ont montré que la Descemet présentait des excroissances localisées, semblables à celles de la guttata mais contenant des mucopolysaccharides. Les cellules endothéliales, elles-mêmes, présentent la même anomalie métabolique que les kératocytes, avec des vacuoles remplies de mucopolysaccharides.

Spanish

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