{"id":2239,"date":"2018-10-03T09:12:27","date_gmt":"2018-10-03T09:12:27","guid":{"rendered":"https:\/\/exciting-elbakyan.137-74-93-36.plesk.page\/index.php\/2018\/10\/03\/informacion-esencial-para-la-atencion-a-los-pacientes-con-ataxia-telangiectasia-2\/"},"modified":"2026-03-02T12:02:14","modified_gmt":"2026-03-02T12:02:14","slug":"informacion-esencial-para-la-atencion-a-los-pacientes-con-ataxia-telangiectasia-2","status":"publish","type":"post","link":"https:\/\/exciting-elbakyan.137-74-93-36.plesk.page\/index.php\/2018\/10\/03\/informacion-esencial-para-la-atencion-a-los-pacientes-con-ataxia-telangiectasia-2\/","title":{"rendered":"INFORMACI\u00d3N ESENCIAL PARA LA ATENCI\u00d3N A LOS PACIENTES CON ATAXIA TELANGIECTASIA"},"content":{"rendered":"<p>Elaborado por: Jenny Wright y Howard M. Lederman del A-T Clinical Center del Johns Hopkins Children\u2019s Center y John T. Sandlund de la A-T Cancer Consultations del St. Jude Children\u2019s Research Hospital.<\/p>\n<p>La ataxia telangiectasia (A-T) es una enfermedad autos\u00f3mica recesiva caracterizada por:<\/p>\n<ul>\n<li>Degeneraci\u00f3n neurol\u00f3gica progresiva con ataxia, movimientos oculares anormales, disartria y problemas al tragar.<\/li>\n<li>Inmunodeficiencia con linfopenia e hipogammaglobulinemia de gravedad variable.\n<ul>\n<li>\u20de\u20de\u20de &nbsp;&nbsp;Tengo d\u00e9ficit de anticuerpos y recibo terapia de gammaglobulinas<\/li>\n<\/ul>\n<\/li>\n<li>Predisposici\u00f3n a una amplia variedad de c\u00e1nceres (linfomas y leucemias son los m\u00e1s frecuentes, especialmente en ni\u00f1os)<\/li>\n<li>Telangiectasias (presentes frecuentemente), especialmente sobre la escler\u00f3tica. En algunos pocos casos las telangiectasias pueden causar complicaciones en la vejiga, cerebro y otros \u00f3rganos.<\/li>\n<\/ul>\n<p><b>Uso diagn\u00f3stico de rayos X y Tomograf\u00eda Computerizada en A-T<\/b><\/p>\n<p>Los pacientes con A-T presentan una mayor sensibilidad a las radiaciones ionizantes ( rayos X y radiaci\u00f3n gamma).<\/p>\n<ul>\n<li>Los rayos X deber\u00edan emplearse s\u00f3lo si de su resultado puede cambiar la atenci\u00f3n m\u00e9dica.<\/li>\n<li>Si un paciente con A-T tiene fiebre, tos y sonidos respiratorios caracter\u00edsticos de neumon\u00eda, se puede realizar el diagn\u00f3stico cl\u00ednico de neumon\u00eda y pueden administrarse antibi\u00f3ticos sin confirmaci\u00f3n radiogr\u00e1fica. Si los s\u00edntomas persisten a pesar de los antibi\u00f3ticos, una radiograf\u00eda tor\u00e1cica de seguimiento puede ser \u00fatil.<\/li>\n<li>Los procedimientos radiol\u00f3gicos deben evitarse como cribado rutinario de problemas dentales, pero pueden ser razonables para valorar un dolor dental.<\/li>\n<li>A fin de minimizar la radiaci\u00f3n recibida, a los pacientes deber\u00edan realizarse radiograf\u00edas tor\u00e1cicas frontales, o t\u00e9cnicas que minimicen radiaci\u00f3n en TC (p.ej. menor n\u00famero de im\u00e1genes por TC).<\/li>\n<li>No existe ninguna contraindicaci\u00f3n para la realizaci\u00f3n de pruebas por resonancia magn\u00e9tica o ultrasonidos.<\/li>\n<\/ul>\n<p><b>Manejo de infecci\u00f3n aguda<\/b><\/p>\n<p>Los pacientes con A-T presentan predisposici\u00f3n a infecciones del tracto respiratorio superior e inferior debido a la inmunodeficiencia, aspiraci\u00f3n, alteraciones de la tos.<\/p>\n<ul>\n<li>Deber\u00eda considerarse la administraci\u00f3n de antibi\u00f3ticos para el tratamiento de las infecciones del tracto respiratorio superior o inferior de gravedad, acompa\u00f1ados de fiebre o que persisten por m\u00e1s de 7 d\u00edas.<\/li>\n<li>Se deber\u00edan tomar precauciones para reducir el riesgo de aspiraci\u00f3n durante la enfermedad respiratoria.<\/li>\n<\/ul>\n<p><b>Evaluaci\u00f3n preopearotaria y cirug\u00eda<\/b><\/p>\n<p>Antes de una intervenci\u00f3n quir\u00fargica deber\u00eda valorarse la funci\u00f3n pulmonar en todos los pacientes con A-T independientemente de la edad o que no presenten s\u00edntomas respiratorios cr\u00f3nicos.<\/p>\n<ul>\n<li>Ni\u00f1os y adultos con A-T pueden tener dificultad tras al retirarle la ventilaci\u00f3n despu\u00e9s de la cirug\u00eda o de cualquier otro procedimiento que requiera de cirug\u00eda general.<\/li>\n<li>Deben considerarse posibles alternativas a la anestesia general y estrategias que maximicen la apertura de la v\u00eda respiratoria tras la anestesia.<\/li>\n<li>Despu\u00e9s de colocar el tubo para la gastrostom\u00eda, la alimentaci\u00f3n enteral deber\u00eda introducirse con precauci\u00f3n.<\/li>\n<\/ul>\n<p><b>Traducido por AEFAT del original ingl\u00e9s ofrecido por el AT Children\u2019s Project: <\/b><a href=\"https:\/\/www.atcp.org\/wp-content\/uploads\/2018\/05\/Quick-Info-Card-2017-web.pdf\">https:\/\/www.atcp.org\/wp-content\/uploads\/2018\/05\/Quick-Info-Card-2017-web.pdf<\/a><\/p>\n","protected":false},"excerpt":{"rendered":"<p>Elaborado por: Jenny Wright y Howard M. Lederman del A-T Clinical Center del Johns Hopkins Children\u2019s Center y John T. Sandlund de la A-T Cancer Consultations del St. Jude Children\u2019s Research Hospital. La ataxia telangiectasia (A-T) es una enfermedad autos\u00f3mica recesiva caracterizada por: Degeneraci\u00f3n neurol\u00f3gica progresiva con ataxia, movimientos oculares anormales, disartria y problemas al [&hellip;]<\/p>\n","protected":false},"author":1,"featured_media":0,"comment_status":"closed","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_acf_changed":false,"footnotes":""},"categories":[808],"tags":[],"class_list":["post-2239","post","type-post","status-publish","format-standard","hentry","category-divulgacion-y-medios"],"acf":[],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.2 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>INFORMACI\u00d3N ESENCIAL PARA LA ATENCI\u00d3N A LOS PACIENTES CON ATAXIA TELANGIECTASIA - Aefat<\/title>\n<meta name=\"robots\" content=\"noindex, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<meta property=\"og:locale\" content=\"es_ES\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"INFORMACI\u00d3N ESENCIAL PARA LA ATENCI\u00d3N A LOS PACIENTES CON ATAXIA TELANGIECTASIA - Aefat\" \/>\n<meta property=\"og:description\" content=\"Elaborado por: Jenny Wright y Howard M. 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