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        "resumen" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A s&#237;ndroma de Ehlers-Danlos &#40;<span class="elsevierStyleItalic">cutis hyperelastica</span>&#41;&#44; constitui uma patologia do tecido conjuntivo caracterizada por altera&#231;&#245;es da pele&#44; ligamentos e &#243;rg&#227;os internos&#46;</p><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Apresenta transmiss&#227;o heredit&#225;ria&#44; em geral autoss&#243;mica dominante&#46; Afecta primordialmente a s&#237;ntese do colag&#233;neo&#44; pelo que a pele e os vasos sangu&#237;neos se tornam extremamente el&#225;sticos e fr&#225;geis&#46; A pele &#233; macia&#44; com consist&#234;ncia de borracha&#44; e as equimoses surgem facilmente&#46; As articula&#231;&#245;es s&#227;o hiperextens&#237;veis e t&#234;m mobilidade aumentada&#46;</p><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Os autores apresentam o caso cl&#237;nico de um doente de dezasseis anos&#44; com hist&#243;ria de hipermobilidade articular desde a inf&#226;ncia e fractura espl&#233;nica&#44; a quem foi feito o diagn&#243;stico de s&#237;ndroma de Ehlers-Danlos na sequ&#234;ncia de pneumot&#243;rax espont&#226;neo recidivante&#46;</p><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Apresentam-se as complica&#231;&#245;es pulmonares mais frequentes desta s&#237;ndroma e discute-se a import&#226;ncia de n&#227;o esquecer as etiologias menos comuns&#44; perante casos de pneumot&#243;rax espont&#226;neo&#46;</p>"
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        "resumen" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Ehlers-Danlos syndrome &#40;<span class="elsevierStyleItalic">cutis hyperelastica</span>&#41;&#44; is a group of connective tissue disorders characterized by abnormalities of the skin&#44; ligaments and internal organs&#46;</p><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">It is a hereditary syndrome&#44; usually with autossomal dominant inheritance&#59; that primarily affects the collagen synthesis&#46; The skin and blood vessels are extremely fragile and elastic&#46; The skin is soft with rubber consistency and easily bruising&#46; There are hypermobile joints with increased extensibility&#46;</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&#46;</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&#44; in cases of spontaneous pneumothorax&#46;</p>"
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Síndroma de Ehlers-Danlos – Uma causa rara de pneumotórax espontâneo
Ehlers-Danlos syndrome – A rare cause of spontaneous pneumothorax
Carlos Lopes1,
Corresponding author
carlos.lopes@hsm.min-saude.pt

Correspondência: Carlos Lopes Serviço de Pneumologia, Hospital de Santa Maria, Lisboa, Portugal.
, Alda Manique2, Renato Sotto-Mayor3, Jorge Cruz4, Margarida Mendes de Almeida5, João Cravino6, A. Bugalho de Almeida7, Afonso Fernandes8
1 Interno do Internato Complementar de Pneumologia
2 Assistente Hospitalar Graduada de Pneumologia
3 Chefe de Serviço de Pneumologia. Assistente Convidado da Faculdade de Medicina de Lisboa
4 Assistente Hospitalar Graduado de Cirurgia Cardiotorácica
5 Assistente Hospitalar Graduada de Anatomia Patológica
6 Director do Serviço de Cirurgia Cardiotorácica
7 Director do Serviço de Pneumologia. Professor Associado da Faculdade de Medicina de Lisboa
8 Serviço de Anatomia Patológica Hospital de Santa Maria, Lisboa
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Article information
ISSN: 21735115
Original language: Portuguese
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