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Retinitis pigmentosa (RP) is a bilateral and usually symmetrical pigmentary dystrophy that at first predominantly affects the retinal rods, with the retinal cones more involved as the condition progresses. RP encompasses a group of conditions, some purely ocular in nature and others that have significant systemic components.
Examples of the latter include Bassen-Kornzwieg syndrome, Refsum's disease, Usher's syndrome, Laurence-Moon and Bardet-Biedl syndromes, and Kearns-Sayer syndrome. RP may be inherited as an autosomal dominant, autosomal recessive or an X-linked recessive trait. An autosomal recessive pattern is the more common, followed by the dominant type. The X-linked type is the least common. The recessive forms of the disease tend to occur earlier in life and are more visually destructive.
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