{"id":74777,"date":"2021-11-23T15:26:14","date_gmt":"2021-11-23T15:26:14","guid":{"rendered":"https:\/\/www.atlasrleye.com\/atrophie-gyrate\/"},"modified":"2025-07-14T23:39:44","modified_gmt":"2025-07-14T23:39:44","slug":"atrophie-gyrate","status":"publish","type":"page","link":"https:\/\/www.atlasrleye.com\/fr\/atrophie-gyrate\/","title":{"rendered":"ATROPHIE GYRATE"},"content":{"rendered":"

[vc_row][vc_column][vc_custom_heading text=\u00a0\u00bbSYNDROMES ET DYSTROPHIES R\u00c9TINIENNES\u00a0\u00bb font_container=\u00a0\u00bbtag:h5|font_size:22px|text_align:center|color:%23000000″ use_theme_fonts=\u00a0\u00bbyes\u00a0\u00bb link=\u00a0\u00bburl:https%3A%2F%2Fwww.atlasrleye.com%2Ffr%2Fatlas%2F%231636632807269-1444524b-3ed4|\u00a0\u00bb][vc_text_separator title=\u00a0\u00bb\u00a0\u00bb i_icon_fontawesome=\u00a0\u00bbfas fa-eye\u00a0\u00bb i_color=\u00a0\u00bbcustom\u00a0\u00bb i_size=\u00a0\u00bblg\u00a0\u00bb color=\u00a0\u00bbcustom\u00a0\u00bb border_width=\u00a0\u00bb8″ css=\u00a0\u00bb.vc_custom_1637080776101{margin-bottom: -5px !important;}\u00a0\u00bb add_icon=\u00a0\u00bbtrue\u00a0\u00bb accent_color=\u00a0\u00bb#bababa\u00a0\u00bb i_custom_color=\u00a0\u00bb#bababa\u00a0\u00bb][vc_custom_heading source=\u00a0\u00bbpost_title\u00a0\u00bb font_container=\u00a0\u00bbtag:h1|font_size:44px|text_align:center|color:%235f1b52″ use_theme_fonts=\u00a0\u00bbyes\u00a0\u00bb]

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L\u2019atrophie gyrate est une dystrophie choro\u00efdienne g\u00e9n\u00e9ralis\u00e9e, de transmission autosomique r\u00e9cessive. Les patients atteints de cette alt\u00e9ration ont des niveaux \u00e9lev\u00e9s d’ornithine dans le plasma en raison d’une d\u00e9ficience g\u00e9n\u00e9tique de l’ornithine aminotransf\u00e9rase.<\/p>\n

Le g\u00e8ne codant pour cette enzyme a \u00e9t\u00e9 assign\u00e9 au chromosome 10. Ont \u00e9t\u00e9 identifi\u00e9es diff\u00e9rentes mutations dans ce g\u00e8ne qui incluent des mutations missense, nonsense et frameshift. Les anomalies mol\u00e9culaires des g\u00e8nes de r\u00e9ponse \u00e0 la pyridoxine et de non-r\u00e9ponse \u00e0 la pyridoxine ne sont pas \u00e9gales et elles ont des implications diff\u00e9rentes pour le pronostic et le traitement.<\/p>\n

Les premiers sympt\u00f4mes comprennent la difficult\u00e9 \u00e0 l\u2019adaptation \u00e0 l’obscurit\u00e9 et la perte de la vision p\u00e9riph\u00e9rique (similaires \u00e0 ceux de la r\u00e9tinopathie pigmentaire), parce que la maladie affecte d’abord la r\u00e9tine moyenne p\u00e9riph\u00e9rique. Les observations au fond de l\u2019\u0153il sont tout \u00e0 fait uniques et permettent g\u00e9n\u00e9ralement un diagnostic imm\u00e9diat. Les tests de la fonction visuelle sont alt\u00e9r\u00e9s dans les premiers stades de la maladie avec constriction du champ visuel et absence de r\u00e9ponses scotopique et photopique \u00e0 l’\u00e9lectror\u00e9tinogramme.<\/p>\n

Nous avons observ\u00e9 des zones focales d’atrophie choro\u00efdienne avec des fronti\u00e8res bien d\u00e9finies qui s\u00e9parent le tissu normal de ce qui est chang\u00e9. Ces l\u00e9sions progressent vers la macula et l’ora serrata Initialement sont rondes ou ovales et coalescent pour former de vastes zones d’atrophie avec un aspect festonn\u00e9 formant une couronne (d’o\u00f9 le terme \u00ab gyrate \u00bb). Les bords des l\u00e9sions montrent une ligne de d\u00e9marcation tr\u00e8s nette du tissu normal surlign\u00e9 par hyperpigmentation. La macula est apparemment la r\u00e9gion plus r\u00e9sistante, avec une acuit\u00e9 visuelle centrale conserv\u00e9e dans le cours de la maladie.<\/p>\n<\/div>\n

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Cas Cliniques<\/span><\/strong><\/h1>\n

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Cas Clinique 1<\/a><\/h4>\n

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Cas Clinique 2<\/a><\/h4>\n

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0<\/h3><\/div> <\/div> <\/div> [\/vc_column_inner][\/vc_row_inner][\/vc_column][\/vc_row]<\/p>\n<\/div>","protected":false},"excerpt":{"rendered":"

[vc_row][vc_column][vc_custom_heading text=\u00a0\u00bbSYNDROMES ET DYSTROPHIES R\u00c9TINIENNES\u00a0\u00bb font_container=\u00a0\u00bbtag:h5|font_size:22px|text_align:center|color:%23000000″ use_theme_fonts=\u00a0\u00bbyes\u00a0\u00bb link=\u00a0\u00bburl:https%3A%2F%2Fwww.atlasrleye.com%2Ffr%2Fatlas%2F%231636632807269-1444524b-3ed4|\u00a0\u00bb][vc_text_separator title=\u00a0\u00bb\u00a0\u00bb i_icon_fontawesome=\u00a0\u00bbfas fa-eye\u00a0\u00bb i_color=\u00a0\u00bbcustom\u00a0\u00bb i_size=\u00a0\u00bblg\u00a0\u00bb color=\u00a0\u00bbcustom\u00a0\u00bb border_width=\u00a0\u00bb8″ css=\u00a0\u00bb.vc_custom_1637080776101{margin-bottom: -5px !important;}\u00a0\u00bb add_icon=\u00a0\u00bbtrue\u00a0\u00bb accent_color=\u00a0\u00bb#bababa\u00a0\u00bb i_custom_color=\u00a0\u00bb#bababa\u00a0\u00bb][vc_custom_heading source=\u00a0\u00bbpost_title\u00a0\u00bb font_container=\u00a0\u00bbtag:h1|font_size:44px|text_align:center|color:%235f1b52″ use_theme_fonts=\u00a0\u00bbyes\u00a0\u00bb][\/vc_column][\/vc_row][vc_row][vc_column width=\u00a0\u00bb3\/4″][vc_column_text] L\u2019atrophie gyrate est une dystrophie choro\u00efdienne g\u00e9n\u00e9ralis\u00e9e, de transmission autosomique r\u00e9cessive. Les patients atteints de […]<\/p>\n","protected":false},"author":3,"featured_media":0,"parent":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"_monsterinsights_skip_tracking":false,"_monsterinsights_sitenote_active":false,"_monsterinsights_sitenote_note":"","_monsterinsights_sitenote_category":0,"footnotes":""},"class_list":["post-74777","page","type-page","status-publish","hentry"],"_links":{"self":[{"href":"https:\/\/www.atlasrleye.com\/fr\/wp-json\/wp\/v2\/pages\/74777","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/www.atlasrleye.com\/fr\/wp-json\/wp\/v2\/pages"}],"about":[{"href":"https:\/\/www.atlasrleye.com\/fr\/wp-json\/wp\/v2\/types\/page"}],"author":[{"embeddable":true,"href":"https:\/\/www.atlasrleye.com\/fr\/wp-json\/wp\/v2\/users\/3"}],"replies":[{"embeddable":true,"href":"https:\/\/www.atlasrleye.com\/fr\/wp-json\/wp\/v2\/comments?post=74777"}],"version-history":[{"count":22,"href":"https:\/\/www.atlasrleye.com\/fr\/wp-json\/wp\/v2\/pages\/74777\/revisions"}],"predecessor-version":[{"id":118068,"href":"https:\/\/www.atlasrleye.com\/fr\/wp-json\/wp\/v2\/pages\/74777\/revisions\/118068"}],"wp:attachment":[{"href":"https:\/\/www.atlasrleye.com\/fr\/wp-json\/wp\/v2\/media?parent=74777"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}