{"id":3100,"date":"2022-10-15T15:08:11","date_gmt":"2022-10-15T15:08:11","guid":{"rendered":"https:\/\/neuropediatoolbox.org\/?p=3100"},"modified":"2022-10-15T15:24:07","modified_gmt":"2022-10-15T15:24:07","slug":"miastenia-congenita-y-trastorno-por-inactivacion-de-achr-fetal","status":"publish","type":"post","link":"https:\/\/neuropediatoolkit.org\/en\/miastenia-congenita-y-trastorno-por-inactivacion-de-achr-fetal\/","title":{"rendered":"Miastenia cong\u00e9nita y trastorno por inactivaci\u00f3n de AChR fetal."},"content":{"rendered":"\n
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La miastenia neonatal transitoria es una complicaci\u00f3n rara de la miastenia gravis materna. <\/p>\n\n\n\n

Alrededor del 10-15% de los ni\u00f1os nacidos de madres con anticuerpos anti receptor de acetil-colina (AChR), y menos frecuentemente anti kinasa espec\u00edfica muscular (MuSK). <\/p>\n\n\n\n

Los s\u00edntomas suelen ser evidentes a partir del 3\u00ba dia de vida, y en casi todos los casos existe paresia facial bilateral y dificultades para la succi\u00f3n. Los s\u00edntomas suelen desaparecer al mes de vida. <\/p>\n\n\n\n

No obstante en un grupo reducido de pacientes se puede producir un cuadro mucho m\u00e1s grave, con artrogriposis fetal y paresia bulbofacial persistente hasta la edad adulta, llamado s\u00edndrome de inactivaci\u00f3n fetal de AChR, por anticuerpos contra la subunidad gamma fetal, que es reemplazada en el adulto por la subunidad epsilon. <\/p>\n\n\n\n

La inactivaci\u00f3n del receptor se produce por la exposici\u00f3n a los anticuerpos espec\u00edficos en un per\u00edodo cr\u00edtico del desarrollo.<\/p>\n\n\n

\n\n\t\t332710<\/span>\n\t\t{332710:D36HAPFF}<\/span>\n\t\t<\/span>\n\t\t<\/span>\n\t\t<\/span>\n\t\t<\/span>\n <\/span>\n\t\t1<\/span>\n\t\tvancouver<\/span>\n\t\t50<\/span>\n\t\tdefault<\/span>\n\t\t<\/span>\n\t\t<\/span>\n\t\t<\/span>\n\t\t<\/span>\n\t\t<\/span>\n\t\t<\/span>\n\t\t<\/span>\n\t\t<\/span>\n\t\t<\/span>\n\t\t<\/span>\n\t\t<\/span>\n <\/span>\n 3100<\/span>\n\t\thttps:\/\/neuropediatoolkit.org\/wp-content\/plugins\/zotpress\/<\/span>\n\n\t\t
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Hoffmann K, M\u00fcller JS, Stricker S, Megarbane A, Rajab A, Lindner TH, et al. Escobar Syndrome Is a Prenatal Myasthenia Caused by Disruption of the Acetylcholine Receptor Fetal \u03b3 Subunit. The American Journal of Human Genetics [Internet]. 2006 Aug 1 [cited 2022 Oct 15];79(2):303\u201312. Available from: https:\/\/www.sciencedirect.com\/science\/article\/pii\/S0002929707631371<\/a><\/div>\n <\/div>\n<\/div>\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t<\/div>\n\n\n","protected":false},"excerpt":{"rendered":"

La miastenia neonatal transitoria es una complicaci\u00f3n rara de la miastenia gravis materna. Alrededor del 10-15% de los ni\u00f1os nacidos de madres con anticuerpos anti receptor de acetil-colina (AChR), y menos frecuentemente anti kinasa espec\u00edfica muscular (MuSK). Los s\u00edntomas suelen ser evidentes a partir del 3\u00ba dia de vida, y en casi todos los casos … <\/p>\n

Continue reading \u00abMiastenia cong\u00e9nita y trastorno por inactivaci\u00f3n de AChR fetal.\u00bb<\/span><\/a><\/p>","protected":false},"author":1,"featured_media":3101,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"_themeisle_gutenberg_block_has_review":false,"footnotes":""},"categories":[48,8,11],"tags":[],"class_list":["post-3100","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-especificamente-pediatricos","category-neonatal","category-neuromuscular","entry"],"_links":{"self":[{"href":"https:\/\/neuropediatoolkit.org\/en\/wp-json\/wp\/v2\/posts\/3100","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/neuropediatoolkit.org\/en\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/neuropediatoolkit.org\/en\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/neuropediatoolkit.org\/en\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/neuropediatoolkit.org\/en\/wp-json\/wp\/v2\/comments?post=3100"}],"version-history":[{"count":0,"href":"https:\/\/neuropediatoolkit.org\/en\/wp-json\/wp\/v2\/posts\/3100\/revisions"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/neuropediatoolkit.org\/en\/wp-json\/wp\/v2\/media\/3101"}],"wp:attachment":[{"href":"https:\/\/neuropediatoolkit.org\/en\/wp-json\/wp\/v2\/media?parent=3100"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/neuropediatoolkit.org\/en\/wp-json\/wp\/v2\/categories?post=3100"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/neuropediatoolkit.org\/en\/wp-json\/wp\/v2\/tags?post=3100"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}