Correspondência: Carlos Lopes Serviço de Pneumologia, Hospital de Santa Maria, Lisboa, Portugal.
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Afecta primordialmente a síntese do colagéneo, pelo que a pele e os vasos sanguíneos se tornam extremamente elásticos e frágeis. A pele é macia, com consistência de borracha, e as equimoses surgem facilmente. As articulações são hiperextensíveis e têm mobilidade aumentada.</p><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Os autores apresentam o caso clínico de um doente de dezasseis anos, com história de hipermobilidade articular desde a infância e fractura esplénica, a quem foi feito o diagnóstico de síndroma de Ehlers-Danlos na sequência de pneumotórax espontâneo recidivante.</p><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Apresentam-se as complicações pulmonares mais frequentes desta síndroma e discute-se a importância de não esquecer as etiologias menos comuns, perante casos de pneumotórax espontâneo.</p>" ] "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Ehlers-Danlos syndrome (<span class="elsevierStyleItalic">cutis hyperelastica</span>), is a group of connective tissue disorders characterized by abnormalities of the skin, ligaments and internal organs.</p><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">It is a hereditary syndrome, usually with autossomal dominant inheritance; that primarily affects the collagen synthesis. The skin and blood vessels are extremely fragile and elastic. The skin is soft with rubber consistency and easily bruising. There are hypermobile joints with increased extensibility.</p><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">We summarize the case of a sixteen year old boy with a history of joint hypermobility since childhood and splenic fracture that was diagnosed with Ehlers-Danlos syndrome after the occurrence of recidivant spontaneous pneumothorax.</p><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">We present the most common pulmonary complications of Ehlers-Danlos syndrome and discuss the importance of not forgetting the least commons etiologies of pneumothorax, in cases of spontaneous pneumothorax.</p>" ] ] "bibliografia" => array:2 [ "titulo" => "Bibliografia" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:11 [ 0 => array:3 [ "identificador" => "bib1" "etiqueta" => "1." 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