array:24 [
  "pii" => "S2173511509702507"
  "issn" => "21735115"
  "doi" => "10.1016/S2173-5115(09)70250-7"
  "estado" => "S300"
  "fechaPublicacion" => "2008-01-01"
  "aid" => "70250"
  "copyright" => "Sociedade Portuguesa de Pneumologia"
  "copyrightAnyo" => "2008"
  "documento" => "simple-article"
  "crossmark" => 0
  "licencia" => "http://www.elsevier.com/open-access/userlicense/1.0/"
  "subdocumento" => "cor"
  "cita" => "Rev Port Pneumol. 2008;14:159-64"
  "abierto" => array:3 [
    "ES" => true
    "ES2" => true
    "LATM" => true
  ]
  "gratuito" => true
  "lecturas" => array:2 [
    "total" => 3504
    "formatos" => array:3 [
      "EPUB" => 244
      "HTML" => 1854
      "PDF" => 1406
    ]
  ]
  "itemSiguiente" => array:19 [
    "pii" => "S2173511509702519"
    "issn" => "21735115"
    "doi" => "10.1016/S2173-5115(09)70251-9"
    "estado" => "S300"
    "fechaPublicacion" => "2008-01-01"
    "aid" => "70251"
    "copyright" => "Sociedade Portuguesa de Pneumologia"
    "documento" => "simple-article"
    "crossmark" => 0
    "licencia" => "http://www.elsevier.com/open-access/userlicense/1.0/"
    "subdocumento" => "err"
    "cita" => "Rev Port Pneumol. 2008;14:165"
    "abierto" => array:3 [
      "ES" => true
      "ES2" => true
      "LATM" => true
    ]
    "gratuito" => true
    "lecturas" => array:2 [
      "total" => 3164
      "formatos" => array:3 [
        "EPUB" => 195
        "HTML" => 1633
        "PDF" => 1336
      ]
    ]
    "pt" => array:6 [
      "idiomaDefecto" => true
      "titulo" => "Corrigenda"
      "tienePdf" => "pt"
      "tieneTextoCompleto" => 0
      "paginas" => array:1 [
        0 => array:1 [
          "paginaInicial" => "165"
        ]
      ]
      "contienePdf" => array:1 [
        "pt" => true
      ]
    ]
    "idiomaDefecto" => "pt"
    "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2173511509702519?idApp=UINPBA00004E"
    "url" => "/21735115/0000001400000001/v1_201305151449/S2173511509702519/v1_201305151449/pt/main.assets"
  ]
  "itemAnterior" => array:19 [
    "pii" => "S2173511509702490"
    "issn" => "21735115"
    "doi" => "10.1016/S2173-5115(09)70249-0"
    "estado" => "S300"
    "fechaPublicacion" => "2008-01-01"
    "aid" => "70249"
    "copyright" => "Sociedade Portuguesa de Pneumologia"
    "documento" => "simple-article"
    "crossmark" => 0
    "licencia" => "http://www.elsevier.com/open-access/userlicense/1.0/"
    "subdocumento" => "cor"
    "cita" => "Rev Port Pneumol. 2008;14:151-7"
    "abierto" => array:3 [
      "ES" => true
      "ES2" => true
      "LATM" => true
    ]
    "gratuito" => true
    "lecturas" => array:2 [
      "total" => 4775
      "formatos" => array:3 [
        "EPUB" => 214
        "HTML" => 2076
        "PDF" => 2485
      ]
    ]
    "pt" => array:10 [
      "idiomaDefecto" => true
      "titulo" => "Pneumonia por <span class="elsevierStyleItalic">Pneumocystis</span> e citomegalov&#237;rus no doente VIH &#8211; A prop&#243;sito de dois casos cl&#237;nicos"
      "tienePdf" => "pt"
      "tieneTextoCompleto" => 0
      "tieneResumen" => array:2 [
        0 => "pt"
        1 => "en"
      ]
      "paginas" => array:1 [
        0 => array:2 [
          "paginaInicial" => "151"
          "paginaFinal" => "157"
        ]
      ]
      "titulosAlternativos" => array:1 [
        "en" => array:1 [
          "titulo" => "Pneumocystis and cytomegalovirus pneumonia in HIV patients &#8211; Two clinical cases"
        ]
      ]
      "contieneResumen" => array:2 [
        "pt" => true
        "en" => true
      ]
      "contienePdf" => array:1 [
        "pt" => true
      ]
      "autores" => array:1 [
        0 => array:2 [
          "autoresLista" => "Sofia Louren&#231;o, Marta Amaral, Raquel Almeida, Paulo Marcelino, Susan Marum, M&#46;&#170; Adelaide Milheiro, Judite Oliveira, Lu&#237;s Mour&#227;o"
          "autores" => array:8 [
            0 => array:2 [
              "nombre" => "Sofia"
              "apellidos" => "Louren&#231;o"
            ]
            1 => array:2 [
              "nombre" => "Marta"
              "apellidos" => "Amaral"
            ]
            2 => array:2 [
              "nombre" => "Raquel"
              "apellidos" => "Almeida"
            ]
            3 => array:2 [
              "nombre" => "Paulo"
              "apellidos" => "Marcelino"
            ]
            4 => array:2 [
              "nombre" => "Susan"
              "apellidos" => "Marum"
            ]
            5 => array:2 [
              "nombre" => "M&#46;&#170;"
              "apellidos" => "Adelaide Milheiro"
            ]
            6 => array:2 [
              "nombre" => "Judite"
              "apellidos" => "Oliveira"
            ]
            7 => array:2 [
              "nombre" => "Lu&#237;s"
              "apellidos" => "Mour&#227;o"
            ]
          ]
        ]
      ]
    ]
    "idiomaDefecto" => "pt"
    "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2173511509702490?idApp=UINPBA00004E"
    "url" => "/21735115/0000001400000001/v1_201305151449/S2173511509702490/v1_201305151449/pt/main.assets"
  ]
  "pt" => array:14 [
    "idiomaDefecto" => true
    "titulo" => "Aspectos respirat&#243;rios da doen&#231;a de Pompe&#58; Relato de caso"
    "tieneTextoCompleto" => 0
    "paginas" => array:1 [
      0 => array:2 [
        "paginaInicial" => "159"
        "paginaFinal" => "164"
      ]
    ]
    "autores" => array:1 [
      0 => array:4 [
        "autoresLista" => "Ewandro L&#46;R&#46; Moura, Thiago A&#46; Jabuonski, Veronica M&#46; Amado"
        "autores" => array:3 [
          0 => array:3 [
            "nombre" => "Ewandro L&#46;R&#46;"
            "apellidos" => "Moura"
            "referencia" => array:1 [
              0 => array:2 [
                "etiqueta" => "<span class="elsevierStyleSup">1</span>"
                "identificador" => "aff0005"
              ]
            ]
          ]
          1 => array:3 [
            "nombre" => "Thiago A&#46;"
            "apellidos" => "Jabuonski"
            "referencia" => array:1 [
              0 => array:2 [
                "etiqueta" => "<span class="elsevierStyleSup">1</span>"
                "identificador" => "aff0005"
              ]
            ]
          ]
          2 => array:4 [
            "nombre" => "Veronica M&#46;"
            "apellidos" => "Amado"
            "email" => array:1 [
              0 => "veronicaamado&#64;uol&#46;com&#46;br"
            ]
            "referencia" => array:2 [
              0 => array:2 [
                "etiqueta" => "<span class="elsevierStyleSup">2</span>"
                "identificador" => "aff0010"
              ]
              1 => array:2 [
                "etiqueta" => "<span class="elsevierStyleSup">¿</span>"
                "identificador" => "cor0005"
              ]
            ]
          ]
        ]
        "afiliaciones" => array:2 [
          0 => array:3 [
            "entidad" => "Alunos do curso de gradua&#231;&#227;o em Medicina da Faculdade de Medicina&#44; Universidade de Bras&#237;lia&#44; Bras&#237;lia-DF"
            "etiqueta" => "<span class="elsevierStyleSup">1</span>"
            "identificador" => "aff0005"
          ]
          1 => array:3 [
            "entidad" => "Professor Adjunto do Departamento de Cl&#237;nica M&#233;dica&#44; Faculdade de Medicina&#44; Universidade de Bras&#237;lia &#8211; UnB&#44; Bras&#237;lia-DF"
            "etiqueta" => "<span class="elsevierStyleSup">2</span>"
            "identificador" => "aff0010"
          ]
        ]
        "correspondencia" => array:1 [
          0 => array:3 [
            "identificador" => "cor0005"
            "etiqueta" => "&#8270;"
            "correspondencia" => "Correspond&#234;ncia&#58; SQS-315 Bl-A Ap-201&#59; CEP&#58; 70&#46;384-010&#46; Bras&#237;lia &#8211;DF&#46;"
          ]
        ]
      ]
    ]
    "titulosAlternativos" => array:1 [
      "en" => array:1 [
        "titulo" => "Respiratory aspects of Pompe disease&#58; Case report"
      ]
    ]
    "pdfFichero" => "main.pdf"
    "tienePdf" => true
    "fechaRecibido" => "2007-04-24"
    "fechaAceptado" => "2007-09-12"
    "PalabrasClave" => array:2 [
      "pt" => array:1 [
        0 => array:4 [
          "clase" => "keyword"
          "titulo" => "Palavras-chave"
          "identificador" => "xpalclavsec159430"
          "palabras" => array:5 [
            0 => "Doen&#231;a de dep&#243;sito de glicog&#233;nio tipo II"
            1 => "doen&#231;a de Pompe"
            2 => "miopatia"
            3 => "insufici&#234;ncia respirat&#243;ria"
            4 => "ventila&#231;&#227;o mec&#226;nica"
          ]
        ]
      ]
      "en" => array:1 [
        0 => array:4 [
          "clase" => "keyword"
          "titulo" => "Key-words"
          "identificador" => "xpalclavsec159431"
          "palabras" => array:6 [
            0 => "Glycogen storage disease type II"
            1 => "Pompe disease"
            2 => "myopathy"
            3 => "respiratory failure"
            4 => "ventilation"
            5 => "mechanical"
          ]
        ]
      ]
    ]
    "tieneResumen" => true
    "resumen" => array:2 [
      "pt" => array:2 [
        "titulo" => "Resumo"
        "resumen" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Relatamos o caso de doente feminina&#44; 38 anos&#44; com diagn&#243;stico de doen&#231;a de Pompe h&#225; oito anos&#46; H&#225; cinco anos vinha com queixas respirat&#243;rias&#44; evoluindo com insufici&#234;ncia respirat&#243;ria cr&#243;nica&#46; Em uso de CPAP &#40;<span class="elsevierStyleItalic">continuous positive airway pressure</span>&#41; nocturno h&#225; um ano&#44; apresentava agravamento cl&#237;nico progressivo&#46; Veio encaminhada ao Hospital Universit&#225;rio de Bras&#237;lia&#44; com descompensa&#231;&#227;o respirat&#243;ria e <span class="elsevierStyleItalic">cor pulmonale</span>&#46; Iniciou-se ventila&#231;&#227;o n&#227;o invasiva com dois n&#237;veis de press&#245;es &#40;VNI&#41; e realizou-se polissonografia&#44; que demonstrou a necessidade do ajuste de frequ&#234;ncia respirat&#243;ria associada aos ajustes das press&#245;es para adequa&#231;&#227;o da ventila&#231;&#227;o durante o sono&#46; Evoluiu com melhora importante ap&#243;s uso da VNI</p>"
      ]
      "en" => array:2 [
        "titulo" => "Abstract"
        "resumen" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">We report the case of a 38 years-old female patient with Pompe disease&#44; diagnosed eight years ago&#46; Respiratory complaints appeared five years ago progressing to chronic respiratory failure&#46; Nocturnal CPAP &#40;continuous positive airway pressure&#41; was prescribed last year&#44; presenting progressive clinical worsening&#46; She was referred to Hospital Universit&#225;rio de Bras&#237;lia&#44; on account of respiratory failure and cor pulmonale&#46; Thus&#44; she began non-invasive ventilation &#40;NIV&#41; with bi-level positive airways pressure&#44; and a polysomnography showed the need of spontaneous&#47;timed mode associated with adjustments in breathing pressures to improve ventilation during sleep&#46; There was significant clinical improvement after NIV support was established</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:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Alpha-Glucosidase deficiency in generalized glycogen storage disease &#40;Pompe&#39;s disease&#41;"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:1 [
                            0 => "H&#46;G&#46; Hers"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:6 [
                        "tituloSerie" => "Biochem J"
                        "fecha" => "1963"
                        "volumen" => "86"
                        "paginaInicial" => "11"
                        "paginaFinal" => "16"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/13954110"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            1 => array:3 [
              "identificador" => "bib2"
              "etiqueta" => "2&#46;"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "The natural course of infantile Pompe&#39;s disease&#58; 20 original cases compared with 133 cases from the literature"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "H&#46;M&#46; Van den Hout"
                            1 => "W&#46; Hop"
                            2 => "O&#46;P&#46; Van Diggelen"
                            3 => "J&#46;A&#46; Smeitink"
                            4 => "G&#46;P&#46; Smit"
                            5 => "B&#46;T&#46; Poll-The"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:7 [
                        "tituloSerie" => "Pediatrics"
                        "fecha" => "2003"
                        "volumen" => "112"
                        "numero" => "2"
                        "paginaInicial" => "332"
                        "paginaFinal" => "340"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/12897283"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            2 => array:3 [
              "identificador" => "bib3"
              "etiqueta" => "3&#46;"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "A retrospective&#44; multinational&#44; multicenter study on the natural history of infantile-onset Pompe disease"
                      "autores" => array:1 [
                        0 => array:3 [
                          "colaboracion" => "Infantile-onset Pompe Disease Natural History Study Group"
                          "etal" => false
                          "autores" => array:6 [
                            0 => "P&#46; Kishnani"
                            1 => "W&#46; Hwu"
                            2 => "H&#46; Mandel"
                            3 => "M&#46; Nicolino"
                            4 => "F&#46; Yong"
                            5 => "D&#46; Corzo"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1016/j.jpeds.2005.11.033"
                      "Revista" => array:7 [
                        "tituloSerie" => "J Pediatr"
                        "fecha" => "2006"
                        "volumen" => "148"
                        "numero" => "5"
                        "paginaInicial" => "671"
                        "paginaFinal" => "676"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/16737883"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            3 => array:3 [
              "identificador" => "bib4"
              "etiqueta" => "4&#46;"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Disease severity in children and adults with Pompe disease related to age and disease duration"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:6 [
                            0 => "M&#46;L&#46;C&#46; Hagemans"
                            1 => "L&#46;P&#46;F&#46; Winkel"
                            2 => "W&#46;C&#46;J&#46; Hop"
                            3 => "A&#46;J&#46;J&#46; Reuser"
                            4 => "P&#46;A&#46; Van Doorn"
                            5 => "A&#46;T&#46; Van der Ploeg"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1212/01.WNL.0000165979.46537.56"
                      "Revista" => array:6 [
                        "tituloSerie" => "Neurology"
                        "fecha" => "2005"
                        "volumen" => "64"
                        "paginaInicial" => "2139"
                        "paginaFinal" => "2141"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/15985590"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            4 => array:3 [
              "identificador" => "bib5"
              "etiqueta" => "5&#46;"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Approach to gene therapy of glycogenosis type II &#40;Pompe disease&#41;"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:1 [
                            0 => "L&#46; Poenaru"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1006/mgme.2000.3003"
                      "Revista" => array:7 [
                        "tituloSerie" => "Mol Genet Metab"
                        "fecha" => "2000"
                        "volumen" => "70"
                        "numero" => "3"
                        "paginaInicial" => "163"
                        "paginaFinal" => "169"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/10924270"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            5 => array:3 [
              "identificador" => "bib6"
              "etiqueta" => "6&#46;"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Recent advances in respiratory care for neuromuscular disease"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:1 [
                            0 => "A&#46;K&#46; Simonds"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1378/chest.130.6.1879"
                      "Revista" => array:7 [
                        "tituloSerie" => "Chest"
                        "fecha" => "2006"
                        "volumen" => "130"
                        "numero" => "6"
                        "paginaInicial" => "1879"
                        "paginaFinal" => "1886"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/17167012"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            6 => array:3 [
              "identificador" => "bib7"
              "etiqueta" => "7&#46;"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Mechanisms of improvement of respiratory failure in patients with restrictive thoracic disease treated with non-invasive ventilation"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:6 [
                            0 => "A&#46;H&#46; Nickol"
                            1 => "N&#46; Hart"
                            2 => "N&#46;S&#46; Hopkinson"
                            3 => "J&#46; Moxham"
                            4 => "A&#46; Simonds"
                            5 => "M&#46; Polkey"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1136/thx.2004.039388"
                      "Revista" => array:6 [
                        "tituloSerie" => "Thorax"
                        "fecha" => "2005"
                        "volumen" => "60"
                        "paginaInicial" => "754"
                        "paginaFinal" => "760"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/15939731"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            7 => array:3 [
              "identificador" => "bib8"
              "etiqueta" => "8&#46;"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Patterns and predictors of sleep disordered breathing in primary myopathies"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:5 [
                            0 => "R&#46; Ragette"
                            1 => "U&#46; Mellies"
                            2 => "C&#46; Schwake"
                            3 => "T&#46; Voit"
                            4 => "H&#46; Teschler"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:7 [
                        "tituloSerie" => "Thorax"
                        "fecha" => "2002"
                        "volumen" => "57"
                        "numero" => "8"
                        "paginaInicial" => "724"
                        "paginaFinal" => "728"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/12149535"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            8 => array:3 [
              "identificador" => "bib9"
              "etiqueta" => "9&#46;"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Respiratory failure in Pompe disease&#58; Treatment with noninvasive ventilation"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:6 [
                            0 => "U&#46; Mellies"
                            1 => "F&#46; Stehling"
                            2 => "C&#46; Dohna-Schwake"
                            3 => "R&#46; Ragette"
                            4 => "H&#46; Teschler"
                            5 => "T&#46; Voit"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1212/01.WNL.0000158682.85052.C0"
                      "Revista" => array:6 [
                        "tituloSerie" => "Neurology"
                        "fecha" => "2005"
                        "volumen" => "64"
                        "paginaInicial" => "1465"
                        "paginaFinal" => "1467"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/15851748"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            9 => array:3 [
              "identificador" => "bib10"
              "etiqueta" => "10&#46;"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Enzyme replacement therapy in classical infantile Pompe disease&#58; results of a ten-month follow-up study"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:4 [
                            0 => "L&#46; Klinge"
                            1 => "V&#46; Straub"
                            2 => "U&#46; Neudorf"
                            3 => "T&#46; Voit"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1055/s-2005-837543"
                      "Revista" => array:6 [
                        "tituloSerie" => "Neuropediatrics"
                        "fecha" => "2005"
                        "volumen" => "36"
                        "paginaInicial" => "6"
                        "paginaFinal" => "11"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/15776317"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            10 => array:3 [
              "identificador" => "bib11"
              "etiqueta" => "11&#46;"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Safety and efficacy of recombinant acid alpha-glucosidase &#40;rhGAA&#41; in patients with classical infantile Pompe disease&#58; results of a phase II clinical trial"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "L&#46; Klinge"
                            1 => "V&#46; Straub"
                            2 => "U&#46; Neudorf"
                            3 => "J&#46; Schaper"
                            4 => "T&#46; Bosbach"
                            5 => "K&#46; Gorlinger"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1016/j.nmd.2004.10.009"
                      "Revista" => array:7 [
                        "tituloSerie" => "Neuromuscul Disord"
                        "fecha" => "2005"
                        "volumen" => "15"
                        "numero" => "1"
                        "paginaInicial" => "24"
                        "paginaFinal" => "31"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/15639117"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
          ]
        ]
      ]
    ]
  ]
  "idiomaDefecto" => "pt"
  "url" => "/21735115/0000001400000001/v1_201305151449/S2173511509702507/v1_201305151449/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/21735115/0000001400000001/v1_201305151449/S2173511509702507/v1_201305151449/pt/main.pdf?idApp=UINPBA00004E&text.app=https://journalpulmonology.org/"
  "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2173511509702507?idApp=UINPBA00004E"
]
Share
Journal Information
Vol. 14. Issue 1.
Pages 159-164 (January - February 2008)
Share
Share
Download PDF
More article options
Vol. 14. Issue 1.
Pages 159-164 (January - February 2008)
Full text access
Aspectos respiratórios da doença de Pompe: Relato de caso
Respiratory aspects of Pompe disease: Case report
Visits
847
Ewandro L.R. Moura1, Thiago A. Jabuonski1, Veronica M. Amado2,
Corresponding author
veronicaamado@uol.com.br

Correspondência: SQS-315 Bl-A Ap-201; CEP: 70.384-010. Brasília –DF.
1 Alunos do curso de graduação em Medicina da Faculdade de Medicina, Universidade de Brasília, Brasília-DF
2 Professor Adjunto do Departamento de Clínica Médica, Faculdade de Medicina, Universidade de Brasília – UnB, Brasília-DF
This item has received
Article information
Resumo

Relatamos o caso de doente feminina, 38 anos, com diagnóstico de doença de Pompe há oito anos. Há cinco anos vinha com queixas respiratórias, evoluindo com insuficiência respiratória crónica. Em uso de CPAP (continuous positive airway pressure) nocturno há um ano, apresentava agravamento clínico progressivo. Veio encaminhada ao Hospital Universitário de Brasília, com descompensação respiratória e cor pulmonale. Iniciou-se ventilação não invasiva com dois níveis de pressões (VNI) e realizou-se polissonografia, que demonstrou a necessidade do ajuste de frequência respiratória associada aos ajustes das pressões para adequação da ventilação durante o sono. Evoluiu com melhora importante após uso da VNI

Palavras-chave:
Doença de depósito de glicogénio tipo II
doença de Pompe
miopatia
insuficiência respiratória
ventilação mecânica
Abstract

We report the case of a 38 years-old female patient with Pompe disease, diagnosed eight years ago. Respiratory complaints appeared five years ago progressing to chronic respiratory failure. Nocturnal CPAP (continuous positive airway pressure) was prescribed last year, presenting progressive clinical worsening. She was referred to Hospital Universitário de Brasília, on account of respiratory failure and cor pulmonale. Thus, she began non-invasive ventilation (NIV) with bi-level positive airways pressure, and a polysomnography showed the need of spontaneous/timed mode associated with adjustments in breathing pressures to improve ventilation during sleep. There was significant clinical improvement after NIV support was established

Key-words:
Glycogen storage disease type II
Pompe disease
myopathy
respiratory failure
ventilation
mechanical
Full text is only aviable in PDF
Bibliografia
[1.]
H.G. Hers.
Alpha-Glucosidase deficiency in generalized glycogen storage disease (Pompe's disease).
Biochem J, 86 (1963), pp. 11-16
[2.]
H.M. Van den Hout, W. Hop, O.P. Van Diggelen, J.A. Smeitink, G.P. Smit, B.T. Poll-The, et al.
The natural course of infantile Pompe's disease: 20 original cases compared with 133 cases from the literature.
Pediatrics, 112 (2003), pp. 332-340
[3.]
P. Kishnani, W. Hwu, H. Mandel, M. Nicolino, F. Yong, D. Corzo, Infantile-onset Pompe Disease Natural History Study Group.
A retrospective, multinational, multicenter study on the natural history of infantile-onset Pompe disease.
J Pediatr, 148 (2006), pp. 671-676
[4.]
M.L.C. Hagemans, L.P.F. Winkel, W.C.J. Hop, A.J.J. Reuser, P.A. Van Doorn, A.T. Van der Ploeg.
Disease severity in children and adults with Pompe disease related to age and disease duration.
[5.]
L. Poenaru.
Approach to gene therapy of glycogenosis type II (Pompe disease).
Mol Genet Metab, 70 (2000), pp. 163-169
[6.]
A.K. Simonds.
Recent advances in respiratory care for neuromuscular disease.
Chest, 130 (2006), pp. 1879-1886
[7.]
A.H. Nickol, N. Hart, N.S. Hopkinson, J. Moxham, A. Simonds, M. Polkey.
Mechanisms of improvement of respiratory failure in patients with restrictive thoracic disease treated with non-invasive ventilation.
Thorax, 60 (2005), pp. 754-760
[8.]
R. Ragette, U. Mellies, C. Schwake, T. Voit, H. Teschler.
Patterns and predictors of sleep disordered breathing in primary myopathies.
Thorax, 57 (2002), pp. 724-728
[9.]
U. Mellies, F. Stehling, C. Dohna-Schwake, R. Ragette, H. Teschler, T. Voit.
Respiratory failure in Pompe disease: Treatment with noninvasive ventilation.
[10.]
L. Klinge, V. Straub, U. Neudorf, T. Voit.
Enzyme replacement therapy in classical infantile Pompe disease: results of a ten-month follow-up study.
Neuropediatrics, 36 (2005), pp. 6-11
[11.]
L. Klinge, V. Straub, U. Neudorf, J. Schaper, T. Bosbach, K. Gorlinger, et al.
Safety and efficacy of recombinant acid alpha-glucosidase (rhGAA) in patients with classical infantile Pompe disease: results of a phase II clinical trial.
Neuromuscul Disord, 15 (2005), pp. 24-31
Copyright © 2008. Sociedade Portuguesa de Pneumologia
Download PDF
Pulmonology
Article options
Tools

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