{"id":2051,"date":"2022-09-11T11:10:23","date_gmt":"2022-09-11T11:10:23","guid":{"rendered":"https:\/\/neuropediatoolbox.org\/?p=2051"},"modified":"2022-09-12T15:27:21","modified_gmt":"2022-09-12T15:27:21","slug":"neurofibromatosis-tipo-1","status":"publish","type":"post","link":"https:\/\/neuropediatoolkit.org\/en\/neurofibromatosis-tipo-1\/","title":{"rendered":"Neurofibromatosis tipo 1."},"content":{"rendered":"\n<div id=\"wp-block-themeisle-blocks-accordion-9d3ba09b\" class=\"wp-block-themeisle-blocks-accordion\">\n<details class=\"wp-block-themeisle-blocks-accordion-item\"><summary class=\"wp-block-themeisle-blocks-accordion-item__title\"><div>Criterios diagn\u00f3sticos:<\/div><\/summary><div class=\"wp-block-themeisle-blocks-accordion-item__content\">\n<ul class=\"wp-block-list\"><li>Se establece el diagn\u00f3stico de NF-1 cuando un indiv\u00edduo cumple 2 o m\u00e1s de los siguientes: <ul><li>6 o m\u00e1s manchas caf\u00e9 con leche &gt;5 mm de di\u00e1metro mayor en prep\u00faberes, &gt;15 mm de di\u00e1metro mayor en postp\u00faberes. <\/li><li>Pecas en las regiones axilares o inguinales (freckling). <\/li><li>2 o m\u00e1s neurofibromas de cualquier tipo, o 1 neurofibroma plexiforme. <\/li><li>Glioma del nervio \u00f3ptico. <\/li><li>2 o m\u00e1s n\u00f3dulos de Lisch identificados mediante valoraci\u00f3n con l\u00e1mpara de hendidura, o 2 o m\u00e1s anomalias coroidales (n\u00f3dulos parcheados brillantes identificados por OCT o t\u00e9cnicas de imagen del infrarrojo cercano). <\/li><li>Una lesi\u00f3n \u00f3sea distintiva como la displasia del esfenoides, la curvatura anterolateral de la tibia o la pseudoartrosis de un hueso largo. <\/li><li>Padre o madre que cumple criterios diagn\u00f3sticos de NF-1. <\/li><li>Una mutaci\u00f3n patog\u00e9nica germinal en el gen NF1. <\/li><\/ul><\/li><\/ul>\n<\/div><\/details>\n<\/div>\n\n\n\n<div id=\"wp-block-themeisle-blocks-accordion-f7edd837\" class=\"wp-block-themeisle-blocks-accordion\">\n<details class=\"wp-block-themeisle-blocks-accordion-item\"><summary class=\"wp-block-themeisle-blocks-accordion-item__title\"><div>Diagn\u00f3stico diferencial.<\/div><\/summary><div class=\"wp-block-themeisle-blocks-accordion-item__content\">\n<ul class=\"wp-block-list\"><li>Otras formas de neurofibromatosis: <ul><li>Neurofibromatosis segmentaria.<\/li><li>S\u00edndrome de Legius.<\/li><li>Neurofibromatosis tipo 2.<\/li><li>Schwannomatosis.<\/li><li>S\u00edndrome NF1-Noonan (S\u00edndrome de Watson).<\/li><\/ul><\/li><li>S\u00edndromes de sobrecrecimiento: <ul><li>S\u00edndrome de Klippel-Trenaunay-Weber.<\/li><li>S\u00edndrome de Proteus. <\/li><\/ul><\/li><li>Otras enfermedades con los mismos cambios pigmentarios: <ul><li>S\u00edndrome de McCune-Albright.<\/li><li>S\u00edndrome LEOPARD.<\/li><li>Piebaldismo. <\/li><li>S\u00edndrome de Peutz-Jeghers. <\/li><\/ul><\/li><li>Otras enfermedades con tumores confundibles con neurofibromas. <ul><li>MEN 2B. <\/li><li>Fibromatosis.<\/li><li>Lipomatosis.<\/li><li>S\u00edndrome de Bannayan-Riley-Ruvalcava.<\/li><\/ul><\/li><\/ul>\n<\/div><\/details>\n<\/div>\n\n\n\n<div id=\"wp-block-themeisle-blocks-accordion-61d7960a\" class=\"wp-block-themeisle-blocks-accordion\">\n<details class=\"wp-block-themeisle-blocks-accordion-item\"><summary class=\"wp-block-themeisle-blocks-accordion-item__title\"><div>Cronolog\u00eda de aparici\u00f3n de los s\u00edntomas:<\/div><\/summary><div class=\"wp-block-themeisle-blocks-accordion-item__content\">\n<figure class=\"wp-block-image size-full\"><img fetchpriority=\"high\" decoding=\"async\" width=\"1024\" height=\"538\" src=\"http:\/\/neuropediaclinic.com\/wp-content\/uploads\/2022\/09\/image-18.png\" alt=\"\" class=\"wp-image-2057\" srcset=\"https:\/\/neuropediatoolkit.org\/wp-content\/uploads\/2022\/09\/image-18.png 1024w, https:\/\/neuropediatoolkit.org\/wp-content\/uploads\/2022\/09\/image-18-300x158.png 300w, 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ZP_ATTR\">%7B%22status%22%3A%22success%22%2C%22updateneeded%22%3Afalse%2C%22instance%22%3Afalse%2C%22meta%22%3A%7B%22request_last%22%3A0%2C%22request_next%22%3A0%2C%22used_cache%22%3Atrue%7D%2C%22data%22%3A%5B%7B%22key%22%3A%22U2YLTKQB%22%2C%22library%22%3A%7B%22id%22%3A332710%7D%2C%22meta%22%3A%7B%22creatorSummary%22%3A%22Friedman%22%2C%22parsedDate%22%3A%222022-04-21%22%2C%22numChildren%22%3A1%7D%2C%22bib%22%3A%22%26lt%3Bdiv%20class%3D%26quot%3Bcsl-bib-body%26quot%3B%20style%3D%26quot%3Bline-height%3A%201.35%3B%20%26quot%3B%26gt%3B%5Cn%20%20%26lt%3Bdiv%20class%3D%26quot%3Bcsl-entry%26quot%3B%20style%3D%26quot%3Bclear%3A%20left%3B%20%26quot%3B%26gt%3B%5Cn%20%20%20%20%26lt%3Bdiv%20class%3D%26quot%3Bcsl-left-margin%26quot%3B%20style%3D%26quot%3Bfloat%3A%20left%3B%20padding-right%3A%200.5em%3B%20text-align%3A%20right%3B%20width%3A%201em%3B%26quot%3B%26gt%3B1.%26lt%3B%5C%2Fdiv%26gt%3B%26lt%3Bdiv%20class%3D%26quot%3Bcsl-right-inline%26quot%3B%20style%3D%26quot%3Bmargin%3A%200%20.4em%200%201.5em%3B%26quot%3B%26gt%3BFriedman%20JM.%20Neurofibromatosis%201%20%5BInternet%5D.%20University%20of%20Washington%2C%20Seattle%3B%202022%20%5Bcited%202022%20Sept%2012%5D.%20Available%20from%3A%20%26lt%3Ba%20class%3D%26%23039%3Bzp-ItemURL%26%23039%3B%20href%3D%26%23039%3Bhttps%3A%5C%2F%5C%2Fwww.ncbi.nlm.nih.gov%5C%2Fbooks%5C%2FNBK1109%5C%2F%26%23039%3B%26gt%3Bhttps%3A%5C%2F%5C%2Fwww.ncbi.nlm.nih.gov%5C%2Fbooks%5C%2FNBK1109%5C%2F%26lt%3B%5C%2Fa%26gt%3B%26lt%3B%5C%2Fdiv%26gt%3B%5Cn%20%20%26lt%3B%5C%2Fdiv%26gt%3B%5Cn%26lt%3B%5C%2Fdiv%26gt%3B%22%2C%22data%22%3A%7B%22itemType%22%3A%22book%22%2C%22title%22%3A%22Neurofibromatosis%201%22%2C%22creators%22%3A%5B%7B%22creatorType%22%3A%22author%22%2C%22firstName%22%3A%22Jan%20M.%22%2C%22lastName%22%3A%22Friedman%22%7D%5D%2C%22abstractNote%22%3A%22Neurofibromatosis%201%20%28NF1%29%20is%20a%20multisystem%20disorder%20characterized%20by%20multiple%20caf%5Cu00e9%20au%20lait%20macules%2C%20intertriginous%20freckling%2C%20multiple%20cutaneous%20neurofibromas%2C%20and%20learning%20disability%20or%20behavior%20problems.%20About%20half%20of%20people%20with%20NF1%20have%20plexiform%20neurofibromas%2C%20but%20most%20are%20internal%20and%20not%20suspected%20clinically.%20Plexiform%20neurofibromas%20can%20cause%20pain%2C%20neurologic%20deficits%2C%20and%20abnormalities%20of%20involved%20or%20adjacent%20structures.%20Less%20common%20but%20potentially%20more%20serious%20manifestations%20include%20optic%20nerve%20and%20other%20central%20nervous%20system%20gliomas%2C%20malignant%20peripheral%20nerve%20sheath%20tumors%2C%20scoliosis%2C%20tibial%20dysplasia%2C%20vasculopathy%2C%20and%20gastrointestinal%2C%20endocrine%2C%20or%20pulmonary%20disease.%22%2C%22date%22%3A%222022%5C%2F04%5C%2F21%22%2C%22originalDate%22%3A%22%22%2C%22originalPublisher%22%3A%22%22%2C%22originalPlace%22%3A%22%22%2C%22format%22%3A%22%22%2C%22ISBN%22%3A%22%22%2C%22DOI%22%3A%22%22%2C%22citationKey%22%3A%22%22%2C%22url%22%3A%22https%3A%5C%2F%5C%2Fwww.ncbi.nlm.nih.gov%5C%2Fbooks%5C%2FNBK1109%5C%2F%22%2C%22ISSN%22%3A%22%22%2C%22language%22%3A%22en%22%2C%22collections%22%3A%5B%229PE95M6Q%22%5D%2C%22dateModified%22%3A%222025-11-13T17%3A50%3A53Z%22%7D%7D%5D%7D<\/span>\n\n\t\t\t\t<div id=\"zp-ID-2051-332710-U2YLTKQB\" data-zp-author-date='Friedman-2022-04-21' data-zp-date-author='2022-04-21-Friedman' data-zp-date='2022-04-21' data-zp-year='2022' data-zp-itemtype='book' class=\"zp-Entry zpSearchResultsItem\">\n<div class=\"csl-bib-body\" style=\"line-height: 1.35; \">\n  <div class=\"csl-entry\" style=\"clear: left; \">\n    <div class=\"csl-left-margin\" style=\"float: left; padding-right: 0.5em; text-align: right; width: 1em;\">1.<\/div><div class=\"csl-right-inline\" style=\"margin: 0 .4em 0 1.5em;\">Friedman JM. Neurofibromatosis 1 [Internet]. University of Washington, Seattle; 2022 [cited 2022 Sept 12]. Available from: <a class='zp-ItemURL' href='https:\/\/www.ncbi.nlm.nih.gov\/books\/NBK1109\/'>https:\/\/www.ncbi.nlm.nih.gov\/books\/NBK1109\/<\/a><\/div>\n  <\/div>\n<\/div>\n\t\t\t\t<\/div><!-- .zp-Entry .zpSearchResultsItem -->\n\t\t\t<\/div><!-- .zp-zp-SEO-Content -->\n\t\t<\/div><!-- .zp-List -->\n\t<\/div><!--.zp-Zotpress-->\n\n\n\n\n\n<p><a href=\"https:\/\/neurofibromatosis.es\/\">Asociaci\u00f3n de afectados de neurofibromatosis.<\/a><\/p>\n","protected":false},"excerpt":{"rendered":"<p>Asociaci\u00f3n de afectados de neurofibromatosis.<\/p>","protected":false},"author":1,"featured_media":2063,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"_themeisle_gutenberg_block_has_review":false,"footnotes":""},"categories":[45,46],"tags":[],"class_list":["post-2051","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-neurocutaneas","category-riesgo-oncologico","entry"],"_links":{"self":[{"href":"https:\/\/neuropediatoolkit.org\/en\/wp-json\/wp\/v2\/posts\/2051","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=2051"}],"version-history":[{"count":0,"href":"https:\/\/neuropediatoolkit.org\/en\/wp-json\/wp\/v2\/posts\/2051\/revisions"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/neuropediatoolkit.org\/en\/wp-json\/wp\/v2\/media\/2063"}],"wp:attachment":[{"href":"https:\/\/neuropediatoolkit.org\/en\/wp-json\/wp\/v2\/media?parent=2051"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/neuropediatoolkit.org\/en\/wp-json\/wp\/v2\/categories?post=2051"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/neuropediatoolkit.org\/en\/wp-json\/wp\/v2\/tags?post=2051"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}