Artigo de Revisão\Revision Article
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
Diva Ferreira1,
, Joana Amado2, Raquel Duarte2, José Almeida2, Paulo Morgado3, Teresa Shiang4
Corresponding author
divafferreira@sapo.pt
Correspondência: Diva de Fátima Gonçalves Ferreira, Rua Conceição Fernandes 4434-502 Vila Nova de Gaia.
Correspondência: Diva de Fátima Gonçalves Ferreira, Rua Conceição Fernandes 4434-502 Vila Nova de Gaia.
1 Interna complementar de Pneumologia, Centro Hospitalar de Vila Nova de Gaia
2 Assistente Hospitalar de Pneumologia, Centro Hospitalar de Vila Nova de Gaia
3 Assistente Hospitalar de Radiologia, Centro Hospitalar de Vila Nova de Gaia
4 Assistente Graduada de Pneumologia, Centro Hospitalar de Vila Nova de Gaia
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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" => "xpalclavsec567767" "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" => "xpalclavsec567766" "palabras" => array:3 [ 0 => "Pulmonary arteriovenous malformations" 1 => "hereditary hemorrhagic telangiectasia" 2 => "embolization" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "pt" => array:2 [ "titulo" => "Resumo" "resumen" => "<span id="as0005" class="elsevierStyleSection elsevierViewall"><p id="sp0005" 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="sp0110" 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="sp0010" 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><p id="sp0015" class="elsevierStyleSimplePara elsevierViewall"><span class="elsevierStyleBold">Rev Port Pneumol 2006; XII (4): 383-399</span></p></span>" ] "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<span id="as0010" class="elsevierStyleSection elsevierViewall"><p id="sp0020" class="elsevierStyleSimplePara elsevierViewall">Pulmonary arteriovenous malformations are a rare disorder associated to hereditary hemorrhagic telangiectasia in over<span class="elsevierStyleHsp" style=""></span>50 % of the cases.</p><p id="sp0025" 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="sp0030" 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><p id="sp0035" class="elsevierStyleSimplePara elsevierViewall"><span class="elsevierStyleBold">Rev Port Pneumol 2006; XII (4): 383-399</span></p></span>" ] ] "lecturaRecomendada" => array:1 [ 0 => array:3 [ "vista" => "all" "titulo" => "<span class="elsevierStyleSectionTitle" id="st0025">Bibliografia</span>" "seccion" => array:1 [ 0 => array:2 [ "vista" => "all" "bibliografiaReferencia" => array:19 [ 0 => array:3 [ "identificador" => "bb0005" "etiqueta" => "1." "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Pulmonary arteriovenous malformations: A state of the art review" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "J.R. Gossage" 1 => "G. 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Article information
ISSN: 08732159Original language: Portuguese
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