{"id":2222,"date":"2010-01-03T13:29:05","date_gmt":"2010-01-03T18:29:05","guid":{"rendered":"http:\/\/insidesurgery.com\/?p=2222"},"modified":"2010-01-03T17:49:35","modified_gmt":"2010-01-03T22:49:35","slug":"retinitis-pigmentosa","status":"publish","type":"post","link":"https:\/\/insidesurgery.com\/2010\/01\/retinitis-pigmentosa\/","title":{"rendered":"Retinitis Pigmentosa"},"content":{"rendered":"

Pathophysiology<\/strong><\/p>\n

1)<\/strong> group of different conditions all marked by rod and cone dystrophy 2)<\/strong> most common causes are mutations in genes for peripherin, rhodopsin, and rod photopigment<\/p>\n

Signs and Symptoms<\/strong><\/p>\n

1)<\/strong> progressive night blindness 2)<\/strong> loss of visual acuity 3)<\/strong> constriction of visual ring 4)<\/strong> ring scotoma<\/p>\n

Characteristic Test Findings<\/strong><\/p>\n

abnormal electroretinogram<\/p>\n

Histology\/Gross Pathology<\/strong><\/p>\n

“bone spicules” in peripheral retina (not actually bone but an abnormal collection of black pigment)<\/p>\n

Associated Conditions<\/strong><\/p>\n

component of – Bassen-Kornzweig syndrome, Kearns-Sayre syndrome, Refsum’s disease, olivopontocerebellar degeneration<\/p>\n

Inheritance\/Epidemiology<\/strong><\/p>\n

occurs in sporadic, autosomal dominant, autosomal recessive, and X-linked forms<\/p>\n

Treatment<\/strong><\/p>\n

1)<\/strong> no effective treatment 2)<\/strong> vitamin A has slight palliative effect but does not affect overall course of disease 3)<\/strong> gene therapy trials are under way<\/p>\n

Tips for USMLE<\/strong> <\/p>\n

1)<\/strong> name of disease is a misonmer – there is no inflammation in these conditions 2)<\/strong> if question mentions peripherin, think retinitis pigmentosa 3)<\/strong> if question mentions rhodopsin, think retinitis pigmentosa<\/p>\n","protected":false},"excerpt":{"rendered":"

Pathophysiology 1) group of different conditions all marked by rod and cone dystrophy 2) most common causes are mutations in genes for peripherin, rhodopsin, and rod photopigment Signs and Symptoms 1) progressive night blindness 2) loss of visual acuity 3) constriction of visual ring 4) ring scotoma Characteristic Test Findings abnormal electroretinogram Histology\/Gross Pathology “bone […]<\/p>\n","protected":false},"author":1,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_exactmetrics_skip_tracking":false,"_exactmetrics_sitenote_active":false,"_exactmetrics_sitenote_note":"","_exactmetrics_sitenote_category":0,"_genesis_hide_title":false,"_genesis_hide_breadcrumbs":false,"_genesis_hide_singular_image":false,"_genesis_hide_footer_widgets":false,"_genesis_custom_body_class":"","_genesis_custom_post_class":"","_genesis_layout":"","footnotes":""},"categories":[63],"tags":[919,916,920,912,914,918,921,913,915,917],"yoast_head":"\nRetinitis Pigmentosa - 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