{"id":74805,"date":"2021-11-23T15:23:58","date_gmt":"2021-11-23T15:23:58","guid":{"rendered":"https:\/\/www.atlasrleye.com\/amaurosis-congenita-de-leber\/"},"modified":"2025-07-15T12:07:17","modified_gmt":"2025-07-15T12:07:17","slug":"amaurosis-congenita-de-leber","status":"publish","type":"page","link":"https:\/\/www.atlasrleye.com\/es\/amaurosis-congenita-de-leber\/","title":{"rendered":"AMAUROSIS CONG\u00c9NITA DE LEBER"},"content":{"rendered":"

[vc_row][vc_column][vc_custom_heading text=\u00bbS\u00cdNDROMES Y DISTROFIAS RETINIANAS\u00bb font_container=\u00bbtag:h5|font_size:22px|text_align:center|color:%23000000″ use_theme_fonts=\u00bbyes\u00bb link=\u00bburl:https%3A%2F%2Fwww.atlasrleye.com%2Fes%2Fatlas%2F%231636632807269-1444524b-3ed4|\u00bb][vc_text_separator title=\u00bb\u00bb i_icon_fontawesome=\u00bbfas fa-eye\u00bb i_color=\u00bbcustom\u00bb i_size=\u00bblg\u00bb color=\u00bbcustom\u00bb border_width=\u00bb8″ css=\u00bb.vc_custom_1637080776101{margin-bottom: -5px !important;}\u00bb add_icon=\u00bbtrue\u00bb accent_color=\u00bb#bababa\u00bb i_custom_color=\u00bb#bababa\u00bb][vc_custom_heading source=\u00bbpost_title\u00bb font_container=\u00bbtag:h1|font_size:44px|text_align:center|color:%235f1b52″ use_theme_fonts=\u00bbyes\u00bb]

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La amaurosis cong\u00e9nita de Leber (LCA) es una distrofia retiniana y\/o una displasia de inicio pre-natal (autos\u00f3mica recesiva en la mayor\u00eda de los casos). La LCA es gen\u00e9ticamente heterog\u00e9nea.<\/p>\n

Y fueran identificados hasta la fecha 6 genes asociados: AIPL1, CRB1, CRX, GUCY2D, RPE65 y RPGRIP1. La LCA es asociada a retraso mental, anomal\u00edas musculares y esquel\u00e9ticas, sordez y convulsiones. Los s\u00edntomas oculares incluyen deficiencia visual grave, pupilas lentas, nictalop\u00eda, sensibilidad a la luz (50%) y nistagmus.<\/p>\n

Est\u00e1 muchas veces asociada con hipermetrop\u00eda alta, queratocono y cataratas subcapsulares posteriores. El fondo de ojo en el primero mes de vida es frecuentemente normal pero m\u00e1s tarde se observa una granularidad progresiva del epitelio pigmentario de la retina, atenuaci\u00f3n vascular, colobomas maculares, manchas amarillas, fondo en \u201csal y pimienta\u201d, atrofia retinocoroidea o una apariencia de retinopat\u00eda pigmentar.<\/p>\n

Aproximadamente el 10% de los casos tendr\u00e1 una lesi\u00f3n colobomatosa. El electrorretinograma muestra la funci\u00f3n retiniana reducida o ausente. Los resultados con terapia g\u00e9nica, en algunos estudios experimentales, han sido alentadores.<\/p>\n<\/div>\n

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Casos Cl\u00ednicos<\/div><\/div><\/div>[\/vc_column][\/vc_row][vc_row][vc_column][vc_column_text css=\u00bb.vc_custom_1752580911162{margin-bottom: 10px !important;}\u00bb]<\/p>\n

Casos Cl\u00ednicos<\/span><\/strong><\/h1>\n

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Caso Cl\u00ednico 1<\/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=\u00bbS\u00cdNDROMES Y DISTROFIAS RETINIANAS\u00bb font_container=\u00bbtag:h5|font_size:22px|text_align:center|color:%23000000″ use_theme_fonts=\u00bbyes\u00bb link=\u00bburl:https%3A%2F%2Fwww.atlasrleye.com%2Fes%2Fatlas%2F%231636632807269-1444524b-3ed4|\u00bb][vc_text_separator title=\u00bb\u00bb i_icon_fontawesome=\u00bbfas fa-eye\u00bb i_color=\u00bbcustom\u00bb i_size=\u00bblg\u00bb color=\u00bbcustom\u00bb border_width=\u00bb8″ css=\u00bb.vc_custom_1637080776101{margin-bottom: -5px !important;}\u00bb add_icon=\u00bbtrue\u00bb accent_color=\u00bb#bababa\u00bb i_custom_color=\u00bb#bababa\u00bb][vc_custom_heading source=\u00bbpost_title\u00bb font_container=\u00bbtag:h1|font_size:44px|text_align:center|color:%235f1b52″ use_theme_fonts=\u00bbyes\u00bb][\/vc_column][\/vc_row][vc_row][vc_column width=\u00bb3\/4″][vc_column_text] La amaurosis cong\u00e9nita de Leber (LCA) es una distrofia retiniana y\/o una displasia de inicio […]<\/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-74805","page","type-page","status-publish","hentry"],"_links":{"self":[{"href":"https:\/\/www.atlasrleye.com\/es\/wp-json\/wp\/v2\/pages\/74805","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/www.atlasrleye.com\/es\/wp-json\/wp\/v2\/pages"}],"about":[{"href":"https:\/\/www.atlasrleye.com\/es\/wp-json\/wp\/v2\/types\/page"}],"author":[{"embeddable":true,"href":"https:\/\/www.atlasrleye.com\/es\/wp-json\/wp\/v2\/users\/3"}],"replies":[{"embeddable":true,"href":"https:\/\/www.atlasrleye.com\/es\/wp-json\/wp\/v2\/comments?post=74805"}],"version-history":[{"count":8,"href":"https:\/\/www.atlasrleye.com\/es\/wp-json\/wp\/v2\/pages\/74805\/revisions"}],"predecessor-version":[{"id":118156,"href":"https:\/\/www.atlasrleye.com\/es\/wp-json\/wp\/v2\/pages\/74805\/revisions\/118156"}],"wp:attachment":[{"href":"https:\/\/www.atlasrleye.com\/es\/wp-json\/wp\/v2\/media?parent=74805"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}