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DYSTROPHIE RETICULEE SJOGREN [1 record]

Record 1 2025-08-26

English

Subject field(s)
  • Visual Disorders
CONT

Sjögren reticular dystrophy is an exceedingly rare condition ... with both autosomal recessive and dominant modes of inheritance. A bilateral and symmetric reticular pattern of RPE [retinal pigment epithelium] clumping, hyperplasia, and associated atrophic degenerative changes is noted that may or may not be associated with a slight decrease in central vision. In the initial stages, pigment granules accumulate at the site of the fovea with a network that resembles a "fishnet with knots." The midperiphery and periphery may be spared, but in some cases this may be the principal area of involvement. ... The reticular changes probably appear in infancy and are often fully developed by 15 years of age.

French

Domaine(s)
  • Troubles de la vision
CONT

La dystrophie réticulée de Sjögren [...] C'est une affection rare, apparaissant dans l'enfance, caractérisée par des altérations pigmentaires paracentrales donnant un aspect typique, en filet comportant des nœuds.

OBS

La dystrophie réticulée de Sjögren est de transmission autosomique dominante ou récessive.

Spanish

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