quilotórax recorrente – uma história com 15 anos

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ID: 129082
2004
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Abstract
RESUMO: Os autores descrevem o caso de uma adolescente de 15 anos com internamentos sucessivos por quilotórax bilateral desde os primeiros meses de vida. A clínica e as imagens da tomografia axial computorizada estabeleceram o diagnóstico de linfangiectasia pulmonar. A maioria dos casos de linfangiectasia pulmonar, descritos na literatura, apresenta muito mau prognóstico, sendo geralmente letal no período neonatal. Neste caso, apesar dos múltiplos internamentos hospitalares, tem-se verificado razoável qualidade de vida entre as exacerbações. Para além desta manifestação clínica, encontramos linfedema congénito, alterações faciais (face inexpressiva, edemas palpebrais, boca pequena, alterações dentárias) e atraso ligeiro de desenvolvimento. Esta constelação de manifestações permite-nos encará-lo como o primeiro caso português de síndroma de Hennekam. O recurso a estudos moleculares poderá ajudar a explicar muitas das manifestações clínicas encontradas nestes doentes e a englobar outros casos na mesma entidade clínica.REV PORT PNEUMOL 2004; X (3): 253-258 ABSTRACT: The authors describe the clinical case of a 15 year-old adolescent with recurrent bilateral chylothorax leading to multiple hospitalizations. As in other cases described the diagnosis was made on the basis of classical clinical features and characteristic CT scan. Most cases of pulmonary lymphangiectasis, described in the literature, are lethal in the neonatal period. In this case the girl has a reasonable life quality, in spite of multiple hospitalizations. Lower limb lymphedema, facial anomalies (flat face, palpebral edema, tooth anomalies and small mouth) and mild development retardation allow us to consider our case as the first portuguese case of Hennekam Syndrome. Molecular studies will allow, in the future to explain most of the clinical manifestations and to include other patients in the same clinical entity.REV PORT PNEUMOL 2004; X (3): 253-258 Palavras-chave: quilotórax, linfangiectasia pulmonar, síndroma de Hennekam, Key-words: chylothorax, pulmonary lymphangiectasis, Hennekam syndrome
Reference Key
guardiano2004revistaquilotrax Use this key to autocite in the manuscript while using SciMatic Manuscript Manager or Thesis Manager
Authors ;Micaela Guardiano;Ana Lobo;Teresa Nunes;Luísa Vaz
Journal the journal of applied psychology
Year 2004
DOI
DOI not found
URL
http://www.sciencedirect.com/science/article/pii/S0873215915305808
Keywords
diseases of the respiratory system

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