{"id":7476,"date":"2020-04-24T15:50:39","date_gmt":"2020-04-24T18:50:39","guid":{"rendered":"https:\/\/doctorhoogstra.com\/es\/sindrome-de-chediak-higashi-2\/"},"modified":"2020-04-27T16:17:53","modified_gmt":"2020-04-27T19:17:53","slug":"sindrome-de-chediak-higashi-2","status":"publish","type":"wiki","link":"https:\/\/doctorhoogstra.com\/pt\/wiki\/sindrome-de-chediak-higashi-2\/","title":{"rendered":"S\u00edndrome de Chediak-Higashi"},"content":{"rendered":"<p><\/p><div id=\"ez-toc-container\" class=\"ez-toc-v2_0_88 counter-hierarchy ez-toc-counter ez-toc-grey ez-toc-container-direction\">\n<div class=\"ez-toc-title-container\">\n<p class=\"ez-toc-title\" style=\"cursor:inherit\">Conte\u00fado<\/p>\n<span class=\"ez-toc-title-toggle\"><a href=\"#\" class=\"ez-toc-pull-right ez-toc-btn ez-toc-btn-xs ez-toc-btn-default ez-toc-toggle\" aria-label=\"Alternar tabela de conte\u00fado\"><span class=\"ez-toc-js-icon-con\"><span class=\"\"><span class=\"eztoc-hide\" style=\"display:none;\">Alternar<\/span><span class=\"ez-toc-icon-toggle-span\"><svg style=\"fill: #999;color:#999\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\" class=\"list-377408\" width=\"20px\" height=\"20px\" viewbox=\"0 0 24 24\" fill=\"none\"><path d=\"M6 6H4v2h2V6zm14 0H8v2h12V6zM4 11h2v2H4v-2zm16 0H8v2h12v-2zM4 16h2v2H4v-2zm16 0H8v2h12v-2z\" fill=\"currentColor\"><\/path><\/svg><svg style=\"fill: #999;color:#999\" class=\"arrow-unsorted-368013\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\" width=\"10px\" height=\"10px\" viewbox=\"0 0 24 24\" version=\"1.2\" baseprofile=\"tiny\"><path d=\"M18.2 9.3l-6.2-6.3-6.2 6.3c-.2.2-.3.4-.3.7s.1.5.3.7c.2.2.4.3.7.3h11c.3 0 .5-.1.7-.3.2-.2.3-.5.3-.7s-.1-.5-.3-.7zM5.8 14.7l6.2 6.3 6.2-6.3c.2-.2.3-.5.3-.7s-.1-.5-.3-.7c-.2-.2-.4-.3-.7-.3h-11c-.3 0-.5.1-.7.3-.2.2-.3.5-.3.7s.1.5.3.7z\"\/><\/svg><\/span><\/span><\/span><\/a><\/span><\/div>\n<nav><ul class='ez-toc-list ez-toc-list-level-1 eztoc-toggle-hide-by-default' ><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-1\" href=\"https:\/\/doctorhoogstra.com\/pt\/wiki\/sindrome-de-chediak-higashi-2\/#%C2%BFQue-es-Chediak-Higashi-sindrome\" >O que \u00e9 o Chediak-Higashi? s\u00edndrome?<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-2\" href=\"https:\/\/doctorhoogstra.com\/pt\/wiki\/sindrome-de-chediak-higashi-2\/#%C2%BFQuien-contrae-el-sindrome-de-Chediak-Higashi\" >Quem contrai a s\u00edndrome de Chediak-Higashi?<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-3\" href=\"https:\/\/doctorhoogstra.com\/pt\/wiki\/sindrome-de-chediak-higashi-2\/#%C2%BFQue-causa-el-sindrome-de-Chediak-Higashi\" >O que causa a s\u00edndrome de Chediak-Higashi?<\/a><ul class='ez-toc-list-level-5' ><li class='ez-toc-heading-level-5'><ul class='ez-toc-list-level-5' ><li class='ez-toc-heading-level-5'><ul class='ez-toc-list-level-5' ><li class='ez-toc-heading-level-5'><a class=\"ez-toc-link ez-toc-heading-4\" href=\"https:\/\/doctorhoogstra.com\/pt\/wiki\/sindrome-de-chediak-higashi-2\/#Genetica-del-sindrome-de-Chediak-Higashi\" >Gen\u00e9tica da s\u00edndrome de Chediak-Higashi *<\/a><\/li><\/ul><\/li><\/ul><\/li><\/ul><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-5\" href=\"https:\/\/doctorhoogstra.com\/pt\/wiki\/sindrome-de-chediak-higashi-2\/#%C2%BFCuales-son-los-sintomas-clinicos-del-sindrome-de-Chediak-Higashi\" >Quais s\u00e3o os sintomas cl\u00ednicos da s\u00edndrome de Chediak-Higashi?<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-6\" href=\"https:\/\/doctorhoogstra.com\/pt\/wiki\/sindrome-de-chediak-higashi-2\/#Complicaciones-del-sindrome-de-Chediak-Higashi\" >Complica\u00e7\u00f5es da s\u00edndrome de Chediak-Higashi<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-7\" href=\"https:\/\/doctorhoogstra.com\/pt\/wiki\/sindrome-de-chediak-higashi-2\/#%C2%BFComo-se-diagnostica-el-sindrome-de-Chediak-Higashi\" >Como \u00e9 diagnosticada a s\u00edndrome de Chediak-Higashi?<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-8\" href=\"https:\/\/doctorhoogstra.com\/pt\/wiki\/sindrome-de-chediak-higashi-2\/#%C2%BFComo-se-trata-el-sindrome-de-Chediak-Higashi\" >Como \u00e9 tratada a s\u00edndrome de Chediak-Higashi?<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-9\" href=\"https:\/\/doctorhoogstra.com\/pt\/wiki\/sindrome-de-chediak-higashi-2\/#%C2%BFCual-es-el-pronostico-para-el-sindrome-de-Chediak-Higashi\" >Qual \u00e9 o progn\u00f3stico da s\u00edndrome de Chediak-Higashi?<\/a><\/li><\/ul><\/nav><\/div>\n\n<section class=\"textBlock\">\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFQue-es-Chediak-Higashi-sindrome\"><\/span>O que \u00e9 o Chediak-Higashi? <span class=\"term\" data-term-id=\"350\">s\u00edndrome<\/span>?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p class=\"normal\">A s\u00edndrome de Chediak-Higashi \u00e9 um dist\u00farbio infantil que interfere na fun\u00e7\u00e3o do sistema imunol\u00f3gico. Tamb\u00e9m conhecida como s\u00edndrome de Begnez-Cesar, <span class=\"term\" data-term-id=\"438\">gl\u00f3bulo branco<\/span> anormalidade ou defeito do albinismo no assassino natural <span class=\"term\" data-term-id=\"421\">linf\u00f3citos<\/span>.<\/p>\n<p class=\"normal\">Foi descrito pela primeira vez em 1943 pelo Dr. Beguez-Cesar. <\/p>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFQuien-contrae-el-sindrome-de-Chediak-Higashi\"><\/span>Quem contrai a s\u00edndrome de Chediak-Higashi?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p class=\"normal\">A s\u00edndrome de Chediak-Higashi \u00e9 rara.<\/p>\n<ul>\n<li>Estima-se que entre 200 e 500 casos tenham sido relatados em todo o mundo.<\/li>\n<li>a <span class=\"term\" data-term-id=\"578\">incid\u00eancia<\/span> \u00c9 o mesmo em homens e mulheres.<\/li>\n<li>Afeta todas as ra\u00e7as, mas os pesquisadores suspeitam que sua ocorr\u00eancia possa ser pouco relatada.<\/li>\n<\/ul>\n<div id=\"square-placement-country-holder\" class=\"country-dependent advert\">\n<div class=\"box-placement square-placement\">\n<div class=\"advert__frame\">\n<div id=\"dermnet-dermnet-mobbox\">\n<\/div><\/div><\/div><\/div>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFQue-causa-el-sindrome-de-Chediak-Higashi\"><\/span>O que causa a s\u00edndrome de Chediak-Higashi?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p class=\"normal\">A s\u00edndrome de Chediak-Higashi \u00e9 uma <span class=\"term\" data-term-id=\"828\">autoss\u00f4mico<\/span> condi\u00e7\u00e3o recessiva causada por um <span class=\"term\" data-term-id=\"870\">muta\u00e7\u00e3o<\/span> sobre ele <em>LISTA<\/em> <span class=\"term\" data-term-id=\"774\">gene<\/span>. Geralmente devido \u00e0 paternidade <span class=\"term\" data-term-id=\"835\">consanguinidade<\/span>. a <em>LISTA<\/em> gene afeta a cria\u00e7\u00e3o e manuten\u00e7\u00e3o de armazenamento <span class=\"term\" data-term-id=\"520\">gr\u00e2nulos<\/span> e causa problemas com o transporte de uma prote\u00edna atrav\u00e9s das c\u00e9lulas.<\/p>\n<section class=\"textBlock imageLinkBlockTitle\">\n<h5 class=\"colour--primary\"><span class=\"ez-toc-section\" id=\"Genetica-del-sindrome-de-Chediak-Higashi\"><\/span>Gen\u00e9tica da s\u00edndrome de Chediak-Higashi *<span class=\"ez-toc-section-end\"><\/span><\/h5>\n<\/section>\n<section class=\"imageLinkBlock\">\n<div class=\"flex\">\n<div class=\"imageLinkBlock__item__image\">\n<p>                        <img alt='S\u00edndrome de chediak-higashi__protectwyjqcm90zwn0il0_focusfillwzi5ncwymjisingildfd-7365485-1814492' src='https:\/\/doctorhoogstra.com\/wp-content\/uploads\/2020\/04\/Chediak-Higashi-Syndrome__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsIngiLDFd-7365485.png'>\n<\/div>\n<p class=\"p-small\">S\u00edndrome de Chediak Higashi<\/p>\n<\/p><\/div>\n<\/section>\n<div class=\"clearfix imageLinkBlock\"><\/div>\n<p style=\"font-size: 10px;\">* Imagem cortesia de Genetics 4 Medics<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFCuales-son-los-sintomas-clinicos-del-sindrome-de-Chediak-Higashi\"><\/span>Quais s\u00e3o os sintomas cl\u00ednicos da s\u00edndrome de Chediak-Higashi?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p class=\"normalCxSpFirst\">Sinais e sintomas come\u00e7am a afetar crian\u00e7as com s\u00edndrome de Chediak-Higashi logo ap\u00f3s o nascimento ou antes dos cinco anos de idade.<\/p>\n<ul>\n<li>Albinismo oculocut\u00e2neo, ou <span class=\"term\" data-term-id=\"139\">hipopigmenta\u00e7\u00e3o<\/span> do <span class=\"term\" data-term-id=\"122\">cabelo<\/span>olhos e pele; o cabelo \u00e9 descrito como de cor clara com um brilho met\u00e1lico<\/li>\n<li>Sangramento prolongado e contus\u00f5es f\u00e1ceis devido a <span class=\"term\" data-term-id=\"1089\">plaquet\u00e1ria<\/span> anormalidades<\/li>\n<li>Virais frequentes <span class=\"term\" data-term-id=\"937\">bacteriano<\/span> infec\u00e7\u00f5es f\u00fangicas<\/li>\n<li><span class=\"term\" data-term-id=\"706\">Fotossensibilidade<\/span><\/li>\n<li>Nistagmo: movimentos oculares anormais e involunt\u00e1rios<\/li>\n<li>Dorm\u00eancia \/ formigamento nos membros devido a <span class=\"term\" data-term-id=\"1374\">perif\u00e9rico<\/span> <span class=\"term\" data-term-id=\"400\">neuropatia<\/span>\n<\/li>\n<li>Baixa imunidade devido a fun\u00e7\u00e3o anormal e ao modo de <span class=\"term\" data-term-id=\"424\">c\u00e9lulas assassinas naturais<\/span>\n<\/li>\n<\/ul>\n<p class=\"normal\">O in\u00edcio da s\u00edndrome de Chediak-Higashi na idade adulta est\u00e1 associado a sintomas mais leves. Estes podem incluir:<\/p>\n<ul>\n<li><span class=\"term\" data-term-id=\"732\">Icter\u00edcia<\/span><\/li>\n<li>Infec\u00e7\u00f5es de pele<\/li>\n<li>\n<span class=\"term\" data-term-id=\"805\">Recorrente<\/span> infec\u00e7\u00f5es do seio ou do trato respirat\u00f3rio<\/li>\n<li>Linfonodos aumentados<\/li>\n<li>Doen\u00e7a gengival (gengivite)<\/li>\n<li>\n<span class=\"term\" data-term-id=\"1928\">Hiperidrose<\/span> e miliaria\n<\/li>\n<li>\n<span class=\"term\" data-term-id=\"1176\">Febre<\/span> sem <span class=\"term\" data-term-id=\"145\">infec\u00e7\u00e3o<\/span>.<\/li>\n<\/ul>\n<h2 class=\"normal\"><span class=\"ez-toc-section\" id=\"Complicaciones-del-sindrome-de-Chediak-Higashi\"><\/span>Complica\u00e7\u00f5es da s\u00edndrome de Chediak-Higashi<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p class=\"normal\">A maioria dos pacientes (cerca de 80%) passar\u00e1 por uma fase acelerada da s\u00edndrome de Chediak-Higashi. Esta fase \u00e9 marcada pela reprodu\u00e7\u00e3o n\u00e3o maligna de gl\u00f3bulos brancos em m\u00faltiplos \u00f3rg\u00e3os do corpo. Esta fase acelerada pode ser <span class=\"term\" data-term-id=\"1497\">precipitado<\/span> pela presen\u00e7a de uma infec\u00e7\u00e3o viral e geralmente \u00e9 fatal se ocorrer na inf\u00e2ncia. Essa fase \u00e9 chamada de linfo-histiocitose hemofagoc\u00edtica (HLH).<\/p>\n<div id=\"square-placement-country-holder\" class=\"country-dependent advert\">\n<div class=\"box-placement square-placement\">\n<div class=\"advert__frame\">\n<div id=\"dermnet-dermnet-mobbox\">\n<\/div><\/div><\/div><\/div>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFComo-se-diagnostica-el-sindrome-de-Chediak-Higashi\"><\/span>Como \u00e9 diagnosticada a s\u00edndrome de Chediak-Higashi?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p class=\"normal\">O diagn\u00f3stico da s\u00edndrome de Chediak-Higashi \u00e9 feito por:<\/p>\n<ul>\n<li>Teste de gl\u00f3bulos brancos para detectar a presen\u00e7a de grandes gr\u00e2nulos.<\/li>\n<li>Luz <span class=\"term\" data-term-id=\"867\">microscopia<\/span> cabelo para encontrar <span class=\"term\" data-term-id=\"231\">pigmento<\/span> aglomera\u00e7\u00e3o<\/li>\n<\/ul>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFComo-se-trata-el-sindrome-de-Chediak-Higashi\"><\/span>Como \u00e9 tratada a s\u00edndrome de Chediak-Higashi?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p class=\"normal\">O tratamento para a s\u00edndrome de Chediak-Higashi pode incluir:<\/p>\n<ul>\n<li>\n<span class=\"term\" data-term-id=\"1762\">Cabo<\/span> sangue ou medula \u00f3ssea <span class=\"term\" data-term-id=\"1792\">transplante<\/span>, que \u00e9 considerado o tratamento de escolha; \u00e9 mais bem sucedido se for feito antes que a crian\u00e7a atinja a fase acelerada da doen\u00e7a<\/li>\n<li>\n<span class=\"term\" data-term-id=\"1619\">Quimioterapia<\/span> \u00e9 necess\u00e1rio colocar hemofagocitose (a fase HLH) em <span class=\"term\" data-term-id=\"1260\">remiss\u00e3o<\/span> antes que um transplante possa ocorrer<\/li>\n<li>Evite a exposi\u00e7\u00e3o \u00e0 luz solar e medidas de prote\u00e7\u00e3o solar.<\/li>\n<\/ul>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFCual-es-el-pronostico-para-el-sindrome-de-Chediak-Higashi\"><\/span>Qual \u00e9 o progn\u00f3stico da s\u00edndrome de Chediak-Higashi?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p class=\"normal\">Sem tratamento, as perspectivas para a s\u00edndrome de Chediak-Higashi s\u00e3o ruins. Cerca de 50-85% de crian\u00e7as com esse dist\u00farbio entrar\u00e1 na fase acelerada do HLH. Esse processo \u00e9 fatal sem tratamento e a maioria dos pacientes morre aos 10 anos.<\/p>\n<\/section>","protected":false},"excerpt":{"rendered":"<p>\u00bfQu\u00e9 es Chediak-Higashi? s\u00edndrome? El s\u00edndrome de Chediak-Higashi es un trastorno infantil que interfiere con la funci\u00f3n del sistema inmunitario. Tambi\u00e9n se conoce como s\u00edndrome de Begnez-Cesar, leucocito anomal\u00eda albinismo&#8230;<\/p>","protected":false},"author":8,"featured_media":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","format":"standard","meta":{"footnotes":""},"categories":[204],"tags":[],"class_list":["post-7476","wiki","type-wiki","status-publish","format-standard","category-glosario-definiciones"],"_links":{"self":[{"href":"https:\/\/doctorhoogstra.com\/pt\/wp-json\/wp\/v2\/wiki\/7476","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/doctorhoogstra.com\/pt\/wp-json\/wp\/v2\/wiki"}],"about":[{"href":"https:\/\/doctorhoogstra.com\/pt\/wp-json\/wp\/v2\/types\/wiki"}],"author":[{"embeddable":true,"href":"https:\/\/doctorhoogstra.com\/pt\/wp-json\/wp\/v2\/users\/8"}],"replies":[{"embeddable":true,"href":"https:\/\/doctorhoogstra.com\/pt\/wp-json\/wp\/v2\/comments?post=7476"}],"version-history":[{"count":0,"href":"https:\/\/doctorhoogstra.com\/pt\/wp-json\/wp\/v2\/wiki\/7476\/revisions"}],"wp:attachment":[{"href":"https:\/\/doctorhoogstra.com\/pt\/wp-json\/wp\/v2\/media?parent=7476"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/doctorhoogstra.com\/pt\/wp-json\/wp\/v2\/categories?post=7476"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/doctorhoogstra.com\/pt\/wp-json\/wp\/v2\/tags?post=7476"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}