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  <front>
    <journal-meta>
      <journal-id journal-id-type="publisher-id">abc</journal-id>
      <journal-title-group>
        <journal-title>Arquivos Brasileiros de Cardiologia</journal-title>
        <abbrev-journal-title abbrev-type="publisher">Arq. Bras. Cardiol.</abbrev-journal-title>
      </journal-title-group>
      <issn pub-type="ppub">0066-782X</issn>
      <issn pub-type="epub">1678-4170</issn>
      <publisher>
        <publisher-name>Sociedade Brasileira de Cardiologia - SBC</publisher-name>
        <publisher-loc>S&#227;o Paulo, SP, Brazil</publisher-loc>
      </publisher>
    </journal-meta>
    <article-meta>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v3">9ZzMsVJGrX4ZC6ngmxYBLKj</article-id>
      <article-id pub-id-type="other">00026</article-id>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v2">S0066-782X2010001400026</article-id>
      <article-id pub-id-type="publisher-id" specific-use="scielo-v1">S0066-782X(10)09500426</article-id>
      <article-id pub-id-type="doi">10.1590/S0066-782X2010001400026</article-id>
      <article-categories>
        <subj-group subj-group-type="heading">
          <subject>Relato de Caso</subject>
        </subj-group>
      </article-categories>
      <title-group>
        <article-title>Doen&#231;a de Takayasu com grave envolvimento card&#237;aco e arterial em pr&#233;-escolar</article-title>
      </title-group>
      <contrib-group>
        <contrib contrib-type="author">
          <name>
            <surname>Ara&#250;jo</surname>
            <given-names>F&#225;tima Derlene da Rocha</given-names>
          </name>
          <xref ref-type="aff" rid="aff01"/>
          <xref ref-type="aff" rid="aff02"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Meira</surname>
            <given-names>Zilda Maria Alves</given-names>
          </name>
          <xref ref-type="aff" rid="aff02"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Ara&#250;jo</surname>
            <given-names>Fabiana Arantes de</given-names>
          </name>
          <xref ref-type="aff" rid="aff02"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Severiano</surname>
            <given-names>Glaucia Maria Vasconcelos</given-names>
          </name>
          <xref ref-type="aff" rid="aff02"/>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Brand&#227;o</surname>
            <given-names>Kelly Nascimento</given-names>
          </name>
          <xref ref-type="aff" rid="aff01"/>
          <xref ref-type="aff" rid="aff02"/>
        </contrib>
      </contrib-group>
      <aff id="aff01">
        <addr-line>
          <named-content content-type="city">Belo Horizonte</named-content>
          <named-content content-type="state"/>
        </addr-line>
        <institution content-type="orgname">Hospital Municipal Odilon Behrens</institution>
        <country country="BR">Brazil</country>
      </aff>
      <aff id="aff02">
        <addr-line>
          <named-content content-type="city">Belo Horizonte</named-content>
          <named-content content-type="state">Minas Gerais</named-content>
        </addr-line>
        <institution content-type="orgname">Universidade Federal de Minas Gerais</institution>
        <institution content-type="orgdiv1">Faculdade de Medicina </institution>
        <country country="BR">Brazil</country>
      </aff>
      <pub-date date-type="collection" publication-format="electronic">
        <month>10</month>
        <year>2010</year>
      </pub-date>
      <volume>95</volume>
      <issue>4</issue>
      <fpage>e101</fpage>
      <lpage>e104</lpage>
      <history>
        <date date-type="received">
          <day>12</day>
          <month>08</month>
          <year>2009</year>
        </date>
        <date date-type="accepted">
          <day>21</day>
          <month>10</month>
          <year>2009</year>
        </date>
        <date date-type="rev-recd">
          <day>07</day>
          <month>09</month>
          <year>2009</year>
        </date>
      </history>
      <permissions>
        <license license-type="open-access" xlink:href="http://creativecommons.org/licenses/by-nc/4.0/" xml:lang="en">
          <license-p>This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.</license-p>
        </license>
      </permissions>
      <abstract>
        <p>Neste artigo os autores descrevem um caso de arterite de Takayasu em uma crian&#231;a de apenas 3 anos de idade, ressaltando-se a raridade dessa doen&#231;a nessa faixa et&#225;ria. A crian&#231;a foi atendida em servi&#231;o de urg&#234;ncia em estado p&#243;s-comicial de convuls&#227;o t&#244;nico-cl&#244;nica generalizada. Ap&#243;s exame cl&#237;nico detalhado, vasta proped&#234;utica e evolu&#231;&#227;o do quadro cl&#237;nico, fez-se o diagn&#243;stico de doen&#231;a de Takayasu com grave envolvimento card&#237;aco e arterial. O relato desse caso alerta pediatras e cardiologistas para o reconhecimento dessa entidade em crian&#231;as de baixa idade, nos pa&#237;ses em que ela &#233; pouco diagnosticada.</p>
      </abstract>
      <kwd-group xml:lang="pt" kwd-group-type="author-generated">
        <kwd>Arterite de Takayasu</kwd>
        <kwd>vasculite</kwd>
        <kwd>pr&#233;-escolar</kwd>
        <kwd>angiografia</kwd>
        <kwd>Takayasu arteritis</kwd>
        <kwd>vasculitis</kwd>
        <kwd>angiography</kwd>
        <kwd>Arteritis de Takayasu</kwd>
        <kwd>angiograf&#237;a</kwd>
      </kwd-group>
      <pub-date publication-format="electronic" date-type="pub">
        <day>26</day>
        <month>01</month>
        <year>2011</year>
      </pub-date>
    </article-meta>
  </front>
  <body>
    <p>
      <bold>RELATO DE CASO</bold>
    </p>
    <p>
      <bold>Doen&#231;a de Takayasu com grave envolvimento card&#237;aco e arterial em pr&#233;-escolar</bold>
    </p>
    <p>
      <bold>F&#225;tima Derlene da Rocha Ara&#250;jo<sup>I, II</sup>; Zilda Maria Alves Meira<sup>II</sup>; Fabiana Arantes de Ara&#250;jo<sup>II</sup>; Glaucia Maria Vasconcelos Severiano<sup>II</sup>; Kelly Nascimento Brand&#227;o<sup>I, II</sup></bold>
    </p>
    <p><sup>I</sup>Hospital Municipal Odilon Behrens, Belo Horizonte, MG - Brasil </p>
    <p><sup>II</sup>Faculdade de Medicina - Universidade Federal de Minas Gerais, Belo Horizonte, MG - Brasil</p>
    <p>
      <xref ref-type="corresp" rid="back">Correspond&#234;ncia</xref>
    </p>
    <p>
      <bold>RESUMO</bold>
    </p>
    <p>Neste artigo os autores descrevem um caso de arterite de Takayasu em uma crian&#231;a de apenas 3 anos de idade, ressaltando-se a raridade dessa doen&#231;a nessa faixa et&#225;ria. A crian&#231;a foi atendida em servi&#231;o de urg&#234;ncia em estado p&#243;s-comicial de convuls&#227;o t&#244;nico-cl&#244;nica generalizada. Ap&#243;s exame cl&#237;nico detalhado, vasta proped&#234;utica e evolu&#231;&#227;o do quadro cl&#237;nico, fez-se o diagn&#243;stico de doen&#231;a de Takayasu com grave envolvimento card&#237;aco e arterial. O relato desse caso alerta pediatras e cardiologistas para o reconhecimento dessa entidade em crian&#231;as de baixa idade, nos pa&#237;ses em que ela &#233; pouco diagnosticada.</p>
    <p><bold>Palavras-chave:</bold> Arterite de Takayasu/complica&#231;&#245;es, vasculite, pr&#233;-escolar, angiografia/m&#233;todos.</p>
    <p>
      <bold>Introdu&#231;&#227;o</bold>
    </p>
    <p>A arterite de Takayasu (AT) &#233; uma vasculite granulomatosa cr&#244;nica de etiologia desconhecida, que envolve a aorta e seus principais ramos, produzindo sequelas vasculares com les&#245;es esten&#243;ticas e/ou forma&#231;&#227;o de trombos. As les&#245;es geralmente s&#227;o graves, acarretando disfun&#231;&#227;o de &#243;rg&#227;os e, a grande maioria exige abordagem cir&#250;rgica. &#201; pouco diagnosticada no Brasil provavelmente por dificuldade diagn&#243;stica, mas estudos brasileiros estimam que metade dos acometidos esteja em faixa et&#225;ria menor que 20 anos<sup>1</sup>. O caso descrito a seguir chama a aten&#231;&#227;o pelo extenso acometimento vascular com sequelas graves, em crian&#231;a de baixa idade.</p>
    <p>
      <bold>Relato do caso</bold>
    </p>
    <p>J.E.F.S., 3 anos e 7 meses de idade, masculino, pesando 12 kg, foi admitido em unidade de urg&#234;ncia em estado p&#243;s-comicial de convuls&#227;o t&#244;nico-cl&#244;nica generalizada. Familiares relataram ser o primeiro epis&#243;dio convulsivo e negaram febre, altera&#231;&#245;es gastrointestinais, respirat&#243;rias e uso de medicamentos. A crian&#231;a estava em acompanhamento em raz&#227;o de baixo ganho ponderal. N&#227;o havia hist&#243;ria familiar de epilepsia, sendo filho &#250;nico de casal h&#237;gido. Epidemiologia para tuberculose (TBC) era negativa e havia "pega" vacinal (BCG) adequada.</p>
    <p>O exame cl&#237;nico evidenciou sinais de desnutri&#231;&#227;o, palidez cut&#226;neo-mucosa leve, aus&#234;ncia de edema ou icter&#237;cia, bom estado geral, afebril e taquipneia leve. O exame cardiovascular mostrou <italic>ictus</italic> desviado inferiormente, hipofonese leve de bulhas, taquicardia, aus&#234;ncia de sopros na regi&#227;o precordial, abdominal e dorso. A PA variava entre 125 x 70 a 130 x 80 mmHg (percentil &gt; 95 para a idade, segundo Task Force, 1996). Os pulsos eram reduzidos em membros inferiores e com temperatura menor em rela&#231;&#227;o aos superiores. O f&#237;gado encontrava-se a 3 cm do rebordo costal direito e n&#227;o havia linfadenomegalias e massas abdominais.</p>
    <p>O hemograma apresentava leucograma e plaquetometria normais, anemia leve com microcitose e hipocromia. A PCR foi negativa, VHS baixo, FAN negativo, fator reumatoide negativo, hemoculturas negativas. O ECG mostrou ritmo sinusal, sobrecarga ventricular esquerda e altera&#231;&#245;es da repolariza&#231;&#227;o ventricular. O exame radiogr&#225;fico do t&#243;rax demonstrou aumento da &#225;rea card&#237;aca &#224; custa do ventr&#237;culo esquerdo (VE) e sinais de congest&#227;o pulmonar (<xref ref-type="fig" rid="f1">fig. 1</xref>). A tomografia computadorizada de enc&#233;falo n&#227;o evidenciou altera&#231;&#245;es. No ecocardiograma Doppler foi constatado fra&#231;&#227;o de eje&#231;&#227;o de VE = 38%, grande aumento de c&#226;maras esquerdas e presen&#231;a de grandes trombos no interior da cavidade do VE (<xref ref-type="fig" rid="f1">fig. 1</xref>).</p>
    <fig id="f1">
      <graphic xlink:href="1678-4170-abc-95-04-e101-00026-gf01.jpg"/></fig>
    <p>A ultrassonografia abdominal demonstrou redu&#231;&#227;o do volume do rim direito e altera&#231;&#245;es corticais sugerindo &#225;rea de infarto no polo superior deste. O <italic>duplex scan</italic> de car&#243;tidas foi normal e o Doppler de aorta abdominal evidenciou aneurisma de aorta com trombo em seu interior e vasos il&#237;acos e renais mal visibilizados. A angiotomografia abdominal mostrou espessamento conc&#234;ntrico difuso da por&#231;&#227;o proximal e suprarrenal da aorta abdominal, al&#233;m de aneurismas fusiformes das art&#233;rias il&#237;acas comuns (maior &#224; direita), com presen&#231;a de trombo intraluminal e hipotrofia renal &#224; direita (<xref ref-type="fig" rid="f2">fig. 2</xref>). A cineangiocoronariografia n&#227;o foi realizada em raz&#227;o dos riscos do acesso vascular por causa dos grandes aneurismas de art&#233;rias il&#237;acas.</p>
    <fig id="f2">
      <graphic xlink:href="1678-4170-abc-95-04-e101-00026-gf02.jpg"/></fig>
    <p>
      <bold>Evolu&#231;&#227;o</bold>
    </p>
    <p>Houve melhora do quadro de ICC com uso de furosemida, espironolactona, digoxina e carvedilol. A crian&#231;a recebeu hepariniza&#231;&#227;o e depois dicumar&#237;nico, n&#227;o sendo visibilizados trombos intracavit&#225;rios ao ecocardiograma, e n&#227;o houve novos eventos tromboemb&#243;licos. A crian&#231;a foi submetida a ressec&#231;&#227;o cir&#250;rgica dos aneurismas das art&#233;rias il&#237;acas, com coloca&#231;&#227;o de pr&#243;tese na il&#237;aca direita e aorta abdominal. An&#225;lise anatomopatol&#243;gica do segmento vascular ressecado mostrou periarterite cr&#244;nica inespec&#237;fica com &#225;reas de aterosclerose.</p>
    <p>Durante interna&#231;&#227;o a crian&#231;a apresentou les&#227;o em dorso do p&#233; direito, sugerindo vasculite, al&#233;m de febre, aumento de VHS e PCR, com melhora cl&#237;nica e laboratorial ap&#243;s pulsoterapia com metilprednisolona. Ecocardiograma Doppler realizado ap&#243;s um ano de tratamento evidenciou normaliza&#231;&#227;o da fun&#231;&#227;o sist&#243;lica de VE e redu&#231;&#227;o das c&#226;maras card&#237;acas esquerdas. A angiotomografia tor&#225;cica e abdominal foi repetida e n&#227;o mostrou comprometimento do arco a&#243;rtico nem do tronco braquiocef&#225;lico, mas evidenciou pouco contraste nas art&#233;rias il&#237;acas e grande rede de colaterais (<xref ref-type="fig" rid="f2">fig. 2</xref>). Atualmente, a crian&#231;a apresenta n&#237;veis press&#243;ricos normais, fun&#231;&#227;o renal normal, sem ICC e ganhando peso.</p>
    <p>
      <bold>Discuss&#227;o</bold>
    </p>
    <p>A AT foi inicialmente descrita pelo oftalmologista Mikito Takayasu, em 1908, ao observar aneurismas e anastomoses arteriovenosas na retina<sup>2</sup>. Trata-se de vasculite inflamat&#243;ria, segmentar, cr&#244;nica, de car&#225;ter destrutivo/degenerativo, de extens&#227;o difusa e multifocal que compromete art&#233;rias de grande e m&#233;dio calibre, particularmente a aorta tor&#225;cica e seus ramos maiores, al&#233;m das art&#233;rias pulmonares e coron&#225;rias<sup>2</sup>. Na fase tardia, h&#225; &#225;reas de estenoses intercaladas por dilata&#231;&#245;es aneurism&#225;ticas e segmentos normais, al&#233;m de oclus&#245;es por trombos e coarcta&#231;&#245;es at&#237;picas<sup>1,2</sup>. Tais altera&#231;&#245;es acarretam isquemia em diversos territ&#243;rios, especialmente cerebral, coronariano, arterial perif&#233;rico e renal. Estudos sugerem que fatores imunol&#243;gicos e/ou gen&#233;ticos estejam implicados na g&#234;nese da doen&#231;a<sup>3,4</sup>. A AT &#233; considerada a terceira causa mundial mais comum de vasculite na inf&#226;ncia. Cerca de tr&#234;s quartos dos acometidos est&#227;o entre 10 e 20 anos de idade, com maior incid&#234;ncia no sexo masculino (1:8)<sup>5</sup>.</p>
    <p>Em geral, a doen&#231;a apresenta manifesta&#231;&#227;o trif&#225;sica. A primeira fase resulta do processo inflamat&#243;rio agudo com sintomas inespec&#237;ficos, sem altera&#231;&#245;es dos pulsos. A segunda fase reflete a exacerba&#231;&#227;o da inflama&#231;&#227;o vascular, manifestando-se por dor no trajeto do vaso. Essas fases podem durar de meses a anos. A terceira fase &#233; a fibr&#243;tica e as manifesta&#231;&#245;es s&#227;o secund&#225;rias &#224;s oclus&#245;es arteriais<sup>6-8</sup>. Associa&#231;&#227;o de AT com TBC e tamb&#233;m com artrite reumatoide, doen&#231;a de Crohn, l&#250;pus eritematoso sist&#234;mico, tiroidite, colite ulcerativa e espondilite anquilosante &#233; descrita por v&#225;rios autores<sup>4,9</sup>. No presente caso, n&#227;o foi realizada proped&#234;utica em rela&#231;&#227;o a essas doen&#231;as em raz&#227;o de aus&#234;ncia de outros sinais e sintomas relativos a elas.</p>
    <p>A angiografia &#233; o m&#233;todo de imagem de escolha<sup>5,10</sup>, sendo tradicionalmente usada para avalia&#231;&#227;o dos vasos comprometidos, por&#233;m tem alto risco em raz&#227;o de exposi&#231;&#227;o a radia&#231;&#245;es, ruptura de vasos, hemorragias e infec&#231;&#245;es. N&#227;o foi indicada pela presen&#231;a de aneurismas gigantes em locais de acesso vascular para a realiza&#231;&#227;o do exame. Pelo baixo risco, optou-se pela angiotomografia abdominal e tor&#225;cica, n&#227;o sendo evidenciadas les&#245;es em arco a&#243;rtico, tronco braquiocef&#225;lico e em toda extens&#227;o da aorta tor&#225;cica, mas foi observado fluxo reduzido em il&#237;acas bilateralmente, com forma&#231;&#227;o de rede vascular colateral.</p>
    <p>O caso analisado preencheu os crit&#233;rios diagn&#243;sticos elaborados pelo Col&#233;gio Americano de Reumatologia (ACR)<sup>8,9</sup> e foi classificado em tipo II segundo a classifica&#231;&#227;o de Ueno e cols.<sup>8,9</sup>, modificada por Lupi e Sanchez-Torres<sup>8,9</sup>, compreendendo: envolvimento da aorta tor&#225;cica, descendente e abdominal, sem envolvimento do arco a&#243;rtico<sup>8,9</sup>.</p>
    <p>A terap&#234;utica cl&#237;nica da AT consiste no controle do processo inflamat&#243;rio vascular com o uso de imunossupressores, quando em fase de atividade<sup>4,5</sup>. Na fase cr&#244;nica, a revasculariza&#231;&#227;o cir&#250;rgica &#233; quase sempre necess&#225;ria, com alta taxa de reestenose<sup>10</sup>. No caso descrito, houve disfun&#231;&#227;o mioc&#225;rdica que respondeu bem ao tratamento cl&#237;nico e ao controle da hipertens&#227;o. As altera&#231;&#245;es do sistema nervoso central foram provavelmente decorrentes do desprendimento de trombos intracard&#237;acos, levando ao acidente vascular cerebral, acarretando hemiplegia.</p>
    <p>Pelo caso apresentado e pela revis&#227;o de literatura, conclui-se que o conhecimento dessa entidade pelos pediatras e cardiologistas possibilita o diagn&#243;stico e a conduta adequada, reduzindo a morbimortalidade. A hip&#243;tese de AT deve ser inclu&#237;da no diagn&#243;stico diferencial de casos de miocardiopatia que cursam com hipertens&#227;o arterial, acidentes vasculares cerebrais isqu&#234;micos, assincronia de pulsos entre os membros e hipertens&#227;o renovascular, mesmo em crian&#231;as de baixa idade. Recomenda-se investiga&#231;&#227;o de outras doen&#231;as, especialmente a tuberculose pela grande preval&#234;ncia em nosso meio.</p>
    <p>
      <bold>Potencial Conflito de Interesses</bold>
    </p>
    <p>Declaro n&#227;o haver conflito de interesses pertinentes.</p>
    <p>
      <bold>Fontes de Financiamento</bold>
    </p>
    <p>O presente estudo n&#227;o teve fontes de financiamento externas.</p>
    <p>
      <bold>Vincula&#231;&#227;o Acad&#234;mica</bold>
    </p>
    <p>N&#227;o h&#225; vincula&#231;&#227;o deste estudo a programas de p&#243;s-gradua&#231;&#227;o.</p>
    <p>
      <fn id="back" fn-type="corresp">
        <label>
          <bold>Correspond&#234;ncia:</bold>
        </label>
        <p> Zilda Maria Alves Meira </p>
        <p> R. Roquete Mendon&#231;a, 184/401 </p>
        <p> S&#227;o Jos&#233; - Pampulha </p>
        <p> 31275030 - Belo Horizonte, MG - Brasil </p>
        <p> E-mail: </p>
        <p>
          <email xlink:href="zilda.m.a.m@terra.com.br">zilda.m.a.m@terra.com.br</email>
        </p>
      </fn>
    </p>
    <p>Artigo recebido em 12/08/09; revisado recebido em 07/09/09; aceito em 21/10/09.</p>
  </body>
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  <sub-article article-type="translation" id="TRes" xml:lang="es">
    <front-stub>
      <article-categories/>
      <title-group>
        <article-title xml:lang="es">Enfermedad de takayasu con grave compromiso card&#237;aco y arterial en pre-escolar</article-title>
      </title-group>
      <abstract xml:lang="es">
        <p>En este art&#237;culo los autores describen un caso de arteritis de Takayasu en una criatura de apenas 3 a&#241;os de edad, destac&#225;ndose la rareza de esa enfermedad en esa franja et&#225;rea. La criatura fue atendida en servicio de urgencia en estado post comicial de convulsi&#243;n t&#243;nico-cl&#243;nica generalizada. Despu&#233;s de examen cl&#237;nico detallado, vasta proped&#233;utica y evoluci&#243;n del cuadro cl&#237;nico, se hizo el diagn&#243;stico de enfermedad de Takayasu con grave compromiso card&#237;aco y arterial. El relato de este caso alerta pediatras y cardi&#243;logos sobre el reconocimiento de esa entidad en criaturas de baja edad, en los pa&#237;ses en que &#233;sta es poco diagnosticada.</p>
      </abstract>
    </front-stub>
    <body>
      <p>
        <bold>CASO CL&#205;NICO</bold>
      </p>
      <p>
        <fn id="top-body2">
          <label>
            <bold>Enfermedad de Takayasu con grave compromiso card&#237;aco y arterial en pre-escolar</bold>
          </label>
          <p>
            <bold>F&#225;tima Derlene da Rocha Ara&#250;jo<sup>I, II</sup>; Zilda Maria Alves Meira<sup>II</sup>; Fabiana Arantes de Ara&#250;jo<sup>II</sup>; Glaucia Maria Vasconcelos Severiano<sup>II</sup>; Kelly Nascimento Brand&#227;o<sup>I, II</sup></bold>
          </p>
        </fn>
      </p>
      <p><sup>I</sup>Hospital Municipal Odilon Behrens, Belo Horizonte, MG - Brasil</p>
      <p><sup>II</sup>Faculdade de Medicina - Universidade Federal de Minas Gerais, Belo Horizonte, MG - Brasil</p>
      <p>Correspond&#234;ncia</p>
      <p>
        <bold>RESUMEN</bold>
      </p>
      <p>En este art&#237;culo los autores describen un caso de arteritis de Takayasu en una criatura de apenas 3 a&#241;os de edad, destac&#225;ndose la rareza de esa enfermedad en esa franja et&#225;rea. La criatura fue atendida en servicio de urgencia en estado post comicial de convulsi&#243;n t&#243;nico-cl&#243;nica generalizada. Despu&#233;s de examen cl&#237;nico detallado, vasta proped&#233;utica y evoluci&#243;n del cuadro cl&#237;nico, se hizo el diagn&#243;stico de enfermedad de Takayasu con grave compromiso card&#237;aco y arterial. El relato de este caso alerta pediatras y cardi&#243;logos sobre el reconocimiento de esa entidad en criaturas de baja edad, en los pa&#237;ses en que &#233;sta es poco diagnosticada.</p>
      <p><bold>Palabras clave:</bold> Arteritis de Takayasu/complicaciones, vasculitis, pre-escolar, angiograf&#237;a/m&#233;todos.</p>
      <p>
        <bold>Introducci&#243;n</bold>
      </p>
      <p>La arteritis de Takayasu (AT) es una vasculitis granulomatosa cr&#243;nica de etiolog&#237;a desconocida, que envuelve la aorta y sus principales ramas, produciendo secuelas vasculares con lesiones esten&#243;ticas y/o formaci&#243;n de trombos. Las lesiones generalmente son graves, acarreando disfunci&#243;n de &#243;rganos, y la gran mayor&#237;a exige abordaje quir&#250;rgico. Es poco diagnosticada en el Brasil probablemente por dificultad diagn&#243;stica, pero estudios brasile&#241;os estiman que la mitad de los afectados est&#233; en la franja et&#225;rea menor de 20 a&#241;os<sup>1</sup>. El caso descripto a seguir llama la atenci&#243;n por la extensa afectaci&#243;n vascular con secuelas graves, en criatura de baja edad.</p>
      <p>
        <bold>Relato del caso</bold>
      </p>
      <p>J.Y.F.S., 3 a&#241;os y 7 meses de edad, masculino, pesando 12 kg, fue admitido en unidad de urgencia en estado post-comicial de convulsi&#243;n t&#243;nico-cl&#243;nica generalizada. Familiares relataron que era el primer episodio convulsivo y negaron fiebre, alteraciones gastrointestinales, respiratorias y uso de medicamentos. El ni&#241;o estaba en control en raz&#243;n de baja ganancia ponderal. No hab&#237;a historia familiar de epilepsia, siendo hijo &#250;nico de pareja sana. Epidemiolog&#237;a para tuberculosis (TBC) era negativa y hab&#237;a vacina (BCG) adecuada.</p>
      <p>El examen cl&#237;nico evidenci&#243; signos de desnutrici&#243;n, palidez cut&#225;neo-mucosa leve, ausencia de edema o ictericia, buen estado general, afebril y taquipnea leve. El examen cardiovascular mostr&#243; <italic>ictus</italic> desviado inferiormente, hipofonesis leve de ruidos, taquicardia, ausencia de soplos en la regi&#243;n precordial, abdominal y dorso. La PA variaba entre 125 x 70 a 130 x 80 mmHg (percentil &gt; 95 para la edad, seg&#250;n Task Force, 1996). Los pulsos eran reducidos en miembros inferiores y con temperatura menor en relaci&#243;n a los superiores. El h&#237;gado se encontraba a 3 cm de la RCD y no hab&#237;a linfadenomegalias y masas abdominales.</p>
      <p>El hemograma presentaba leucograma y plaquetometr&#237;a normales, anemia leve con microcitosis e hipocrom&#237;a. La PCR fue negativa, VHS bajo, FAN negativo, factor reumatoide negativo, hemocultivos negativos. El ECG mostr&#243; ritmo sinusal, sobrecarga ventricular izquierda y alteraciones de la repolarizaci&#243;n ventricular. El examen radiogr&#225;fico del t&#243;rax demostr&#243; aumento del &#225;rea card&#237;aca a costa del ventr&#237;culo izquierdo (VI) y se&#241;ales de congesti&#243;n pulmonar (<xref ref-type="fig" rid="f1-body2">fig. 1</xref>). La tomograf&#237;a computarizada de enc&#233;falo no evidenci&#243; alteraciones. En el ecocardiograma Doppler fue constatado FE de VI = 38%, gran aumento de c&#225;maras izquierdas y presencia de grandes trombos en el interior de la cavidad del VI (<xref ref-type="fig" rid="f1-body2">fig. 1</xref>).</p>
      <fig id="f1-body2">
        <graphic xlink:href="1678-4170-abc-95-04-e101-00026-gf01-es.jpg"/></fig>
      <p>La ultrasonograf&#237;a abdominal demostr&#243; reducci&#243;n del volumen del ri&#241;&#243;n derecho y alteraciones corticales sugiriendo &#225;rea de infarto en el polo superior de este. El duplex scan de car&#243;tidas fue normal y el Doppler de aorta abdominal evidenci&#243; aneurisma de aorta con trombo en su interior y vasos il&#237;acos y renales mal visibilizados. La angiotomograf&#237;a abdominal mostr&#243; engrosamiento conc&#233;ntrico difuso de la porci&#243;n proximal y suprarrenal de la aorta abdominal, adem&#225;s de aneurismas fusiformes de las arterias il&#237;acas comunes (mayor a la derecha), con presencia de trombo intraluminal e hipotrofia renal a la derecha (<xref ref-type="fig" rid="f2-body2">fig. 2</xref>). La cineangiocoronariograf&#237;a no fue realizada en raz&#243;n de los riesgos del acceso vascular por causa de los grandes aneurismas de arterias il&#237;acas.</p>
      <fig id="f2-body2">
        <graphic xlink:href="1678-4170-abc-95-04-e101-00026-gf02-es.jpg"/></fig>
      <p>
        <bold>Evoluci&#243;n</bold>
      </p>
      <p>Hubo mejora del cuadro de ICC con uso de furosemida, espironolactona, digoxina y carvedilol. El ni&#241;o recibi&#243; heparinizaci&#243;n y despu&#233;s dicumar&#237;nico, no siendo visibilizados trombos intracavitarios al ecocardiograma, y no hubo nuevos eventos tromboemb&#243;licos. El ni&#241;o fue sometido a resecci&#243;n quir&#250;rgica de los aneurismas de las arterias il&#237;acas, con colocaci&#243;n de pr&#243;tesis en la il&#237;aca derecha y aorta abdominal. An&#225;lisis anatomopatol&#243;gico del segmento vascular resecado mostr&#243; periarteritis cr&#243;nica inespec&#237;fica con &#225;reas de aterosclerosis.</p>
      <p>Durante internaci&#243;n el ni&#241;o present&#243; lesi&#243;n en dorso del pi&#233; derecho, sugiriendo vasculitis, adem&#225;s de fiebre, aumento de VHS y PCR, con mejora cl&#237;nica y de laboratorio despu&#233;s de pulsoterapia con metilprednisolona. Ecocardiograma Doppler realizado despu&#233;s de un a&#241;o de tratamiento evidenci&#243; normalizaci&#243;n de la funci&#243;n sist&#243;lica de VI y reducci&#243;n de las c&#225;maras card&#237;acas izquierdas. La angiotomograf&#237;a tor&#225;cica y abdominal fue repetida y no mostr&#243; compromiso del arco a&#243;rtico ni del tronco braquiocef&#225;lico, pero evidenci&#243; poco contraste en las arterias il&#237;acas y gran red de colaterales (<xref ref-type="fig" rid="f2-body2">fig. 2</xref>). Actualmente, el ni&#241;o presenta niveles pres&#243;ricos normales, funci&#243;n renal normal, sin ICC y ganando peso.</p>
      <p>
        <bold>Discusi&#243;n</bold>
      </p>
      <p>La AT fue inicialmente descripta por el oftalm&#243;logo Mikito Takayasu, en 1908, al observar aneurismas y anastomosis arteriovenosas en la retina<sup>2</sup>. Se trata de vasculitis inflamatoria, segmentaria, cr&#243;nica, de car&#225;cter destructivo/degenerativo, de extensi&#243;n difusa y multifocal que compromete arterias de grande y medio calibre, particularmente la aorta tor&#225;cica y sus ramas mayores, adem&#225;s de las arterias pulmonares y coronarias<sup>2</sup>. En la fase tard&#237;a, hay &#225;reas de estenosis intercaladas por dilataciones aneurism&#225;ticas y segmentos normales, adem&#225;s de oclusiones por trombos y coartaciones at&#237;picas<sup>1,2</sup>. Tales alteraciones acarrean isquemia en diversos territorios, especialmente cerebral, coronario, arterial perif&#233;rico y renal. Estudios sugieren que factores inmunol&#243;gicos y/o gen&#233;ticos est&#233;n implicados en la g&#233;nesis de la enfermedad<sup>3,4</sup>. La AT es considerada la tercera causa mundial m&#225;s com&#250;n de vasculitis en la infancia. Cerca de tres cuartos de los afectados est&#225;n entre 10 y 20 a&#241;os de edad, con mayor incidencia en el sexo masculino (1:8)<sup>5</sup>.</p>
      <p>En general, la enfermedad presenta manifestaci&#243;n trif&#225;sica. La primera fase resulta del proceso inflamatorio agudo con s&#237;ntomas inespec&#237;ficos, sin alteraciones de los pulsos. La segunda fase refleja la exacerbaci&#243;n de la inflamaci&#243;n vascular, manifest&#225;ndose por dolor en el trayecto del vaso. Esas fases pueden durar de meses a a&#241;os. La tercera fase es la fibr&#243;tica y las manifestaciones son secundarias a las oclusiones arteriales<sup>6-8</sup>. Asociaci&#243;n de AT con TBC y tambi&#233;n con artritis reumatoidea, enfermedad de Crohn, lupus eritematoso sist&#233;mico, tiroiditis, colitis ulcerativa y espondilitis anquilosante es descripta por varios autores<sup>4,9</sup>. En el presente caso, no fue realizada proped&#233;utica en relaci&#243;n a esas enfermedades en raz&#243;n de ausencia de otras se&#241;ales y s&#237;ntomas relativos a ellas.</p>
      <p>La angiograf&#237;a es el m&#233;todo de imagen de elecci&#243;n<sup>5,10</sup>, siendo tradicionalmente usada para evaluaci&#243;n de los vasos comprometidos, sin embargo tiene alto riesgo en raz&#243;n de exposici&#243;n a radiaciones, ruptura de vasos, hemorragias y infecciones. No fue indicada por la presencia de aneurismas gigantes en lugares de acceso vascular para la realizaci&#243;n del examen. Por el bajo riesgo, se opt&#243; por la angiotomograf&#237;a abdominal y tor&#225;cica, no siendo evidenciadas lesiones en arco a&#243;rtico, tronco braquiocef&#225;lico y en toda la extensi&#243;n de la aorta tor&#225;cica, pero fue observado flujo reducido en il&#237;acas bilateralmente, con formaci&#243;n de red vascular colateral.</p>
      <p>El caso analizado llen&#243; los criterios diagn&#243;sticos elaborados pelo Colegio Americano de Reumatolog&#237;a (ACR)<sup>8,9</sup> y fue clasificado en tipo II segundo la clasificaci&#243;n de Ueno y cols.<sup>8,9</sup>, modificada por Lupi y Sanchez-Torres<sup>8,9</sup>, comprendiendo: compromiso de la aorta tor&#225;cica, descendente y abdominal, sin compromiso del arco a&#243;rtico<sup>8,9</sup>.</p>
      <p>La terap&#233;utica cl&#237;nica de la AT consiste en el control del proceso inflamatorio vascular con el uso de inmunosupresores, cuando est&#233; en fase de actividad<sup>4,5</sup>. En la fase cr&#243;nica, la revascularizaci&#243;n quir&#250;rgica es casi siempre necesaria, con alta tasa de reestenosis<sup>10</sup>. En el caso descripto, hubo disfunci&#243;n mioc&#225;rdica que respondi&#243; bien al tratamiento cl&#237;nico y al control de la hipertensi&#243;n. Las alteraciones del SNC fueron probablemente consecuencia del desprendimiento de trombos intracard&#237;acos, levando al AVC, acarreando hemiplej&#237;a.</p>
      <p>Por el caso presentado y por la revisi&#243;n de literatura, se concluye que el conocimiento de esa entidad por los pediatras y cardi&#243;logos posibilita el diagn&#243;stico y la conducta adecuada, reduciendo la morbimortalidad. La hip&#243;tesis de AT debe ser incluida en el diagn&#243;stico diferencial de casos de miocardiopat&#237;a que cursan con hipertensi&#243;n arterial, accidentes vasculares cerebrales isqu&#233;micos, asincron&#237;a de pulsos entre los miembros y hipertensi&#243;n renovascular, aun en criaturas de baja edad. Se recomienda investigaci&#243;n de otras enfermedades, especialmente la tuberculosis por la gran prevalencia en nuestro medio.</p>
      <p>
        <bold>Potencial Conflicto de Intereses</bold>
      </p>
      <p>Declaro no haber conflicto de intereses pertinentes.</p>
      <p>
        <bold>Fuentes de Financiamiento</bold>
      </p>
      <p>El presente estudio no tuvo fuentes de financiamiento externas.</p>
      <p>
        <bold>Vinculaci&#243;n Acad&#233;mica</bold>
      </p>
      <p>No hay vinculaci&#243;n de este estudio a programas de post-grado.</p>
      <p>
        <bold>Referencias</bold>
      </p>
    </body>
  </sub-article>
  <sub-article article-type="translation" id="TRen" xml:lang="en">
    <front-stub>
      <article-categories>
        <subj-group subj-group-type="heading">
          <subject>Case Report</subject>
        </subj-group>
      </article-categories>
      <title-group>
        <article-title xml:lang="en">Takayasu's disease with severe heart and arterial involvement in a preschool-age child</article-title>
      </title-group>
      <abstract xml:lang="en">
        <p>The authors describe a case of Takayasu's arteritis in a child of only 3 years of age, emphasizing the rarity of this disease in this age group. The child was admitted to the emergency room in a post-convulsive state after a tonic-clonic seizure. After a detailed clinical examination, extensive diagnostic tests, and observation of the clinical evolution, the diagnosis was Takayasu's disease with severe cardiac and arterial involvement. The report warns pediatricians and cardiologists to awareness of the possibility of this disease in very small infants, in countries where it is underdiagnosed.</p>
      </abstract>
      <kwd-group xml:lang="en">
        <kwd>child preschool</kwd>
        <kwd>vasculitis</kwd>
        <kwd>pre-escolar</kwd>
      </kwd-group>
    </front-stub>
    <body>
      <p>
        <bold>CASE REPORT</bold>
      </p>
      <p>
        <fn id="top-body3">
          <label>
            <bold>Takayasu's disease with severe heart and arterial involvement in a preschool-age child</bold>
          </label>
          <p>
            <bold>F&#225;tima Derlene da Rocha Ara&#250;jo<sup>I, II</sup>; Zilda Maria Alves Meira<sup>II</sup>; Fabiana Arantes de Ara&#250;jo<sup>II</sup>; Glaucia Maria Vasconcelos Severiano<sup>II</sup>; Kelly Nascimento Brand&#227;o<sup>I, II</sup></bold>
          </p>
        </fn>
      </p>
      <p><sup>I</sup>Hospital Municipal Odilon Behrens, Belo Horizonte, MG - Brazil</p>
      <p><sup>II</sup>Faculdade de Medicina - Universidade Federal de Minas Gerais, Belo Horizonte, MG - Brazil</p>
      <p>Mailing address</p>
      <p>
        <bold>ABSTRACT</bold>
      </p>
      <p>The authors describe a case of Takayasu's arteritis in a child of only 3 years of age, emphasizing the rarity of this disease in this age group. The child was admitted to the emergency room in a post-convulsive state after a tonic-clonic seizure. After a detailed clinical examination, extensive diagnostic tests, and observation of the clinical evolution, the diagnosis was Takayasu's disease with severe cardiac and arterial involvement. The report warns pediatricians and cardiologists to awareness of the possibility of this disease in very small infants, in countries where it is underdiagnosed.</p>
      <p><bold>Key words:</bold> Takayasu arteritis/complications; vasculitis; child preschool; angiography/methods.</p>
      <p>
        <bold>Introduction</bold>
      </p>
      <p>Takayasu's arteritis (TA) is a chronic granulomatous vasculitis of unknown etiology involving the aorta and its branches, which produces vascular sequelae with stenotic lesions and/or thrombus formation. The lesions are usually severe, leading to organ dysfunction, and most require a surgical approach. It is underdiagnosed in Brazil probably due to diagnostic difficulty, but Brazilian studies estimate that half of the affected individuals are in the age group under 20 years<sup>1</sup>. The case described in this study stands out because of the extensive vascular involvement with severe sequelae in a very young child.</p>
      <p>
        <bold>Case report</bold>
      </p>
      <p>JEFS, 3 years and 7 months old, male, weighing 12 kg, was admitted to the emergency room in a post-convulsive state after a tonic-clonic seizure. The family reported that this was his first convulsive episode and denied fever, gastrointestinal or respiratory changes, and use of medication. The child was in attendance because of low weight gain. There was no family history of epilepsy, and the patient was the only child of a healthy couple. The patient had negative epidemiology for tuberculosis (TB) and a visible BCG vaccine scar.</p>
      <p>Clinical examination showed signs of malnutrition, light mucocutaneous pallor, absence of edema or jaundice, good general condition, no fever, and mild tachypnea. The cardiovascular examination showed lower deviation of the apex beat, slight muffling of the heart sounds, tachycardia, and no murmurs on auscultation of the chest, abdomen and back. BP ranged from 125/70 to 130/80 mmHg (percentile&gt; 95 for age, according to Task Force, 1996). The pulses were reduced in the lower limbs, which showed lower temperature than the upper limbs. The liver was located at 3 cm from the RCM, and there were no enlarged lymph nodes or abdominal masses.</p>
      <p>The blood count showed normal white blood cell count and platelet count, mild hypochromic anemia with microcytosis. CRP was negative; HHS was low; ANF was negative; rheumatoid factor was negative; blood cultures were negative. The ECG showed sinus rhythm, left ventricular hypertrophy, and ventricular repolarization changes. The X-ray of the chest showed enlarged heart area due to left ventricle (LV) hypertrophy, and signs of pulmonary congestion (<xref ref-type="fig" rid="f1-body3">Fig. 1</xref>). The computed tomography of the brain showed no changes. The Doppler echocardiogram showed LVEF = 38%, large increase in the left chambers, and the presence of large thrombi within the LV cavity (<xref ref-type="fig" rid="f1-body3">Fig. 1</xref>).</p>
      <fig id="f1-body3">
        <graphic xlink:href="1678-4170-abc-95-04-e101-00026-gf01-en.jpg"/></fig>
      <p>Abdominal ultrasound found a reduction in the volume of the right kidney and changes suggesting a cortical infarct area in its upper pole. Duplex scanning of carotid arteries was normal, and Doppler scanning of the abdominal aorta revealed an aortic aneurysm, with a thrombus within it, as well as poor visualization of iliac and renal vessels. The abdominal angiotomography showed diffuse concentric thickening of the proximal and suprarenal portions of the abdominal aorta, and fusiform aneurysms of the common iliac arteries (larger on the right side), with an intraluminal thrombus, and right renal hypotrophy (<xref ref-type="fig" rid="f2-body3">Fig. 2</xref>). The coronary cineangiography was not performed because of the risks associated with the placement of a vascular access device in the presence of large aneurysms of the iliac arteries.</p>
      <fig id="f2-body3">
        <graphic xlink:href="1678-4170-abc-95-04-e101-00026-gf02-en.jpg"/></fig>
      <p>
        <bold>Evolution</bold>
      </p>
      <p>An improvement of the CHF was observed with the use of furosemide, spironolactone, digoxin and carvedilol. The child received heparin and, afterwards, dicumarol; no intracavitary thrombus was visualized on the echocardiography, and there was no new thromboembolic event. The child underwent surgical resection of iliac artery aneurysms with stent placements in the right iliac artery and the abdominal aorta. Histopathological analysis of the resected vascular segment showed non-specific chronic periarteritis with areas of atherosclerosis.</p>
      <p>During hospitalization, the child presented with a lesion on the dorsum of the right foot, suggesting vasculitis, as well as fever, and increased HSS and CRP. The patient showed clinical and laboratorial improvement after pulse therapy with methylprednisolone. An echocardiogram performed after one year of treatment showed normalization of LV systolic function and reduction of left cardiac chambers. The thoracic and abdominal angiotomography was repeated and showed no impairment of the aortic arch or the innominate artery, but it showed little contrast in the iliac arteries and large collateral network (<xref ref-type="fig" rid="f2-body3">Fig. 2</xref>). Currently, the child has normal blood pressure levels, normal renal function, no CHF, and is gaining weight.</p>
      <p>
        <bold>Discussion</bold>
      </p>
      <p>AT was first described by the ophthalmologist Mikito Takayasu, in 1908; he observed aneurysms and arteriovenous anastomoses in the retina<sup>2</sup>. It is an inflammatory segmental chronic vasculitis, of a destructive/degenerative nature, with diffuse and multifocal extension, affecting large and medium arteries, particularly the thoracic aorta and its major branches, and also the pulmonary and coronary arteries<sup>2</sup>. In the late phase, there are areas of stenosis interspersed with dilatations and normal segments, as well as occlusions by thrombi and atypical coarctations<sup>1,2</sup>. These changes cause ischemia in different territories, especially the cerebral, coronary, peripheral arterial and renal territories. Studies suggest that immune and/or genetic factors are involved in the genesis of the disease<sup>3,4</sup>. AT is considered the third most common worldwide cause of childhood vasculitis. About three quarters of the patients are between 10 and 20 years of age, with a higher incidence in males (1:8)<sup>5</sup>.</p>
      <p>Usually, the disease presents three phases. The first phase results from an acute inflammatory process with nonspecific symptoms, with no pulse changes. The second phase reflects the exacerbation of vascular inflammation, manifested by pain in the vessel. These phases may last from months to years. The third stage is fibrotic, and the manifestations are secondary to arterial occlusions<sup>6-8</sup>. An association of TA with TB and with rheumatoid arthritis; Crohn's disease; lupus erythematosus; thyroiditis; ulcerative colitis; and ankylosing spondylitis is described by several authors<sup>4,9</sup>. In this case, no diagnostic workup was performed in relation to these diseases because of the absence of other signs and symptoms related to them.</p>
      <p>Angiography is the imaging method of choice<sup>5,10</sup>, traditionally used to evaluate the affected vessels, but it is a procedure of high risk due to exposure to radiation, rupture of vessels, hemorrhages, and infections. It was not indicated in this case because of the presence of giant aneurysms at sites of vascular access for the exam. For the low risk, we opted for a thoracic and abdominal angiotomography, which revealed no lesions in the aortic arch, in the brachiocephalic trunk, and in the entire thoracic aorta, but a bilateral reduced flow was observed in the iliac arteries, with formation of a collateral vascular network.</p>
      <p>The case-study met the diagnostic criteria developed by the American College of Rheumatology (ACR)<sup>8,9</sup> and was classified as type II according to the classification of Ueno et al<sup>8,9</sup>, modified by Lupi and Sanchez-Torres<sup>8,9</sup>. It included involvement of the thoracic aorta (descending and abdominal segments), with no involvement of the aortic arch<sup>8,9</sup>.</p>
      <p>The clinical management of TA consists of the control of vascular inflammation with the use of immunosuppressants, during the inflammation activity phase<sup>4,5</sup>. In the chronic phase, revascularization surgery is often necessary, with a high rate of reestenosis<sup>10</sup>. In the case described in this study, there was myocardial dysfunction that responded well to medical treatment and control of high blood pressure. CNS changes were probably due to the detachment of intracardiac thrombi, leading to stroke, resulting in hemiplegia.</p>
      <p>From the case presented in this study and from the literature review, we concluded that the awareness of this disease by pediatricians and cardiologists permits its diagnosis and proper treatment, thus reducing morbidity and mortality. The hypothesis of TA should be included in the differential diagnosis of cases of cardiomyopathy that occur concomitantly with high blood pressure, ischemic strokes, pulse asynchrony between limbs, and renovascular hypertension, even in very small infants. It is recommended that other diseases be investigated, especially tuberculosis, because of its high prevalence in our country.</p>
      <p>
        <bold>Potential Conflict of Interest</bold>
      </p>
      <p>No potential conflict of interest relevant to this article was reported.</p>
      <p>
        <bold>Sources of Funding</bold>
      </p>
      <p>There were no external funding sources for this study.</p>
      <p>
        <bold>Study Association</bold>
      </p>
      <p>This study is not associated with any post-graduation program.</p>
      <p>
        <bold>References</bold>
      </p>
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