{"id":107543,"date":"2026-02-26T14:52:53","date_gmt":"2026-02-26T17:52:53","guid":{"rendered":"https:\/\/med.estrategia.com\/portal\/?p=107543"},"modified":"2026-02-26T14:52:58","modified_gmt":"2026-02-26T17:52:58","slug":"resumo-sobre-sindrome-de-dubin-johnson-definicao-manifestacoes-clinicas-e-mais","status":"publish","type":"post","link":"https:\/\/med.estrategia.com\/portal\/conteudos-gratis\/doencas\/resumo-sobre-sindrome-de-dubin-johnson-definicao-manifestacoes-clinicas-e-mais\/","title":{"rendered":"Resumo sobre S\u00edndrome de Dubin-Johnson: defini\u00e7\u00e3o, manifesta\u00e7\u00f5es cl\u00ednicas e mais!"},"content":{"rendered":"<p>E a&iacute;, doc! Vamos explorar mais um tema essencial? Hoje o foco &eacute; a <strong>S&iacute;ndrome de Dubin-Johnson<\/strong>, uma condi&ccedil;&atilde;o heredit&aacute;ria rara caracterizada por hiperbilirrubinemia conjugada cr&ocirc;nica, causada por um defeito no transporte da bilirrubina pelos hepat&oacute;citos. Apesar da icter&iacute;cia persistente, trata-se de uma s&iacute;ndrome benigna, sem comprometimento da fun&ccedil;&atilde;o hep&aacute;tica e com excelente progn&oacute;stico.<p>O <strong>Estrat&eacute;gia MED<\/strong> est&aacute; aqui para descomplicar esse conceito e ajudar voc&ecirc; a aprofundar seus conhecimentos, promovendo uma pr&aacute;tica cl&iacute;nica cada vez mais eficaz e segura.<\/p><p>Vamos nessa!<\/p><div id=\"ez-toc-container\" class=\"ez-toc-v2_0_79_2 counter-hierarchy ez-toc-counter ez-toc-transparent ez-toc-container-direction\">\n<div class=\"ez-toc-title-container\"><p class=\"ez-toc-title\" style=\"cursor:inherit\">Navegue pelo conte\u00fado<\/p>\n<\/div><nav><ul class='ez-toc-list ez-toc-list-level-1 ' ><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-1\" href=\"https:\/\/med.estrategia.com\/portal\/conteudos-gratis\/doencas\/resumo-sobre-sindrome-de-dubin-johnson-definicao-manifestacoes-clinicas-e-mais\/#Definicao-de-Sindrome-de-Dubin-Johnson\" >Defini&ccedil;&atilde;o de S&iacute;ndrome de Dubin-Johnson<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-2\" href=\"https:\/\/med.estrategia.com\/portal\/conteudos-gratis\/doencas\/resumo-sobre-sindrome-de-dubin-johnson-definicao-manifestacoes-clinicas-e-mais\/#Epidemiologia-da-Sindrome-de-Dubin-Johnson\" >Epidemiologia da S&iacute;ndrome de Dubin-Johnson<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-3\" href=\"https:\/\/med.estrategia.com\/portal\/conteudos-gratis\/doencas\/resumo-sobre-sindrome-de-dubin-johnson-definicao-manifestacoes-clinicas-e-mais\/#Manifestacoes-clinicas-da-Sindrome-de-Dubin-Johnson\" >Manifesta&ccedil;&otilde;es cl&iacute;nicas da S&iacute;ndrome de Dubin-Johnson<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-4\" href=\"https:\/\/med.estrategia.com\/portal\/conteudos-gratis\/doencas\/resumo-sobre-sindrome-de-dubin-johnson-definicao-manifestacoes-clinicas-e-mais\/#Diagnostico-da-Sindrome-de-Dubin-Johnson\" >Diagn&oacute;stico da S&iacute;ndrome de Dubin-Johnson<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-5\" href=\"https:\/\/med.estrategia.com\/portal\/conteudos-gratis\/doencas\/resumo-sobre-sindrome-de-dubin-johnson-definicao-manifestacoes-clinicas-e-mais\/#Tratamento-da-Sindrome-de-Dubin-Johnson\" >Tratamento da S&iacute;ndrome de Dubin-Johnson<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-6\" href=\"https:\/\/med.estrategia.com\/portal\/conteudos-gratis\/doencas\/resumo-sobre-sindrome-de-dubin-johnson-definicao-manifestacoes-clinicas-e-mais\/#Veja-tambem\" >Veja tamb&eacute;m!<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-7\" href=\"https:\/\/med.estrategia.com\/portal\/conteudos-gratis\/doencas\/resumo-sobre-sindrome-de-dubin-johnson-definicao-manifestacoes-clinicas-e-mais\/#Referencias\" >Refer&ecirc;ncias<\/a><\/li><\/ul><\/nav><\/div>\n<h2 class=\"wp-block-heading\" id=\"h-definicao-de-sindrome-de-dubin-johnson\"><span class=\"ez-toc-section\" id=\"Definicao-de-Sindrome-de-Dubin-Johnson\"><\/span>Defini&ccedil;&atilde;o de S&iacute;ndrome de Dubin-Johnson<span class=\"ez-toc-section-end\"><\/span><\/h2><p>A <strong>s&iacute;ndrome de Dubin Johnson<\/strong> &eacute; uma doen&ccedil;a heredit&aacute;ria rara caracterizada por <strong>hiperbilirrubinemia conjugada cr&ocirc;nica<\/strong>, de origem n&atilde;o hemol&iacute;tica, sem sinais de les&atilde;o hep&aacute;tica estrutural ou funcional. Resulta de um defeito gen&eacute;tico que compromete a <strong>excre&ccedil;&atilde;o da bilirrubina conjugada pelos hepat&oacute;citos<\/strong>, levando ao seu ac&uacute;mulo no sangue e ao dep&oacute;sito de um pigmento escuro caracter&iacute;stico no f&iacute;gado.<\/p><p>Geralmente manifesta-se na <strong>segunda d&eacute;cada de vida<\/strong>, embora possa ocorrer mais precocemente, inclusive na inf&acirc;ncia. Trata-se de uma condi&ccedil;&atilde;o <strong>benigna<\/strong>, sem repercuss&otilde;es cl&iacute;nicas significativas a longo prazo, que <strong>n&atilde;o requer tratamento espec&iacute;fico<\/strong>, sendo fundamental principalmente para o diagn&oacute;stico diferencial de outras doen&ccedil;as hepatobiliares potencialmente mais graves.<\/p><h2 class=\"wp-block-heading\" id=\"h-epidemiologia-da-sindrome-de-dubin-johnson\"><span class=\"ez-toc-section\" id=\"Epidemiologia-da-Sindrome-de-Dubin-Johnson\"><\/span>Epidemiologia da S&iacute;ndrome de Dubin-Johnson<span class=\"ez-toc-section-end\"><\/span><\/h2><p>A <strong>epidemiologia da s&iacute;ndrome de Dubin Johnson<\/strong> caracteriza-se por se tratar de uma condi&ccedil;&atilde;o <strong>rara<\/strong>, com ocorr&ecirc;ncia semelhante entre <strong>homens e mulheres<\/strong>. A manifesta&ccedil;&atilde;o cl&iacute;nica ocorre, na maioria dos casos, durante a <strong>adolesc&ecirc;ncia ou no in&iacute;cio da vida adulta<\/strong>, sendo <strong>incomum o diagn&oacute;stico na primeira d&eacute;cada de vida<\/strong>.<\/p><p>Embora acometa indiv&iacute;duos de <strong>todas as ra&ccedil;as e nacionalidades<\/strong>, observa-se uma <strong>maior frequ&ecirc;ncia entre judeus sefarditas<\/strong>. Apesar de afetar ambos os sexos, a s&iacute;ndrome tende a apresentar <strong>in&iacute;cio mais precoce em indiv&iacute;duos do sexo masculino<\/strong>.<\/p><h2 class=\"wp-block-heading\" id=\"h-manifestacoes-clinicas-da-sindrome-de-dubin-johnson\"><span class=\"ez-toc-section\" id=\"Manifestacoes-clinicas-da-Sindrome-de-Dubin-Johnson\"><\/span>Manifesta&ccedil;&otilde;es cl&iacute;nicas da S&iacute;ndrome de Dubin-Johnson<span class=\"ez-toc-section-end\"><\/span><\/h2><p>As <strong>manifesta&ccedil;&otilde;es cl&iacute;nicas da s&iacute;ndrome de Dubin Johnson<\/strong> s&atilde;o geralmente <strong>leves ou ausentes<\/strong>, sendo mais comum em <strong>adultos jovens<\/strong>. Na maioria dos casos, a <strong>hiperbilirrubinemia conjugada<\/strong> &eacute; identificada de forma <strong>incidental<\/strong>, durante exames laboratoriais de rotina ou investiga&ccedil;&otilde;es por outros motivos.<\/p><p>Quando h&aacute; sintomas, estes costumam ser discretos, destacando-se <strong>icter&iacute;cia leve<\/strong>, podendo ocorrer <strong>astenia<\/strong> e, raramente, <strong>dor abdominal superior<\/strong>. O <strong>prurido n&atilde;o est&aacute; presente<\/strong>, uma vez que os n&iacute;veis s&eacute;ricos de &aacute;cidos biliares permanecem normais.<\/p><p>Em mulheres, a condi&ccedil;&atilde;o pode permanecer <strong>assintom&aacute;tica<\/strong> e tornar-se evidente durante o <strong>uso de contraceptivos orais<\/strong> ou na <strong>gesta&ccedil;&atilde;o<\/strong>, situa&ccedil;&otilde;es que podem desencadear ou intensificar a icter&iacute;cia. De modo geral, o <strong>exame f&iacute;sico &eacute; normal<\/strong>, exceto pela presen&ccedil;a de icter&iacute;cia discreta.<\/p><h2 class=\"wp-block-heading\" id=\"h-diagnostico-da-sindrome-de-dubin-johnson\"><span class=\"ez-toc-section\" id=\"Diagnostico-da-Sindrome-de-Dubin-Johnson\"><\/span>Diagn&oacute;stico da S&iacute;ndrome de Dubin-Johnson<span class=\"ez-toc-section-end\"><\/span><\/h2><p>O principal achado laboratorial &eacute; a <strong>hiperbilirrubinemia conjugada<\/strong>, com n&iacute;veis de bilirrubina total geralmente entre <strong>2 e 5 mg\/dL<\/strong>, podendo raramente atingir valores mais elevados. Os demais exames laboratoriais permanecem <strong>normais<\/strong>, incluindo hemograma, albumina s&eacute;rica, aminotransferases, fosfatase alcalina, colesterol e tempo de protrombina. N&atilde;o h&aacute; evid&ecirc;ncias de <strong>hem&oacute;lise<\/strong>.<\/p><h3 class=\"wp-block-heading\" id=\"h-coproporfirinas-urinarias\">Coproporfirinas urin&aacute;rias<\/h3><p>Um dado diagn&oacute;stico caracter&iacute;stico &eacute; a altera&ccedil;&atilde;o na <strong>excre&ccedil;&atilde;o urin&aacute;ria de coproporfirinas<\/strong>. Embora a quantidade total seja normal, observa-se uma <strong>predomin&acirc;ncia do is&ocirc;mero I<\/strong>, correspondendo a mais de <strong>80% do total<\/strong>, enquanto em indiv&iacute;duos saud&aacute;veis predomina o is&ocirc;mero III.<\/p><h3 class=\"wp-block-heading\" id=\"h-exames-de-imagem\">Exames de imagem<\/h3><p>Exames como a <strong>cintilografia hepatobiliar (HIDA)<\/strong> e a <strong>tomografia computadorizada<\/strong> podem apresentar achados sugestivos, como reten&ccedil;&atilde;o prolongada do radiof&aacute;rmaco no f&iacute;gado. No entanto, esses m&eacute;todos <strong>n&atilde;o s&atilde;o necess&aacute;rios<\/strong> quando o diagn&oacute;stico &eacute; suspeitado clinicamente e confirmado por exames laboratoriais, pois podem levar a interpreta&ccedil;&otilde;es equivocadas e procedimentos desnecess&aacute;rios.<\/p><h3 class=\"wp-block-heading\" id=\"h-testes-funcionais-antigos\">Testes funcionais antigos<\/h3><p>O teste de depura&ccedil;&atilde;o da <strong>bromossulfale&iacute;na<\/strong> n&atilde;o &eacute; mais utilizado na pr&aacute;tica cl&iacute;nica. Quando realizado, pode mostrar um padr&atilde;o bif&aacute;sico indicativo de defeito excretor, por&eacute;m n&atilde;o &eacute; espec&iacute;fico da s&iacute;ndrome.<\/p><h3 class=\"wp-block-heading\" id=\"h-biopsia-hepatica\">Bi&oacute;psia hep&aacute;tica<\/h3><p>A bi&oacute;psia hep&aacute;tica pode evidenciar o dep&oacute;sito de <strong>pigmento escuro granular<\/strong> nos hepat&oacute;citos centrolobulares, com arquitetura hep&aacute;tica preservada. Apesar disso, <strong>n&atilde;o &eacute; recomendada<\/strong> como m&eacute;todo diagn&oacute;stico de rotina.<\/p><h3 class=\"wp-block-heading\" id=\"h-confirmacao-diagnostica\">Confirma&ccedil;&atilde;o diagn&oacute;stica<\/h3><p>O diagn&oacute;stico da s&iacute;ndrome de Dubin Johnson &eacute; estabelecido pela presen&ccedil;a de <strong>hiperbilirrubinemia conjugada isolada<\/strong>, associada a <strong>testes de fun&ccedil;&atilde;o hep&aacute;tica normais<\/strong> e <strong>aumento da fra&ccedil;&atilde;o urin&aacute;ria de coproporfirina I<\/strong>. M&eacute;todos invasivos devem ser evitados. A an&aacute;lise gen&eacute;tica do gene <strong>ABCC2<\/strong> pode ser realizada, mas &eacute; restrita a fins cient&iacute;ficos e n&atilde;o &eacute; utilizada rotineiramente na pr&aacute;tica cl&iacute;nica.<\/p><h2 class=\"wp-block-heading\" id=\"h-tratamento-da-sindrome-de-dubin-johnson\"><span class=\"ez-toc-section\" id=\"Tratamento-da-Sindrome-de-Dubin-Johnson\"><\/span>Tratamento da S&iacute;ndrome de Dubin-Johnson<span class=\"ez-toc-section-end\"><\/span><\/h2><p>O tratamento da s&iacute;ndrome de Dubin Johnson baseia-se no reconhecimento de que se trata de uma <strong>condi&ccedil;&atilde;o benigna<\/strong>, que <strong>n&atilde;o evolui para fibrose hep&aacute;tica nem cirrose<\/strong>. Dessa forma, <strong>n&atilde;o h&aacute; necessidade de tratamento espec&iacute;fico<\/strong> na maioria dos casos.<\/p><p>A principal relev&acirc;ncia cl&iacute;nica do diagn&oacute;stico est&aacute; em <strong>excluir outras doen&ccedil;as hepatobiliares<\/strong> que possam causar les&atilde;o hep&aacute;tica e que demandem interven&ccedil;&atilde;o terap&ecirc;utica.<\/p><p>Em situa&ccedil;&otilde;es especiais, como na <strong>forma neonatal da s&iacute;ndrome<\/strong>, pode ocorrer <strong>colestase significativa<\/strong>. Nesses casos, o uso de <strong>fenobarbital<\/strong> e <strong>&aacute;cido ursodesoxic&oacute;lico<\/strong> pode ser indicado, com o objetivo de melhorar o fluxo biliar e reduzir a colestase.<\/p><h2 class=\"wp-block-heading\" id=\"h-veja-tambem\"><span class=\"ez-toc-section\" id=\"Veja-tambem\"><\/span>Veja tamb&eacute;m!<span class=\"ez-toc-section-end\"><\/span><\/h2><ul class=\"wp-block-list\">\n<li><a href=\"https:\/\/med.estrategia.com\/portal\/conteudos-gratis\/doencas\/resumo-sobre-sindrome-de-rotor-definicao-manifestacoes-clinicas-e-mais\/\">Resumo sobre S&iacute;ndrome de Rotor: defini&ccedil;&atilde;o, manifesta&ccedil;&otilde;es cl&iacute;nicas e mais!<\/a><\/li>\n\n\n\n<li><a href=\"https:\/\/med.estrategia.com\/portal\/conteudos-gratis\/doencas\/resumo-sobre-doenca-de-hirschsprung-definicao-manifestacoes-clinicas-e-mais\/\">Resumo sobre Doen&ccedil;a de Hirschsprung: defini&ccedil;&atilde;o, manifesta&ccedil;&otilde;es cl&iacute;nicas e mais!<\/a><\/li>\n\n\n\n<li><a href=\"https:\/\/med.estrategia.com\/portal\/conteudos-gratis\/doencas\/resumo-sobre-sindrome-de-silver-russel-definicao-caracteristicas-e-mais\/\">Resumo sobre S&iacute;ndrome de Silver-Russel: defini&ccedil;&atilde;o, caracter&iacute;sticas e mais!<\/a><\/li>\n\n\n\n<li><a href=\"https:\/\/med.estrategia.com\/portal\/conteudos-gratis\/doencas\/resumo-sobre-hepatomegalia-anatomia-hepatica-causas-e-mais\/\">Resumo sobre Hepatomegalia: anatomia hep&aacute;tica, causas e mais!<\/a><\/li>\n\n\n\n<li><a href=\"https:\/\/med.estrategia.com\/portal\/conteudos-gratis\/doencas\/resumo-sobre-hiperplasia-nodular-regenerativa-definicao-etiologia-e-mais\/\">Resumo sobre Hiperplasia Nodular Regenerativa: defini&ccedil;&atilde;o, etiologia e mais!<\/a><\/li>\n\n\n\n<li><a href=\"https:\/\/med.estrategia.com\/portal\/conteudos-gratis\/doencas\/resumo-sobre-sindrome-de-boerhaave-definicao-manejo-e-mais\/\">Resumo sobre S&iacute;ndrome de Boerhaave: defini&ccedil;&atilde;o, manejo e mais!<\/a><\/li>\n<\/ul><h2 class=\"wp-block-heading\" id=\"h-referencias\"><span class=\"ez-toc-section\" id=\"Referencias\"><\/span>Refer&ecirc;ncias<span class=\"ez-toc-section-end\"><\/span><\/h2><p>Talaga ZJ, Vaidya PN. Dubin-Johnson Syndrome. [Updated 2023 Jul 10]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan-. Available from: <a href=\"https:\/\/www.ncbi.nlm.nih.gov\/books\/NBK536994\/\" target=\"_blank\" rel=\"noopener noreferrer\">https:\/\/www.ncbi.nlm.nih.gov\/books\/NBK536994\/<\/a><\/p><\/p>\n","protected":false},"excerpt":{"rendered":"E a&iacute;, doc! Vamos explorar mais um tema essencial? Hoje o foco &eacute; a S&iacute;ndrome de Dubin-Johnson, uma&hellip;\n","protected":false},"author":53,"featured_media":107544,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"wl_entities_gutenberg":"","footnotes":""},"categories":[304],"tags":[],"wl_entity_type":[199],"class_list":{"0":"post-107543","1":"post","2":"type-post","3":"status-publish","4":"format-standard","5":"has-post-thumbnail","7":"category-doencas","8":"wl_entity_type-article"},"acf":[],"yoast_head":"<!-- This site is optimized with the Yoast SEO Premium plugin v26.6 (Yoast SEO v26.6) - https:\/\/yoast.com\/wordpress\/plugins\/seo\/ -->\n<title>Resumo sobre S\u00edndrome de Dubin-Johnson: defini\u00e7\u00e3o, manifesta\u00e7\u00f5es cl\u00ednicas e mais!<\/title>\n<meta name=\"description\" content=\"A S\u00edndrome de Dubin-Johnson \u00e9 uma condi\u00e7\u00e3o heredit\u00e1ria rara caracterizada por hiperbilirrubinemia conjugada cr\u00f4nica. 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