<?xml version="1.0" encoding="ISO-8859-1"?><article xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance">
<front>
<journal-meta>
<journal-id>1688-1249</journal-id>
<journal-title><![CDATA[Archivos de Pediatría del Uruguay]]></journal-title>
<abbrev-journal-title><![CDATA[Arch. Pediatr. Urug.]]></abbrev-journal-title>
<issn>1688-1249</issn>
<publisher>
<publisher-name><![CDATA[Sociedad Uruguaya de Pediatría]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S1688-12492018000200099</article-id>
<article-id pub-id-type="doi">10.31134/ap.89.2.4</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Dermatomiositis juvenil: experiencia de 13 años en un hospital de atención terciaria. Análisis de 17 casos clínicos]]></article-title>
<article-title xml:lang="en"><![CDATA[Juvenile dermatomyositis: 13 years of experience in a tertiary care hospital. Analysis of 17 clinical cases]]></article-title>
<article-title xml:lang="pt"><![CDATA[Dermatomiosite juvenil: 13 anos de experiência em hospital de nível terciário de atenção. Analise de 17 casos clinicos]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Caggiani]]></surname>
<given-names><![CDATA[Marina]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Puente]]></surname>
<given-names><![CDATA[Lucía de la]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Guariglia]]></surname>
<given-names><![CDATA[Raquel]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Jurado]]></surname>
<given-names><![CDATA[Rosario]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Cameto]]></surname>
<given-names><![CDATA[Juan]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Suárez]]></surname>
<given-names><![CDATA[Rodrigo]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,UDELAR Facultad de Medicina Pediatría]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Uruguay</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,CHPR Policlínica Reumatología y Colagenopatías ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
</aff>
<aff id="Af3">
<institution><![CDATA[,CHPR  ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
</aff>
<aff id="Af4">
<institution><![CDATA[,CHPR Policlínica Reumatología ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
</aff>
<aff id="Af5">
<institution><![CDATA[,CHPR Policlínica Colagenopatías ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
</aff>
<aff id="Af6">
<institution><![CDATA[,CHPR Policlínica Reumatología ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>04</month>
<year>2018</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>04</month>
<year>2018</year>
</pub-date>
<volume>89</volume>
<numero>2</numero>
<fpage>99</fpage>
<lpage>107</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.edu.uy/scielo.php?script=sci_arttext&amp;pid=S1688-12492018000200099&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.edu.uy/scielo.php?script=sci_abstract&amp;pid=S1688-12492018000200099&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.edu.uy/scielo.php?script=sci_pdf&amp;pid=S1688-12492018000200099&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[Resumen: La dermatomiositis juvenil (DMJ) es una miopatía inflamatoria adquirida de base inmunológica acompañada por alteraciones cutáneas características. El objetivo de este artículo es describir las características clínicas y exámenes complementarios de un grupo de 17 pacientes con diagnóstico de DMJ, su evolución y tratamiento.  Material y método:  se estudiaron los pacientes que se asistieron en la Policlínica de enfermedades de tejido conectivo del Centro Hospitalario Pereira Rossell (CHPR) en el período del 1 de octubre de 2003 al 1 de abril de 2017.  Resultados:  los rasgos clínicos más frecuentes de presentación fueron las manifestaciones cutáneas características, debilidad muscular, síntomas constitucionales, manifestaciones gastrointestinales y respiratorias. Las enzimas musculares estuvieron aumentadas en todos los casos. La resonancia nuclear magnética, electromiograma y la biopsia muscular fueron patológicos en todos los casos realizados. El tratamiento se realizó fundamentalmente en base a corticoides y fármacos inmunosupresores, siendo el metotrexate la droga de elección. En los casos graves o refractarios se asoció gamaglobulina, ciclofosfamida o ciclosporina. La duración del tratamiento tuvo una mediana de 3 años 10 meses. Se logró remisión en 47% de los pacientes. La evolución fue monofásica en 15,4%, de los casos, polifásica en 8% y crónica en 77%. No hubo fallecimientos registrados, ni enfermedad maligna asociada.  Conclusiones:  la DMJ es una enfermedad de baja incidencia. La mayoría de los pacientes tuvieron una evolución crónica. Esto determina la necesidad de un tratamiento inmunosupresor prolongado con los efectos adversos de la misma. Se logró la remisión en 47% de los pacientes. No se registraron fallecimientos en la serie estudiada.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[Summary: Juvenile dermatomyositis (JDM) is an acquired inflammatory myopathy with an immunologic basis and characteristic cutaneous rash. The aim of this article is to describe clinical features and most important exams of a group of 17 patients with JDM their evolution and treatment.  Methods:  children with JDM recruited from Connective tissue Diseases Office of Pereira Rossell Hospital from 1/10/2003 through 1/4/2017 were studied.  Results: the most frequent features were: characteristic cutaneous rash, muscle weakness, systemic symptoms, gastrointestinal and respiratory manifestations. The diagnostic investigations showed an increase serum muscle enzymes in all patients. The nuclear magnetic resonance, electromyogram and muscle biopsy resulted abnormal in all the investigated cases. Treatment was based on corticosteroids and immunosuppressive drugs being methotrexate the preferred drug. In severe or refractory cases cyclophosphamide, human gammaglobulin or cyclosporine were associated. Median treatment length was 3 years 10 months Remission was achieved in 47 percent. The evolution was monophasic in 15.4 percent, polyphasic in 7.7 and chronic in 77 percent. No deaths were registered neither malignant associated diseases  Conclusions: JDM is an infrequent illness. Most of the patients had chronic evolution. This obliges to prolonged immunosuppression with its adverse effects. Remission was achieved in 47% of the cases. No deaths were registered in this population.]]></p></abstract>
<abstract abstract-type="short" xml:lang="pt"><p><![CDATA[Resumo: A dermatomiosite juvenil (DMJ) é uma miopatia inflamatória imunológica adquirida acompanhada de alterações cutâneas características. O objetivo deste artigo é descrever as características clínicas e os exames complementares de um grupo de 17 pacientes diagnosticados com DMJ, sua evolução e tratamento.  Materiais e métodos:  foram estudados os pacientes que compareceram à Policlínica das Doenças do Tecido Conjuntivo no Centro Hospitalar Pereira Rossell (CHPR) no período de 01/10/2003 a 04/01/2017.  Resultados:  as características clínicas mais frequentes foram manifestações cutâneas características, fraqueza muscular, sintomas constitucionais, manifestações gastrointestinais e respiratórias. As enzimas musculares estiveram aumentadas em todos os casos. A Ressonância nuclear magnética, o eletromiograma e a biópsia muscular foram patológicos em todos os casos. O tratamento foi baseado principalmente em corticosteroides e drogas imunossupressoras, e o metotrexato foi a droga de escolha. Em casos graves ou refratários, também se administrou gamaglobulina, ciclofosfamida ou ciclosporina. A duração do tratamento teve uma mediana de 3 anos e 10 meses. A remissão foi alcançada em 47% dos pacientes. A evolução foi monofásica em 15,4% dos casos, polifásica em 8% e crônica em 77%. Não houve mortes registradas, nem doença maligna associada.  Conclusões: a DMJ é uma doença de baixa incidência. A maioria dos pacientes teve evolução crônica. Isso determina a necessidade de um tratamento imunossupressor prolongado com os seus conseguintes efeitos adversos. A remissão foi alcançada em 47% dos pacientes. Nenhuma morte foi registrada na série estudada.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[Dermatomiositis]]></kwd>
<kwd lng="es"><![CDATA[Niños]]></kwd>
<kwd lng="es"><![CDATA[Signos y síntomas]]></kwd>
<kwd lng="es"><![CDATA[Evolución clínica]]></kwd>
<kwd lng="en"><![CDATA[Dermatomyositis]]></kwd>
<kwd lng="en"><![CDATA[Children]]></kwd>
<kwd lng="en"><![CDATA[Signs and symptoms]]></kwd>
<kwd lng="en"><![CDATA[Clinical evolution]]></kwd>
<kwd lng="pt"><![CDATA[Dermatomiosite]]></kwd>
<kwd lng="pt"><![CDATA[Criança]]></kwd>
<kwd lng="pt"><![CDATA[Signos e sintomas]]></kwd>
<kwd lng="pt"><![CDATA[Evoluçao clinica]]></kwd>
</kwd-group>
</article-meta>
</front><back>
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