Estatísticas

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          "autoresLista" => "João Monteiro de Pina-Neto"
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    "cita" => "J Pediatr (Rio J). 2008;84 Supl 1:2-7"
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          "autoresLista" => "Ricardo Timm de Souza, José Roberto Goldim"
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    "titulo" => "Tratamento de erros inatos do metabolismo"
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          <html><span id="abst0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Objective</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">To describe the current state of treatment for disorders of intermediate metabolism (primarily of amino acids, urea cycle and organic acids) and for diseases related to two subcellular organelles (lysosomes and peroxisomes).</p>\n
          \t\t\t\t\t\t   </span><span id="abst0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Sources</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">In covering the treatment of disorders of intermediate metabolism, priority was given to the most important methods for managing intoxication, in view of the importance for pediatricians to treat acute and life-threatening cases. The article also provides a general overview of the treatment for lysosomal and peroxisomal diseases, with emphasis on enzyme replacement therapy, which is a treatment modality that is growing in use and with which pediatricians should make themselves familiar.</p>\n
          \t\t\t\t\t\t   </span><span id="abst0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Summary of the findings</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">The most important measures used to manage the intoxication present in many inborn errors of intermediate metabolism were presented (restriction of substrate build-up by means of diet or enzymatic inhibition, removal of toxic products, stimulation of residual enzyme activity, replacement of the deficient product). The section on treatment for lysosomal and peroxisomal diseases includes tables providing information on the treatments available.</p>\n
          \t\t\t\t\t\t   </span><span id="abst0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Conclusions</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Treating inborn errors of metabolism is a complex task that should be performed by a multidisciplinary team of which the pediatrician is the key member. This article provides practical information relating to the management of some inborn errors of metabolism and provides pediatricians with a general overview of recent developments in this area of medicine.</p>\n
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        "resumen" => "<html><span id="abst0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Objetivo</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Esta revis&atilde;o teve por objetivo abordar a situa&ccedil;&atilde;o atual do tratamento dos dist&uacute;rbios do metabolismo intermedi&aacute;rio (principalmente dos amino&aacute;cidos, ciclo da ur&eacute;ia e &aacute;cidos org&acirc;nicos) e das doen&ccedil;as relacionadas a duas organelas subcelulares (lisossomos e peroxissomos).</p></span><span id="abst0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Fontes dos dados</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Na abordagem do tratamento dos dist&uacute;rbios do metabolismo intermedi&aacute;rio, foi dada prioridade &agrave;s principais formas de manejo da intoxica&ccedil;&atilde;o, em virtude da import&acirc;ncia para o pediatra do tratamento de quadros agudos e com risco de vida. O artigo apresenta tamb&eacute;m uma vis&atilde;o geral do tratamento das doen&ccedil;as lisoss&ocirc;micas e peroxiss&ocirc;micas, com &ecirc;nfase na terapia de reposi&ccedil;&atilde;o enzim&aacute;tica, uma modalidade de tratamento de uso crescente com a qual o pediatra deve se familiarizar.</p></span><span id="abst0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">S&iacute;ntese dos dados</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">As principais medidas para manejo da intoxica&ccedil;&atilde;o presente em muitos erros inatos do metabolismo intermedi&aacute;rio foram apresentadas (restri&ccedil;&atilde;o de aporte de substrato atrav&eacute;s da dieta ou atrav&eacute;s de inibi&ccedil;&atilde;o enzim&aacute;tica, remo&ccedil;&atilde;o do produto t&oacute;xico, est&iacute;mulo da atividade enzim&aacute;tica residual, reposi&ccedil;&atilde;o do produto deficiente). O material elaborado sobre terapia para doen&ccedil;as lisoss&ocirc;micas e peroxiss&ocirc;micas inclui tabelas informativas sobre os tratamentos dispon&iacute;veis.</p></span><span id="abst0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Conclus&otilde;es</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">O tratamento dos erros inatos do metabolismo &eacute; uma situa&ccedil;&atilde;o complexa e que deve ser abordada por uma equipe multidisciplinar, na qual o pediatra &eacute; pe&ccedil;a-chave. Este cap&iacute;tulo cont&eacute;m informa&ccedil;&otilde;es pr&aacute;ticas relativas ao manejo de alguns erros inatos do metabolismo e proporciona ao pediatra uma vis&atilde;o geral dos desenvolvimentos recentes ocorrido nessa &aacute;rea da medicina.</p></span></html>"
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