Malformações arteriovenosas pulmonares – Associação a telangiectasia hemorrágica hereditária. Casos clínicos e rastreio familiar
Pulmonary arteriovenous malformations – Association with hereditary hemorrhagic telangiectasia. Clinical cases and family screening
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Casos clínicos e rastreio familiar" "tieneTextoCompleto" => 0 "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "383" "paginaFinal" => "399" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "Diva Ferreira, Joana Amado, Raquel Duarte, José Almeida, Paulo Morgado, Teresa Shiang" "autores" => array:6 [ 0 => array:4 [ "nombre" => "Diva" "apellidos" => "Ferreira" "email" => array:1 [ 0 => "divafferreira@sapo.pt" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">1</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">¿</span>" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "Joana" "apellidos" => "Amado" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">2</span>" "identificador" => "aff0010" ] ] ] 2 => array:3 [ "nombre" => "Raquel" "apellidos" => "Duarte" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">2</span>" "identificador" => "aff0010" ] ] ] 3 => array:3 [ "nombre" => "José" "apellidos" => "Almeida" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">2</span>" "identificador" => "aff0010" ] ] ] 4 => array:3 [ "nombre" => "Paulo" "apellidos" => "Morgado" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">3</span>" "identificador" => "aff0015" ] ] ] 5 => array:3 [ "nombre" => "Teresa" "apellidos" => "Shiang" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">4</span>" "identificador" => "aff0020" ] ] ] ] "afiliaciones" => array:4 [ 0 => array:3 [ "entidad" => "Interna complementar de Pneumologia" "etiqueta" => "<span class="elsevierStyleSup">1</span>" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Assistente Hospitalar de Pneumologia" "etiqueta" => "<span class="elsevierStyleSup">2</span>" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Assistente Hospitalar de Radiologia" "etiqueta" => "<span class="elsevierStyleSup">3</span>" "identificador" => "aff0015" ] 3 => array:3 [ "entidad" => "Assistente Graduada de Pneumologia Centro Hospitalar de Vila Nova de Gaia" "etiqueta" => "<span class="elsevierStyleSup">4</span>" "identificador" => "aff0020" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Correspondência: Diva de Fátima Gonçalves Ferreira, Rua Conceição Fernandes 4434-502 Vila Nova de Gaia." ] ] ] ] "titulosAlternativos" => array:1 [ "en" => array:1 [ "titulo" => "Pulmonary arteriovenous malformations – Association with hereditary hemorrhagic telangiectasia. Clinical cases and family screening" ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2006-03-21" "fechaAceptado" => "2006-05-12" "PalabrasClave" => array:2 [ "pt" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palavras-chave" "identificador" => "xpalclavsec158759" "palabras" => array:3 [ 0 => "Malformações arteriovenosas pulmonares" 1 => "telangiectasia hemorrágica hereditária" 2 => "embolização" ] ] ] "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Key-words" "identificador" => "xpalclavsec158758" "palabras" => array:3 [ 0 => "Pulmonary arteriovenous malformations" 1 => "hereditary hemorrhagic telangiectasia" 2 => "embolization" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "pt" => array:2 [ "titulo" => "Resumo" "resumen" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">As malformações arteriovenosas pulmonares são raras e mais de metade dos casos surgem em associação a telangiectasia hemorrágica hereditária.</p><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Faz-se uma revisão teórica sobre a apresentação clínica, abordagem diagnóstica, terapêutica e prognóstico destas malformações vasculares. Estão associadas a morbilidade e mortalidade consideráveis, pelo que se preconiza o seu tratamento, assim como o rastreio dos familiares directos quando se identificam malformações arteriovenosas pulmonares num doente com telangiectasia hemorrágica hereditária. Ainda não existem estudos prospectivos que estabeleçam o melhor plano de estudo do doente e seus familiares.</p><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Descrevem-se dois casos clínicos de malformações arteriovenosas pulmonares no contexto familiar de telangiectasia hemorrágica hereditária. A identificação das doentes implicou o rastreio familiar que permitiu detectar malformações arteriovenosas pulmonares em dois familiares e excluir o envolvimento pulmonar em quatro familiares com a doença.</p>" ] "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Pulmonary arteriovenous malformations are a rare disorder associated to hereditary hemorrhagic telangiectasia in over 50% of the cases.</p><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Clinical presentation, diagnostic work-up, therapeutic options and prognosis are reviewed by the authors. Pulmonary arteriovenous malformations are known to have considerable morbidity and mortality, their treatment being advisable as well as their screening among family members, especially if the index case is diagnosed with both pulmonary arteriovenous malformations and hereditary hemorrhagic telangiectasia. To this moment prospective studies establishing the best diagnostic work-up for the patients and their families are lacking.</p><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">The authors report two pulmonary arteriovenous malformations cases in a family with hereditary hemorrhagic telangiectasia. Patient's diagnosis led to family screening which resulted in the identification of pulmonary arteriovenous malformations in two family members and pulmonary disease exclusion in four patients previously known to have hereditary hemorrhagic telangiectasia.</p>" ] ] "bibliografia" => array:2 [ "titulo" => "Bibliografia" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:19 [ 0 => array:3 [ "identificador" => "bib1" "etiqueta" => "1." 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ISSN: 21735115Original language: Portuguese
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