array:23 [
  "pii" => "S2173511506704158"
  "issn" => "21735115"
  "doi" => "10.1016/S2173-5115(06)70415-8"
  "estado" => "S300"
  "fechaPublicacion" => "2006-07-01"
  "aid" => "70415"
  "copyright" => "Sociedade Portuguesa de Pneumologia"
  "copyrightAnyo" => "2006"
  "documento" => "article"
  "crossmark" => 0
  "licencia" => "http://www.elsevier.com/open-access/userlicense/1.0/"
  "subdocumento" => "fla"
  "cita" => "Rev Port Pneumol. 2006;12:471-80"
  "abierto" => array:3 [
    "ES" => true
    "ES2" => true
    "LATM" => true
  ]
  "gratuito" => true
  "lecturas" => array:2 [
    "total" => 4328
    "formatos" => array:3 [
      "EPUB" => 231
      "HTML" => 2367
      "PDF" => 1730
    ]
  ]
  "itemAnterior" => array:19 [
    "pii" => "S2173511506704146"
    "issn" => "21735115"
    "doi" => "10.1016/S2173-5115(06)70414-6"
    "estado" => "S300"
    "fechaPublicacion" => "2006-07-01"
    "aid" => "70414"
    "copyright" => "Sociedade Portuguesa de Pneumologia"
    "documento" => "article"
    "crossmark" => 0
    "licencia" => "http://www.elsevier.com/open-access/userlicense/1.0/"
    "subdocumento" => "fla"
    "cita" => "Rev Port Pneumol. 2006;12:463-70"
    "abierto" => array:3 [
      "ES" => true
      "ES2" => true
      "LATM" => true
    ]
    "gratuito" => true
    "lecturas" => array:2 [
      "total" => 3670
      "formatos" => array:3 [
        "EPUB" => 252
        "HTML" => 2003
        "PDF" => 1415
      ]
    ]
    "pt" => array:10 [
      "idiomaDefecto" => true
      "titulo" => "Tumor de células granulares endobrônquico – Como abordamos?"
      "tienePdf" => "pt"
      "tieneTextoCompleto" => 0
      "tieneResumen" => array:2 [
        0 => "pt"
        1 => "en"
      ]
      "paginas" => array:1 [
        0 => array:2 [
          "paginaInicial" => "463"
          "paginaFinal" => "470"
        ]
      ]
      "titulosAlternativos" => array:1 [
        "en" => array:1 [
          "titulo" => "Endobronchial granular cell tumor – What approach to take?"
        ]
      ]
      "contieneResumen" => array:2 [
        "pt" => true
        "en" => true
      ]
      "contienePdf" => array:1 [
        "pt" => true
      ]
      "autores" => array:1 [
        0 => array:2 [
          "autoresLista" => "Ana Rego, Joana Amado, Idália Esteves, José Almeida, Antónia Furtado, António Couceiro, João Moura e Sá, Raul César Sá"
          "autores" => array:9 [
            0 => array:2 [
              "nombre" => "Ana"
              "apellidos" => "Rego"
            ]
            1 => array:2 [
              "nombre" => "Joana"
              "apellidos" => "Amado"
            ]
            2 => array:2 [
              "nombre" => "Idália"
              "apellidos" => "Esteves"
            ]
            3 => array:2 [
              "nombre" => "José"
              "apellidos" => "Almeida"
            ]
            4 => array:2 [
              "nombre" => "Antónia"
              "apellidos" => "Furtado"
            ]
            5 => array:2 [
              "nombre" => "António"
              "apellidos" => "Couceiro"
            ]
            6 => array:2 [
              "nombre" => "João"
              "apellidos" => "Moura e Sá"
            ]
            7 => array:3 [
              "preGrado" => "Dr."
              "nombre" => "Raul César"
              "apellidos" => "Sá"
            ]
            8 => array:3 [
              "preGrado" => "Dr."
              "nombre" => "João"
              "apellidos" => "Moura e Sá"
            ]
          ]
        ]
      ]
    ]
    "idiomaDefecto" => "pt"
    "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2173511506704146?idApp=UINPBA00004E"
    "url" => "/21735115/0000001200000004/v1_201305151425/S2173511506704146/v1_201305151425/pt/main.assets"
  ]
  "pt" => array:14 [
    "idiomaDefecto" => true
    "titulo" => "Síndroma de Ehlers-Danlos – Uma causa rara de pneumotórax espontâneo"
    "tieneTextoCompleto" => 0
    "paginas" => array:1 [
      0 => array:2 [
        "paginaInicial" => "471"
        "paginaFinal" => "480"
      ]
    ]
    "autores" => array:1 [
      0 => array:4 [
        "autoresLista" => "Carlos Lopes, Alda Manique, Renato Sotto-Mayor, Jorge Cruz, Margarida Mendes de Almeida, João Cravino, A. Bugalho de Almeida, Afonso Fernandes"
        "autores" => array:8 [
          0 => array:4 [
            "nombre" => "Carlos"
            "apellidos" => "Lopes"
            "email" => array:1 [
              0 => "carlos.lopes@hsm.min-saude.pt"
            ]
            "referencia" => array:2 [
              0 => array:2 [
                "etiqueta" => "<span class="elsevierStyleSup">1</span>"
                "identificador" => "aff0005"
              ]
              1 => array:2 [
                "etiqueta" => "<span class="elsevierStyleSup">¿</span>"
                "identificador" => "cor0005"
              ]
            ]
          ]
          1 => array:3 [
            "nombre" => "Alda"
            "apellidos" => "Manique"
            "referencia" => array:1 [
              0 => array:2 [
                "etiqueta" => "<span class="elsevierStyleSup">2</span>"
                "identificador" => "aff0010"
              ]
            ]
          ]
          2 => array:3 [
            "nombre" => "Renato"
            "apellidos" => "Sotto-Mayor"
            "referencia" => array:1 [
              0 => array:2 [
                "etiqueta" => "<span class="elsevierStyleSup">3</span>"
                "identificador" => "aff0015"
              ]
            ]
          ]
          3 => array:3 [
            "nombre" => "Jorge"
            "apellidos" => "Cruz"
            "referencia" => array:1 [
              0 => array:2 [
                "etiqueta" => "<span class="elsevierStyleSup">4</span>"
                "identificador" => "aff0020"
              ]
            ]
          ]
          4 => array:3 [
            "nombre" => "Margarida"
            "apellidos" => "Mendes de Almeida"
            "referencia" => array:1 [
              0 => array:2 [
                "etiqueta" => "<span class="elsevierStyleSup">5</span>"
                "identificador" => "aff0025"
              ]
            ]
          ]
          5 => array:3 [
            "nombre" => "Jo&#227;o"
            "apellidos" => "Cravino"
            "referencia" => array:1 [
              0 => array:2 [
                "etiqueta" => "<span class="elsevierStyleSup">6</span>"
                "identificador" => "aff0030"
              ]
            ]
          ]
          6 => array:3 [
            "nombre" => "A&#46;"
            "apellidos" => "Bugalho de Almeida"
            "referencia" => array:1 [
              0 => array:2 [
                "etiqueta" => "<span class="elsevierStyleSup">7</span>"
                "identificador" => "aff0035"
              ]
            ]
          ]
          7 => array:3 [
            "nombre" => "Afonso"
            "apellidos" => "Fernandes"
            "referencia" => array:1 [
              0 => array:2 [
                "etiqueta" => "<span class="elsevierStyleSup">8</span>"
                "identificador" => "aff0040"
              ]
            ]
          ]
        ]
        "afiliaciones" => array:8 [
          0 => array:3 [
            "entidad" => "Interno do Internato Complementar de Pneumologia"
            "etiqueta" => "<span class="elsevierStyleSup">1</span>"
            "identificador" => "aff0005"
          ]
          1 => array:3 [
            "entidad" => "Assistente Hospitalar Graduada de Pneumologia"
            "etiqueta" => "<span class="elsevierStyleSup">2</span>"
            "identificador" => "aff0010"
          ]
          2 => array:3 [
            "entidad" => "Chefe de Servi&#231;o de Pneumologia&#46; Assistente Convidado da Faculdade de Medicina de Lisboa"
            "etiqueta" => "<span class="elsevierStyleSup">3</span>"
            "identificador" => "aff0015"
          ]
          3 => array:3 [
            "entidad" => "Assistente Hospitalar Graduado de Cirurgia Cardiotor&#225;cica"
            "etiqueta" => "<span class="elsevierStyleSup">4</span>"
            "identificador" => "aff0020"
          ]
          4 => array:3 [
            "entidad" => "Assistente Hospitalar Graduada de Anatomia Patol&#243;gica"
            "etiqueta" => "<span class="elsevierStyleSup">5</span>"
            "identificador" => "aff0025"
          ]
          5 => array:3 [
            "entidad" => "Director do Servi&#231;o de Cirurgia Cardiotor&#225;cica"
            "etiqueta" => "<span class="elsevierStyleSup">6</span>"
            "identificador" => "aff0030"
          ]
          6 => array:3 [
            "entidad" => "Director do Servi&#231;o de Pneumologia&#46; Professor Associado da Faculdade de Medicina de Lisboa"
            "etiqueta" => "<span class="elsevierStyleSup">7</span>"
            "identificador" => "aff0035"
          ]
          7 => array:3 [
            "entidad" => "Servi&#231;o de Anatomia Patol&#243;gica Hospital de Santa Maria&#44; Lisboa"
            "etiqueta" => "<span class="elsevierStyleSup">8</span>"
            "identificador" => "aff0040"
          ]
        ]
        "correspondencia" => array:1 [
          0 => array:3 [
            "identificador" => "cor0005"
            "etiqueta" => "&#8270;"
            "correspondencia" => "Correspond&#234;ncia&#58; Carlos Lopes Servi&#231;o de Pneumologia&#44; Hospital de Santa Maria&#44; Lisboa&#44; Portugal&#46;"
          ]
        ]
      ]
    ]
    "titulosAlternativos" => array:1 [
      "en" => array:1 [
        "titulo" => "Ehlers-Danlos syndrome &#8211; 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" => "xpalclavsec158801"
          "palabras" => array:3 [
            0 => "S&#237;ndroma de Ehlers-Danlos"
            1 => "pneumot&#243;rax"
            2 => "hipermobilidade articular"
          ]
        ]
      ]
      "en" => array:1 [
        0 => array:4 [
          "clase" => "keyword"
          "titulo" => "Key-words"
          "identificador" => "xpalclavsec158802"
          "palabras" => array:3 [
            0 => "Ehlers-Danlos Syndrome"
            1 => "pneumothorax"
            2 => "joint hypermobility"
          ]
        ]
      ]
    ]
    "tieneResumen" => true
    "resumen" => array:2 [
      "pt" => array:2 [
        "titulo" => "Resumo"
        "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>"
      ]
      "en" => array:2 [
        "titulo" => "Abstract"
        "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>"
      ]
    ]
    "bibliografia" => array:2 [
      "titulo" => "Bibliografia"
      "seccion" => array:1 [
        0 => array:2 [
          "identificador" => "bibs0005"
          "bibliografiaReferencia" => array:11 [
            0 => array:3 [
              "identificador" => "bib1"
              "etiqueta" => "1&#46;"
              "referencia" => array:1 [
                0 => array:1 [
                  "referenciaCompleta" => "Noronha LL&#44; Vieira JR&#46; Pneumot&#243;rax espont&#226;neo&#46; In Tratado de Pneumologia&#44; 1&#46;&#170; ed&#44; Gomes MJM&#44; Sotto-Mayor R&#46; &#40;eds&#46;&#41; Lisboa&#44; Permanyer Portugal&#44; 2003&#59; 1497-503&#46;"
                ]
              ]
            ]
            1 => array:3 [
              "identificador" => "bib2"
              "etiqueta" => "2&#46;"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Rheumatic and connective tissue diseases"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:1 [
                            0 => "C&#46;C&#46; Evans"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "LibroEditado" => array:4 [
                        "titulo" => "Respiratory Medicine"
                        "paginaInicial" => "2040"
                        "edicion" => "3rd"
                        "serieFecha" => "2002"
                      ]
                    ]
                  ]
                ]
              ]
            ]
            2 => array:3 [
              "identificador" => "bib3"
              "etiqueta" => "3&#46;"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Abnormalities of the lungs and thoracic cage in the Ehlers-Danlos syndrome"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:4 [
                            0 => "J&#46;G&#46; Ayres"
                            1 => "F&#46;M&#46; Pope"
                            2 => "J&#46;F&#46; Reidy"
                            3 => "T&#46;J&#46; Clark"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:6 [
                        "tituloSerie" => "Thorax"
                        "fecha" => "1985"
                        "volumen" => "40"
                        "paginaInicial" => "300"
                        "paginaFinal" => "305"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/4023980"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            3 => array:3 [
              "identificador" => "bib4"
              "etiqueta" => "4&#46;"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Bullous lungs&#58; diverse aetiology"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:3 [
                            0 => "S&#46; Kolekar"
                            1 => "P&#46; Sundaram"
                            2 => "J&#46;M&#46; Joshi"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:4 [
                        "tituloSerie" => "Post Grad Med J"
                        "fecha" => "2002"
                        "volumen" => "78"
                        "paginaInicial" => "689"
                      ]
                    ]
                  ]
                ]
              ]
            ]
            4 => array:3 [
              "identificador" => "bib5"
              "etiqueta" => "5&#46;"
              "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&#46;J&#46; Prockop"
                            1 => "H&#46; Kuivaniemi"
                            2 => "G&#46; Tromp"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "LibroEditado" => array:5 [
                        "titulo" => "Harrison&#39;s Principles of Internal Medicine"
                        "paginaInicial" => "2189"
                        "paginaFinal" => "2191"
                        "edicion" => "14th"
                        "serieFecha" => "1998"
                      ]
                    ]
                  ]
                ]
              ]
            ]
            5 => array:3 [
              "identificador" => "bib6"
              "etiqueta" => "6&#46;"
              "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&#46;E&#46; Pyeritz"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:5 [
                        "tituloSerie" => "N Engl J M"
                        "fecha" => "2000"
                        "volumen" => "342"
                        "paginaInicial" => "730"
                        "paginaFinal" => "732"
                      ]
                    ]
                  ]
                ]
              ]
            ]
            6 => array:3 [
              "identificador" => "bib7"
              "etiqueta" => "7&#46;"
              "referencia" => array:1 [
                0 => array:1 [
                  "referenciaCompleta" => "Symptoms and Signs of Respiratory Disease&#46; In Fishman&#39;s Pulmonary Diseases and Disorders&#44; 3rd ed&#44; Fishman A&#44; Elias J&#44; Fishman J&#44; Grippi M&#44; Kaiser L&#44; Senior R &#40;eds&#41;&#46; New York&#44; McGraw-Hill&#44; 1988&#59; 403&#46;"
                ]
              ]
            ]
            7 => array:3 [
              "identificador" => "bib8"
              "etiqueta" => "8&#46;"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Radiological Case of the Month Ehlers-Danlos Syndrome Type IV"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:2 [
                            0 => "K&#46;S&#46; Chiang"
                            1 => "B&#46;C&#46; Walters Jr&#46;"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "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" => "bib9"
              "etiqueta" => "9&#46;"
              "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&#46;L&#46; Darmstadt"
                            1 => "R&#46; Sidbury"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "LibroEditado" => array:5 [
                        "titulo" => "Nelson Textbook of Pediatrics"
                        "paginaInicial" => "2207"
                        "paginaFinal" => "2208"
                        "edicion" => "17th"
                        "serieFecha" => "2004"
                      ]
                    ]
                  ]
                ]
              ]
            ]
            9 => array:3 [
              "identificador" => "bib10"
              "etiqueta" => "10&#46;"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Fatal Hemoptysis in Ehlers-Danlos Syndrome"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:3 [
                            0 => "B&#46;A&#46; Yost"
                            1 => "J&#46;P&#46; Vogelsang"
                            2 => "J&#46;T&#46; Lie"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:6 [
                        "tituloSerie" => "Chest"
                        "fecha" => "1995"
                        "volumen" => "107"
                        "paginaInicial" => "1465"
                        "paginaFinal" => "1467"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/7750352"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            10 => array:3 [
              "identificador" => "bib11"
              "etiqueta" => "11&#46;"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Ehlers-Danlos syndrome type IV with few extrathoracic findings&#58; a newly recognized point mutation in the COL3A1 gene"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:11 [
                            0 => "A&#46; Watanabe"
                            1 => "Y&#46; Kawabata"
                            2 => "O&#46; Okada"
                            3 => "N&#46; Tanabe"
                            4 => "H&#46; Kimura"
                            5 => "A&#46; Hatamochi"
                            6 => "H&#46; Shinkai"
                            7 => "N&#46; Sakai"
                            8 => "T&#46; Shimada"
                            9 => "K&#46; Hiroshima"
                            10 => "T&#46; Kuriyama"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:6 [
                        "tituloSerie" => "Eur Respir J"
                        "fecha" => "2002"
                        "volumen" => "19"
                        "paginaInicial" => "195"
                        "paginaFinal" => "198"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/11843319"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
          ]
        ]
      ]
    ]
  ]
  "idiomaDefecto" => "pt"
  "url" => "/21735115/0000001200000004/v1_201305151425/S2173511506704158/v1_201305151425/pt/main.assets"
  "Apartado" => array:4 [
    "identificador" => "9550"
    "tipo" => "SECCION"
    "en" => array:2 [
      "titulo" => "Case Report&#47;Caso Cl&#237;nico"
      "idiomaDefecto" => true
    ]
    "idiomaDefecto" => "en"
  ]
  "PDF" => "https://static.elsevier.es/multimedia/21735115/0000001200000004/v1_201305151425/S2173511506704158/v1_201305151425/pt/main.pdf?idApp=UINPBA00004E&text.app=https://journalpulmonology.org/"
  "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2173511506704158?idApp=UINPBA00004E"
]
Share
Journal Information
Vol. 12. Issue 4.
Pages 471-480 (July - August 2006)
Share
Share
Download PDF
More article options
Vol. 12. Issue 4.
Pages 471-480 (July - August 2006)
Full text access
Síndroma de Ehlers-Danlos – Uma causa rara de pneumotórax espontâneo
Ehlers-Danlos syndrome – A rare cause of spontaneous pneumothorax
Visits
867
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
This item has received
Article information
Abstract
Bibliography
Download PDF
Statistics
Resumo

A síndroma de Ehlers-Danlos (cutis hyperelastica), constitui uma patologia do tecido conjuntivo caracterizada por alterações da pele, ligamentos e órgãos internos.

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.

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.

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.

Palavras-chave:
Síndroma de Ehlers-Danlos
pneumotórax
hipermobilidade articular
Abstract

Ehlers-Danlos syndrome (cutis hyperelastica), is a group of connective tissue disorders characterized by abnormalities of the skin, ligaments and internal organs.

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.

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.

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.

Key-words:
Ehlers-Danlos Syndrome
pneumothorax
joint hypermobility
Full text is only aviable in PDF
Bibliografia
[1.]
Noronha LL, Vieira JR. Pneumotórax espontâneo. In Tratado de Pneumologia, 1.ª ed, Gomes MJM, Sotto-Mayor R. (eds.) Lisboa, Permanyer Portugal, 2003; 1497-503.
[2.]
C.C. Evans.
Rheumatic and connective tissue diseases.
Respiratory Medicine, 3rd, pp. 2040
[3.]
J.G. Ayres, F.M. Pope, J.F. Reidy, T.J. Clark.
Abnormalities of the lungs and thoracic cage in the Ehlers-Danlos syndrome.
Thorax, 40 (1985), pp. 300-305
[4.]
S. Kolekar, P. Sundaram, J.M. Joshi.
Bullous lungs: diverse aetiology.
Post Grad Med J, 78 (2002), pp. 689
[5.]
D.J. Prockop, H. Kuivaniemi, G. Tromp.
Inherited Disorders of Conective Tissue.
Harrison's Principles of Internal Medicine, 14th, pp. 2189-2191
[6.]
R.E. Pyeritz.
Ehlers-Danlos Syndrome.
N Engl J M, 342 (2000), pp. 730-732
[7.]
Symptoms and Signs of Respiratory Disease. In Fishman's Pulmonary Diseases and Disorders, 3rd ed, Fishman A, Elias J, Fishman J, Grippi M, Kaiser L, Senior R (eds). New York, McGraw-Hill, 1988; 403.
[8.]
K.S. Chiang, B.C. Walters Jr..
Radiological Case of the Month Ehlers-Danlos Syndrome Type IV.
Applied Radiology, 32 (2003), pp. 48-50
[9.]
G.L. Darmstadt, R. Sidbury.
The Skin.
Nelson Textbook of Pediatrics, 17th, pp. 2207-2208
[10.]
B.A. Yost, J.P. Vogelsang, J.T. Lie.
Fatal Hemoptysis in Ehlers-Danlos Syndrome.
Chest, 107 (1995), pp. 1465-1467
[11.]
A. Watanabe, Y. Kawabata, O. Okada, N. Tanabe, H. Kimura, A. Hatamochi, H. Shinkai, N. Sakai, T. Shimada, K. Hiroshima, T. Kuriyama.
Ehlers-Danlos syndrome type IV with few extrathoracic findings: a newly recognized point mutation in the COL3A1 gene.
Eur Respir J, 19 (2002), pp. 195-198
Copyright © 2006. Sociedade Portuguesa de Pneumologia
Download PDF
Pulmonology
Article options
Tools

Are you a health professional able to prescribe or dispense drugs?