{"id":74685,"date":"2022-01-26T17:11:55","date_gmt":"2022-01-26T17:11:55","guid":{"rendered":"https:\/\/www.atlasrleye.com\/deficit-en-lchad\/cas-clinique-2\/"},"modified":"2024-03-17T22:56:40","modified_gmt":"2024-03-17T22:56:40","slug":"cas-clinique-2","status":"publish","type":"page","link":"https:\/\/www.atlasrleye.com\/fr\/deficit-en-lchad\/cas-clinique-2\/","title":{"rendered":"Cas clinique 2"},"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″ add_icon=\u00a0\u00bbtrue\u00a0\u00bb css=\u00a0\u00bb.vc_custom_1637080776101{margin-bottom: -5px !important;}\u00a0\u00bb accent_color=\u00a0\u00bb#bababa\u00a0\u00bb i_custom_color=\u00a0\u00bb#bababa\u00a0\u00bb][vc_custom_heading text=\u00a0\u00bbD\u00e9ficit en LCHAD – Cas clinique 2″ font_container=\u00a0\u00bbtag:h1|text_align:center|color:%235f1b52″ use_theme_fonts=\u00a0\u00bbyes\u00a0\u00bb link=\u00a0\u00bburl:https%3A%2F%2Fwww.atlasrleye.com%2Ffr%2Fdeficit-en-lchad%2F|title:D%C3%89FICIT%20EN%20LCHAD|\u00a0\u00bb]

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R\u00e9tinographie ultra-grand champ chez un patient \u00e2g\u00e9 de 8 ans pr\u00e9sentant un d\u00e9ficit en LCHAD (long-chain 3-hydroxyacil.coA dehydrogenase) secondaire \u00e0 la mutation homozygote la plus fr\u00e9quente du g\u00e8ne HADHA. Notez les d\u00e9p\u00f4ts sur la p\u00e9riph\u00e9rie et la mi-p\u00e9riph\u00e9rie. AV: 20\/20 OD.<\/span><\/p>\n

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\"\"Disque de couleur normale, vascularit\u00e9 normale et zones pigment\u00e9es dans la r\u00e9tine p\u00e9riph\u00e9rique et maculaire. Il y avait une accumulation anormale de pigment dans l’\u00e9pith\u00e9lium pigmentaire r\u00e9tinien ce qui provoque des zones d’atrophie dispers\u00e9es de l’\u00e9pith\u00e9lium pigmentaire r\u00e9tinien.<\/span><\/p>\n

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\u00c9valuations
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Autofluorescence<\/p>\n

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Motif principalement hypofluorescent avec quelques zones de fluorescence normale.<\/span><\/p>\n<\/div>\n<\/div>\n<\/div>\n

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OCT (B-Scan) longitudinal<\/p>\n<\/div>\n<\/div>\n

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R\u00e9tinographie Couleur<\/p>\n<\/div>\n<\/div>\n

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Autofluorescence<\/p>\n<\/div>\n<\/div>\n

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Infrarouge<\/p>\n<\/div>\n<\/div>\n

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RNFL \u2013 Carte d\u2019\u00e9paisseur<\/p>\n<\/div>\n<\/div>\n

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OCT (B-Scan) longitudinal<\/p>\n<\/div>\n<\/div>\n

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R\u00e9tinographie Couleur<\/p>\n

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AV: < 20\/250c OD, 20\/100 OG. <\/span><\/p>\n<\/div>\n<\/div>\n<\/div>\n

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