Caso Clínico\Clinical Case
Síndroma de Ehlers-Danlos – Uma causa rara de pneumotórax espontâneo
Ehlers-Danlos syndrome – A rare cause of spontaneous pneumothorax
Carlos Lopes1,
, Alda Manique2, Renato Sotto-Mayor3, Jorge Cruz4, Margarida Mendes de Almeida5, João Cravino6, A Bugalho de Almeida7
Corresponding author
carlos.lopes@hsm.min-saude.pt
Correspondência: Carlos Lopes, Serviço de Pneumologia, Hospital de Santa Maria, Lisboa, Portugal
Correspondência: Carlos Lopes, Serviço de Pneumologia, Hospital de Santa Maria, Lisboa, Portugal
1 Interno do Internato Complementar de Pneumologia, Serviço 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, Serviço de Cirurgia Cardiotorácica
7 Director do Serviço de Pneumologia. Professor Associado da Faculdade de Medicina de Lisboa
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Assistente Convidado da Faculdade de Medicina de Lisboa" "etiqueta" => "3" "identificador" => "af0015" ] 3 => array:3 [ "entidad" => "Assistente Hospitalar Graduado de Cirurgia Cardiotorácica" "etiqueta" => "4" "identificador" => "af0020" ] 4 => array:3 [ "entidad" => "Assistente Hospitalar Graduada de Anatomia Patológica" "etiqueta" => "5" "identificador" => "af0025" ] 5 => array:3 [ "entidad" => "Director do Serviço de Cirurgia Cardiotorácica, Serviço de Cirurgia Cardiotorácica" "etiqueta" => "6" "identificador" => "af0030" ] 6 => array:3 [ "entidad" => "Director do Serviço de Pneumologia. Professor Associado da Faculdade de Medicina de Lisboa" "etiqueta" => "7" "identificador" => "af0035" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cr0005" "etiqueta" => "*" "correspondencia" => "<span class="elsevierStyleBold">Correspondência:</span> Carlos Lopes, Serviço de Pneumologia, Hospital de Santa Maria, Lisboa, Portugal" ] ] ] ] "titulosAlternativos" => array:1 [ "en" => array:1 [ "titulo" => "Ehlers-Danlos syndrome – A rare cause of spontaneous pneumothorax" ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2005-11-07" "fechaAceptado" => "2006-05-18" "PalabrasClave" => array:2 [ "pt" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palavras-chave" "identificador" => "xpalclavsec567777" "palabras" => array:3 [ 0 => "Síndroma de Ehlers-Danlos" 1 => "pneumotórax" 2 => "hipermobilidade articular" ] ] ] "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Key-words" "identificador" => "xpalclavsec567776" "palabras" => array:3 [ 0 => "Ehlers-Danlos Syndrome" 1 => "pneumothorax" 2 => "joint hypermobility" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "pt" => array:2 [ "titulo" => "Resumo" "resumen" => "<span id="as0005" class="elsevierStyleSection elsevierViewall"><p id="sp0005" class="elsevierStyleSimplePara elsevierViewall">A síndroma de Ehlers-Danlos (<span class="elsevierStyleItalic">cutis hyperelastica</span>), constitui uma patologia do tecido conjuntivo caracterizada por alterações da pele, ligamentos e órgãos internos.</p><p id="sp0105" class="elsevierStyleSimplePara elsevierViewall">Apresenta transmissão hereditária, em geral autossómica dominante. 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="sp0010" 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="sp0015" 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><p id="sp0020" class="elsevierStyleSimplePara elsevierViewall"><span class="elsevierStyleBold">Rev Port Pneumol 2006; XII (4): 471-480</span></p></span>" ] "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<span id="as0010" class="elsevierStyleSection elsevierViewall"><p id="sp0025" 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="sp0030" 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="sp0035" 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="sp0040" 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><p id="sp0045" class="elsevierStyleSimplePara elsevierViewall"><span class="elsevierStyleBold">Rev Port Pneumol 2006; XII (4): 471-480</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:11 [ 0 => array:3 [ "identificador" => "bb0005" "etiqueta" => "1." 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"referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Inherited Disorders of Conective Tissue" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "D.J. Prockop" 1 => "H. Kuivaniemi" 2 => "G. Tromp" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "LibroEditado" => array:5 [ "titulo" => "Harrison’s Principles of Internal Medicine" "paginaInicial" => "2189" "paginaFinal" => "2191" "edicion" => "14th ed" "serieFecha" => "1998" ] ] ] ] ] ] 5 => array:3 [ "identificador" => "bb0030" "etiqueta" => "6." "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Ehlers-Danlos Syndrome" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:1 [ 0 => "R.E. 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Walters Jr." ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:6 [ "tituloSerie" => "Applied Radiology" "fecha" => "2003" "volumen" => "32" "numero" => "5" "paginaInicial" => "48" "paginaFinal" => "50" ] ] ] ] ] ] 8 => array:3 [ "identificador" => "bb0045" "etiqueta" => "9." "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "The Skin" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "G.L. Darmstadt" 1 => "R. Sidbury" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "LibroEditado" => array:5 [ "titulo" => "Nelson Textbook of Pediatrics" "paginaInicial" => "2207" "paginaFinal" => "2208" "edicion" => "17th ed" "serieFecha" => "2004" ] ] ] ] ] ] 9 => array:3 [ "identificador" => "bb0050" "etiqueta" => "10." 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| 2016 July | 4 | 2 | 6 |
| 2016 May | 0 | 2 | 2 |
| 2016 April | 6 | 12 | 18 |
| 2016 March | 4 | 10 | 14 |
| 2016 February | 8 | 9 | 17 |
| 2016 January | 4 | 10 | 14 |
| 2015 December | 5 | 1 | 6 |
| 2015 November | 2 | 2 | 4 |
| 2015 October | 3 | 2 | 5 |
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