array:24 [
  "pii" => "S0873215915302385"
  "issn" => "08732159"
  "doi" => "10.1016/S0873-2159(15)30238-5"
  "estado" => "S350"
  "fechaPublicacion" => "2008-03-01"
  "aid" => "11"
  "copyright" => "Sociedade Portuguesa de Pneumologia/SPP"
  "copyrightAnyo" => "2008"
  "documento" => "simple-article"
  "crossmark" => 0
  "licencia" => "http://creativecommons.org/licenses/by-nc-nd/4.0/"
  "subdocumento" => "fla"
  "cita" => "Rev Port Pneumol. 2008;14:295-302"
  "abierto" => array:3 [
    "ES" => true
    "ES2" => true
    "LATM" => true
  ]
  "gratuito" => true
  "lecturas" => array:2 [
    "total" => 2602
    "formatos" => array:3 [
      "EPUB" => 229
      "HTML" => 1589
      "PDF" => 784
    ]
  ]
  "itemSiguiente" => array:19 [
    "pii" => "S0873215915302397"
    "issn" => "08732159"
    "doi" => "10.1016/S0873-2159(15)30239-7"
    "estado" => "S350"
    "fechaPublicacion" => "2008-03-01"
    "aid" => "12"
    "copyright" => "Sociedade Portuguesa de Pneumologia/SPP"
    "documento" => "simple-article"
    "crossmark" => 0
    "licencia" => "http://creativecommons.org/licenses/by-nc-nd/4.0/"
    "subdocumento" => "fla"
    "cita" => "Rev Port Pneumol. 2008;14:303-8"
    "abierto" => array:3 [
      "ES" => true
      "ES2" => true
      "LATM" => true
    ]
    "gratuito" => true
    "lecturas" => array:2 [
      "total" => 2347
      "formatos" => array:3 [
        "EPUB" => 211
        "HTML" => 1498
        "PDF" => 638
      ]
    ]
    "pt" => array:11 [
      "idiomaDefecto" => true
      "cabecera" => "<span class="elsevierStyleTextfn">Caso Cl&#237;nico&#47;Clinical Case</span>"
      "titulo" => "Hiperventila&#231;&#227;o&#46; A terapia cognitivo-comportamental e a t&#233;cnica dos exerc&#237;cios de indu&#231;&#227;o dos sintomas no transtorno de p&#226;nico"
      "tienePdf" => "pt"
      "tieneTextoCompleto" => 0
      "tieneResumen" => array:2 [
        0 => "pt"
        1 => "en"
      ]
      "paginas" => array:1 [
        0 => array:2 [
          "paginaInicial" => "303"
          "paginaFinal" => "308"
        ]
      ]
      "titulosAlternativos" => array:1 [
        "en" => array:1 [
          "titulo" => "Hyperventilation&#46; The cognitive-behavior-therapy and the technique of the exercises in the induction of panic disorder symptoms"
        ]
      ]
      "contieneResumen" => array:2 [
        "pt" => true
        "en" => true
      ]
      "contienePdf" => array:1 [
        "pt" => true
      ]
      "autores" => array:1 [
        0 => array:2 [
          "autoresLista" => "Anna Lucia Spear King, Alexandre Martins Valen&#231;a, Ant&#243;nio Eg&#237;dio Nardi"
          "autores" => array:3 [
            0 => array:2 [
              "nombre" => "Anna Lucia Spear"
              "apellidos" => "King"
            ]
            1 => array:2 [
              "nombre" => "Alexandre Martins"
              "apellidos" => "Valen&#231;a"
            ]
            2 => array:2 [
              "nombre" => "Ant&#243;nio Eg&#237;dio"
              "apellidos" => "Nardi"
            ]
          ]
        ]
      ]
    ]
    "idiomaDefecto" => "pt"
    "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0873215915302397?idApp=UINPBA00004E"
    "url" => "/08732159/0000001400000002/v1_201509151520/S0873215915302397/v1_201509151520/pt/main.assets"
  ]
  "itemAnterior" => array:19 [
    "pii" => "S0873215915302373"
    "issn" => "08732159"
    "doi" => "10.1016/S0873-2159(15)30237-3"
    "estado" => "S350"
    "fechaPublicacion" => "2008-03-01"
    "aid" => "10"
    "copyright" => "Sociedade Portuguesa de Pneumologia&#47;SPP"
    "documento" => "simple-article"
    "crossmark" => 0
    "licencia" => "http://creativecommons.org/licenses/by-nc-nd/4.0/"
    "subdocumento" => "fla"
    "cita" => "Rev Port Pneumol. 2008;14:291-4"
    "abierto" => array:3 [
      "ES" => true
      "ES2" => true
      "LATM" => true
    ]
    "gratuito" => true
    "lecturas" => array:2 [
      "total" => 3305
      "formatos" => array:3 [
        "EPUB" => 237
        "HTML" => 2386
        "PDF" => 682
      ]
    ]
    "pt" => array:11 [
      "idiomaDefecto" => true
      "cabecera" => "<span class="elsevierStyleTextfn">Caso Cl&#237;nico&#47;Clinical Case</span>"
      "titulo" => "Um caso raro de hemangioma esclerosante m&#250;ltiplo do pulm&#227;o"
      "tienePdf" => "pt"
      "tieneTextoCompleto" => 0
      "tieneResumen" => array:2 [
        0 => "pt"
        1 => "en"
      ]
      "paginas" => array:1 [
        0 => array:2 [
          "paginaInicial" => "291"
          "paginaFinal" => "294"
        ]
      ]
      "titulosAlternativos" => array:1 [
        "en" => array:1 [
          "titulo" => "A rare case of multiple sclerosing hemangiomas of the lung"
        ]
      ]
      "contieneResumen" => array:2 [
        "pt" => true
        "en" => true
      ]
      "contienePdf" => array:1 [
        "pt" => true
      ]
      "autores" => array:1 [
        0 => array:2 [
          "autoresLista" => "Jos&#233; Pedro Bol&#233;o-Tom&#233;, Cristina Matos, Fernando Nogueira, Manuela Maya, Jos&#233; Sena Lino, Margarida Cancela de Abreu"
          "autores" => array:6 [
            0 => array:2 [
              "nombre" => "Jos&#233; Pedro"
              "apellidos" => "Bol&#233;o-Tom&#233;"
            ]
            1 => array:2 [
              "nombre" => "Cristina"
              "apellidos" => "Matos"
            ]
            2 => array:2 [
              "nombre" => "Fernando"
              "apellidos" => "Nogueira"
            ]
            3 => array:2 [
              "nombre" => "Manuela"
              "apellidos" => "Maya"
            ]
            4 => array:2 [
              "nombre" => "Jos&#233; Sena"
              "apellidos" => "Lino"
            ]
            5 => array:2 [
              "nombre" => "Margarida Cancela"
              "apellidos" => "de Abreu"
            ]
          ]
        ]
      ]
    ]
    "idiomaDefecto" => "pt"
    "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0873215915302373?idApp=UINPBA00004E"
    "url" => "/08732159/0000001400000002/v1_201509151520/S0873215915302373/v1_201509151520/pt/main.assets"
  ]
  "pt" => array:15 [
    "idiomaDefecto" => true
    "cabecera" => "<span class="elsevierStyleTextfn">Caso Cl&#237;nico&#47;Clinical Case</span>"
    "titulo" => "D&#233;fice de alfa-1 antitripsina&#46; A prop&#243;sito de dois casos cl&#237;nicos"
    "tieneTextoCompleto" => 0
    "paginas" => array:1 [
      0 => array:2 [
        "paginaInicial" => "295"
        "paginaFinal" => "302"
      ]
    ]
    "autores" => array:1 [
      0 => array:3 [
        "autoresLista" => "S&#243;nia Serra, Gra&#231;a Banha"
        "autores" => array:2 [
          0 => array:3 [
            "nombre" => "S&#243;nia"
            "apellidos" => "Serra"
            "referencia" => array:1 [
              0 => array:2 [
                "etiqueta" => "<span class="elsevierStyleSup">1</span>"
                "identificador" => "af0005"
              ]
            ]
          ]
          1 => array:3 [
            "nombre" => "Gra&#231;a"
            "apellidos" => "Banha"
            "referencia" => array:1 [
              0 => array:2 [
                "etiqueta" => "<span class="elsevierStyleSup">2</span>"
                "identificador" => "af0010"
              ]
            ]
          ]
        ]
        "afiliaciones" => array:2 [
          0 => array:3 [
            "entidad" => "Interna do Complementar de Medicina Interna&#44; Servi&#231;o de Pneumologia&#44; Centro Hospitalar de Set&#250;bal&#44; EPE&#44; Rua Camilo Castelo Branco&#44; 2910-446 Set&#250;bal &#8211; Portugal"
            "etiqueta" => "1"
            "identificador" => "af0005"
          ]
          1 => array:3 [
            "entidad" => "Assistente Hospitalar Graduada de Pneumologia&#44; Servi&#231;o de Pneumologia&#44; Centro Hospitalar de Set&#250;bal&#44; EPE&#44; Rua Camilo Castelo Branco&#44; 2910-446 Set&#250;bal &#8211; Portugal"
            "etiqueta" => "2"
            "identificador" => "af0010"
          ]
        ]
      ]
    ]
    "titulosAlternativos" => array:1 [
      "en" => array:1 [
        "titulo" => "Alpha-1 antitrypsin deficiency&#46; Two cases report"
      ]
    ]
    "pdfFichero" => "main.pdf"
    "tienePdf" => true
    "fechaRecibido" => "2007-10-01"
    "fechaAceptado" => "2007-10-26"
    "PalabrasClave" => array:2 [
      "pt" => array:1 [
        0 => array:4 [
          "clase" => "keyword"
          "titulo" => "Palavras-chave"
          "identificador" => "xpalclavsec570415"
          "palabras" => array:3 [
            0 => "D&#233;fice de alfa-1 antitripsina"
            1 => "fen&#243;tipo Pi ZZ"
            2 => "enfisema"
          ]
        ]
      ]
      "en" => array:1 [
        0 => array:4 [
          "clase" => "keyword"
          "titulo" => "Key-words"
          "identificador" => "xpalclavsec570416"
          "palabras" => array:3 [
            0 => "Alpha-1 antitrypsin deficiency"
            1 => "phenotype Pi ZZ"
            2 => "emphysema"
          ]
        ]
      ]
    ]
    "tieneResumen" => true
    "resumen" => array:2 [
      "pt" => array:2 [
        "titulo" => "Resumo"
        "resumen" => "<span id="as0005" class="elsevierStyleSection elsevierViewall"><p id="sp0005" class="elsevierStyleSimplePara elsevierViewall">O d&#233;fice de alfa-1 antitripsina &#233; uma doen&#231;a heredit&#225;ria autoss&#243;mica codominante&#46; O fen&#243;tipo Pi ZZ est&#225; associado com maior frequ&#234;ncia &#224; doen&#231;a pulmonar&#44; sendo respons&#225;vel pelo aparecimento precoce de enfisema&#44; sobretudo nos fumadores&#46; Os autores apresentam dois casos de diagn&#243;stico tardio e em que estiveram presentes outros factores que ter&#227;o contribu&#237;do para o aparecimento de manifesta&#231;&#245;es cl&#237;nicas&#46;</p><p id="sp0010" class="elsevierStyleSimplePara elsevierViewall"><span class="elsevierStyleBold">Rev Port Pneumol 2007&#59; XIV &#40;2&#41;&#58; 295-302</span></p></span>"
      ]
      "en" => array:2 [
        "titulo" => "Abstract"
        "resumen" => "<span id="as0010" class="elsevierStyleSection elsevierViewall"><p id="sp0015" class="elsevierStyleSimplePara elsevierViewall">The alpha-1 antitrypsin deficiency is an hereditary autosomic codominant disease&#46; The phenotype Pi ZZ is associated more frequently with pulmonary disease and is responsible for the presence of emphysema early in life&#44; particularly in smokers&#46; The author&#8217;s present two cases which diagnosis were performed later in life and in which other factors could be also responsible for clinical manifestations&#46;</p><p id="sp0020" class="elsevierStyleSimplePara elsevierViewall"><span class="elsevierStyleBold">Rev Port Pneumol 2007&#59; XIV &#40;2&#41;&#58; 295-302</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:17 [
              0 => array:3 [
                "identificador" => "bb0005"
                "etiqueta" => "1&#46;"
                "referencia" => array:1 [
                  0 => array:2 [
                    "contribucion" => array:1 [
                      0 => array:2 [
                        "titulo" => "Standards for the Diagnosis and Management of Individuals with Antitrypsin Deficiency"
                        "autores" => array:1 [
                          0 => array:2 [
                            "etal" => false
                            "autores" => array:1 [
                              0 => "American Thoracic Society&#47;European Respiratory Society Statement"
                            ]
                          ]
                        ]
                      ]
                    ]
                    "host" => array:1 [
                      0 => array:2 [
                        "doi" => "10.1164/rccm.168.7.818"
                        "Revista" => array:6 [
                          "tituloSerie" => "Am J Respir Crit Care Med"
                          "fecha" => "2003"
                          "volumen" => "168"
                          "paginaInicial" => "818"
                          "paginaFinal" => "900"
                          "link" => array:1 [
                            0 => array:2 [
                              "url" => "https://www.ncbi.nlm.nih.gov/pubmed/14522813"
                              "web" => "Medline"
                            ]
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
              1 => array:3 [
                "identificador" => "bb0010"
                "etiqueta" => "2&#46;"
                "referencia" => array:1 [
                  0 => array:2 [
                    "contribucion" => array:1 [
                      0 => array:2 [
                        "titulo" => "Worldwide racial and ethnic distribution of alpha-1-antitrypsin deficiency&#58; details of an analysis of published genetic epidemiological surveys"
                        "autores" => array:1 [
                          0 => array:2 [
                            "etal" => false
                            "autores" => array:1 [
                              0 => "F&#46;J&#46; de Serres"
                            ]
                          ]
                        ]
                      ]
                    ]
                    "host" => array:1 [
                      0 => array:1 [
                        "Revista" => array:5 [
                          "tituloSerie" => "Chest"
                          "fecha" => "2002"
                          "volumen" => "122"
                          "paginaInicial" => "29"
                          "paginaFinal" => "1818"
                        ]
                      ]
                    ]
                  ]
                ]
              ]
              2 => array:3 [
                "identificador" => "bb0015"
                "etiqueta" => "3&#46;"
                "referencia" => array:1 [
                  0 => array:2 [
                    "contribucion" => array:1 [
                      0 => array:2 [
                        "titulo" => "&#945;<span class="elsevierStyleInf">1</span>-Antitrypsin deficiency in Europe&#58; geographical distribution of Pi types S and Z"
                        "autores" => array:1 [
                          0 => array:2 [
                            "etal" => false
                            "autores" => array:1 [
                              0 => "D&#46;C&#46; Hutchison"
                            ]
                          ]
                        ]
                      ]
                    ]
                    "host" => array:1 [
                      0 => array:1 [
                        "Revista" => array:5 [
                          "tituloSerie" => "Respir Med"
                          "fecha" => "1998"
                          "volumen" => "92"
                          "paginaInicial" => "77"
                          "paginaFinal" => "367"
                        ]
                      ]
                    ]
                  ]
                ]
              ]
              3 => array:3 [
                "identificador" => "bb0020"
                "etiqueta" => "4&#46;"
                "referencia" => array:1 [
                  0 => array:2 [
                    "contribucion" => array:1 [
                      0 => array:2 [
                        "titulo" => "Genetic epidemiology of alpha-1 antitrypsin deficiency&#58; France&#44; Italy&#44; Portugal and Spain"
                        "autores" => array:1 [
                          0 => array:2 [
                            "etal" => false
                            "autores" => array:3 [
                              0 => "F&#46;J&#46; de Serres"
                              1 => "I&#46; Blanco"
                              2 => "E&#46;F&#46; Bustillo"
                            ]
                          ]
                        ]
                      ]
                    ]
                    "host" => array:1 [
                      0 => array:1 [
                        "Revista" => array:6 [
                          "tituloSerie" => "Clin Genet"
                          "fecha" => "2003"
                          "volumen" => "63"
                          "paginaInicial" => "490"
                          "paginaFinal" => "509"
                          "link" => array:1 [
                            0 => array:2 [
                              "url" => "https://www.ncbi.nlm.nih.gov/pubmed/12786756"
                              "web" => "Medline"
                            ]
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
              4 => array:3 [
                "identificador" => "bb0025"
                "etiqueta" => "5&#46;"
                "referencia" => array:1 [
                  0 => array:2 [
                    "contribucion" => array:1 [
                      0 => array:2 [
                        "titulo" => "Natural history and life expectancy in severe Alpha<span class="elsevierStyleInf">1</span>-antitrypsin deficiency&#44; PiZ"
                        "autores" => array:1 [
                          0 => array:2 [
                            "etal" => false
                            "autores" => array:1 [
                              0 => "C&#46; Larsson"
                            ]
                          ]
                        ]
                      ]
                    ]
                    "host" => array:1 [
                      0 => array:1 [
                        "Revista" => array:6 [
                          "tituloSerie" => "Acta Med Scand"
                          "fecha" => "1978"
                          "volumen" => "204"
                          "paginaInicial" => "345"
                          "paginaFinal" => "351"
                          "link" => array:1 [
                            0 => array:2 [
                              "url" => "https://www.ncbi.nlm.nih.gov/pubmed/309708"
                              "web" => "Medline"
                            ]
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
              5 => array:3 [
                "identificador" => "bb0030"
                "etiqueta" => "6&#46;"
                "referencia" => array:1 [
                  0 => array:2 [
                    "contribucion" => array:1 [
                      0 => array:2 [
                        "titulo" => "Clinical features and history of the destructive lung disease associated with alpha-1 antitrypsin deficiency of adults with pulmonary symptoms"
                        "autores" => array:1 [
                          0 => array:2 [
                            "etal" => false
                            "autores" => array:6 [
                              0 => "M&#46;L&#46; Brantly"
                              1 => "L&#46;D&#46; Paul"
                              2 => "B&#46;H&#46; Miller"
                              3 => "R&#46;T&#46; Falk"
                              4 => "M&#46; Wu"
                              5 => "R&#46;G&#46; Crystal"
                            ]
                          ]
                        ]
                      ]
                    ]
                    "host" => array:1 [
                      0 => array:2 [
                        "doi" => "10.1164/ajrccm/138.2.327"
                        "Revista" => array:6 [
                          "tituloSerie" => "Am Rev Respir Dis"
                          "fecha" => "1988"
                          "volumen" => "138"
                          "paginaInicial" => "327"
                          "paginaFinal" => "336"
                          "link" => array:1 [
                            0 => array:2 [
                              "url" => "https://www.ncbi.nlm.nih.gov/pubmed/3264124"
                              "web" => "Medline"
                            ]
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
              6 => array:3 [
                "identificador" => "bb0035"
                "etiqueta" => "7&#46;"
                "referencia" => array:1 [
                  0 => array:2 [
                    "contribucion" => array:1 [
                      0 => array:2 [
                        "titulo" => "The role of genetic polymorphisms in environmental health"
                        "autores" => array:1 [
                          0 => array:2 [
                            "etal" => false
                            "autores" => array:5 [
                              0 => "S&#46;N&#46; Kelada"
                              1 => "D&#46;L&#46; Eaton"
                              2 => "S&#46;S&#46; Wang"
                              3 => "N&#46;R&#46; Rothman"
                              4 => "M&#46;J&#46; Khoury"
                            ]
                          ]
                        ]
                      ]
                    ]
                    "host" => array:1 [
                      0 => array:1 [
                        "Revista" => array:6 [
                          "tituloSerie" => "Environ Health Perspect"
                          "fecha" => "2003"
                          "volumen" => "111"
                          "paginaInicial" => "1055"
                          "paginaFinal" => "1064"
                          "link" => array:1 [
                            0 => array:2 [
                              "url" => "https://www.ncbi.nlm.nih.gov/pubmed/12826477"
                              "web" => "Medline"
                            ]
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
              7 => array:3 [
                "identificador" => "bb0040"
                "etiqueta" => "8&#46;"
                "referencia" => array:1 [
                  0 => array:2 [
                    "contribucion" => array:1 [
                      0 => array:2 [
                        "titulo" => "Atopy&#44; asthma and emphysema in patients with severe alpha-1-antitrypsin deficiency"
                        "autores" => array:1 [
                          0 => array:2 [
                            "etal" => false
                            "autores" => array:7 [
                              0 => "E&#46; Eden"
                              1 => "D&#46; Mitchell"
                              2 => "B&#46; Mehlman"
                              3 => "H&#46; Khouli"
                              4 => "M&#46; Mejat"
                              5 => "M&#46;H&#46; Grieco"
                              6 => "G&#46;M&#46; Turino"
                            ]
                          ]
                        ]
                      ]
                    ]
                    "host" => array:1 [
                      0 => array:2 [
                        "doi" => "10.1164/ajrccm.156.1.9508014"
                        "Revista" => array:6 [
                          "tituloSerie" => "Am J Respir Crit Care Med"
                          "fecha" => "1997"
                          "volumen" => "156"
                          "paginaInicial" => "68"
                          "paginaFinal" => "74"
                          "link" => array:1 [
                            0 => array:2 [
                              "url" => "https://www.ncbi.nlm.nih.gov/pubmed/9230728"
                              "web" => "Medline"
                            ]
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
              8 => array:3 [
                "identificador" => "bb0045"
                "etiqueta" => "9&#46;"
                "referencia" => array:1 [
                  0 => array:2 [
                    "contribucion" => array:1 [
                      0 => array:2 [
                        "titulo" => "RA Stockley&#46; &#945;<span class="elsevierStyleInf">1</span>-antitrypsin deficiency&#46; Clinical manifestations and natural history"
                        "autores" => array:1 [
                          0 => array:2 [
                            "etal" => false
                            "autores" => array:1 [
                              0 => "M&#46; Needhmam"
                            ]
                          ]
                        ]
                      ]
                    ]
                    "host" => array:1 [
                      0 => array:1 [
                        "Revista" => array:6 [
                          "tituloSerie" => "Thorax"
                          "fecha" => "2004"
                          "volumen" => "59"
                          "paginaInicial" => "441"
                          "paginaFinal" => "445"
                          "link" => array:1 [
                            0 => array:2 [
                              "url" => "https://www.ncbi.nlm.nih.gov/pubmed/15115878"
                              "web" => "Medline"
                            ]
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
              9 => array:3 [
                "identificador" => "bb0050"
                "etiqueta" => "10&#46;"
                "referencia" => array:1 [
                  0 => array:2 [
                    "contribucion" => array:1 [
                      0 => array:2 [
                        "titulo" => "Genetics and respiratory disease&#46; 2&#46; Alpha<span class="elsevierStyleInf">1</span>-antitrypsin deficiency&#44; cirrhosis and emphysema"
                        "autores" => array:1 [
                          0 => array:2 [
                            "etal" => false
                            "autores" => array:2 [
                              0 => "R&#46; Mahadeva"
                              1 => "D&#46;A&#46; Lomas"
                            ]
                          ]
                        ]
                      ]
                    ]
                    "host" => array:1 [
                      0 => array:1 [
                        "Revista" => array:6 [
                          "tituloSerie" => "Thorax"
                          "fecha" => "1998"
                          "volumen" => "53"
                          "paginaInicial" => "501"
                          "paginaFinal" => "505"
                          "link" => array:1 [
                            0 => array:2 [
                              "url" => "https://www.ncbi.nlm.nih.gov/pubmed/9713452"
                              "web" => "Medline"
                            ]
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
              10 => array:3 [
                "identificador" => "bb0055"
                "etiqueta" => "11&#46;"
                "referencia" => array:1 [
                  0 => array:2 [
                    "contribucion" => array:1 [
                      0 => array:2 [
                        "titulo" => "Trends in the diagnosis of symptomatic patients with &#945;<span class="elsevierStyleInf">1</span>- Antitrypsin deficiency between 1968 and 2003"
                        "autores" => array:1 [
                          0 => array:2 [
                            "etal" => false
                            "autores" => array:4 [
                              0 => "Michael A&#46; Campos"
                              1 => "Adam Wanner"
                              2 => "Guoyam Zhang"
                              3 => "Robert A&#46; Sandhaus"
                            ]
                          ]
                        ]
                      ]
                    ]
                    "host" => array:1 [
                      0 => array:1 [
                        "Revista" => array:5 [
                          "tituloSerie" => "Chest"
                          "fecha" => "2005"
                          "volumen" => "128"
                          "paginaInicial" => "86"
                          "paginaFinal" => "1179"
                        ]
                      ]
                    ]
                  ]
                ]
              ]
              11 => array:3 [
                "identificador" => "bb0060"
                "etiqueta" => "12&#46;"
                "referencia" => array:1 [
                  0 => array:2 [
                    "contribucion" => array:1 [
                      0 => array:2 [
                        "titulo" => "Delay in diagnosis of &#945;<span class="elsevierStyleInf">1</span>- Antitrypsin deficiency&#46; A continuing problem"
                        "autores" => array:1 [
                          0 => array:2 [
                            "etal" => false
                            "autores" => array:6 [
                              0 => "James K&#46; Stoller"
                              1 => "Robert A&#46; Sandhaus"
                              2 => "Gerard Turino"
                              3 => "Ryan Dickson"
                              4 => "Keith Rodgers"
                              5 => "Charlie Strange"
                            ]
                          ]
                        ]
                      ]
                    ]
                    "host" => array:1 [
                      0 => array:2 [
                        "doi" => "10.1378/chest.128.1.94"
                        "Revista" => array:6 [
                          "tituloSerie" => "Chest"
                          "fecha" => "2005"
                          "volumen" => "128"
                          "paginaInicial" => "94"
                          "paginaFinal" => "1989"
                          "link" => array:1 [
                            0 => array:2 [
                              "url" => "https://www.ncbi.nlm.nih.gov/pubmed/16002921"
                              "web" => "Medline"
                            ]
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
              12 => array:3 [
                "identificador" => "bb0065"
                "etiqueta" => "13&#46;"
                "referencia" => array:1 [
                  0 => array:2 [
                    "contribucion" => array:1 [
                      0 => array:2 [
                        "titulo" => "Survival of patients with severe &#945;<span class="elsevierStyleInf">1</span>-antitrypsin deficiency with special reference to non-index cases"
                        "autores" => array:1 [
                          0 => array:2 [
                            "etal" => false
                            "autores" => array:3 [
                              0 => "N&#46; Seersholm"
                              1 => "A&#46; Kok-Jensen"
                              2 => "A&#46; Dirksen"
                            ]
                          ]
                        ]
                      ]
                    ]
                    "host" => array:1 [
                      0 => array:1 [
                        "Revista" => array:6 [
                          "tituloSerie" => "Thorax"
                          "fecha" => "1994"
                          "volumen" => "49"
                          "paginaInicial" => "8"
                          "paginaFinal" => "695"
                          "link" => array:1 [
                            0 => array:2 [
                              "url" => "https://www.ncbi.nlm.nih.gov/pubmed/8153946"
                              "web" => "Medline"
                            ]
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
              13 => array:3 [
                "identificador" => "bb0070"
                "etiqueta" => "14&#46;"
                "referencia" => array:1 [
                  0 => array:2 [
                    "contribucion" => array:1 [
                      0 => array:2 [
                        "titulo" => "Decline in FEV1 rotated to smoking status in individuals with severe alpha1&#8211;antitrypsin deficiency &#40;PiZZ&#41;"
                        "autores" => array:1 [
                          0 => array:2 [
                            "etal" => false
                            "autores" => array:2 [
                              0 => "E&#46; Piitulainen"
                              1 => "S&#46; Eriksson"
                            ]
                          ]
                        ]
                      ]
                    ]
                    "host" => array:1 [
                      0 => array:1 [
                        "Revista" => array:5 [
                          "tituloSerie" => "Eur Respir J"
                          "fecha" => "1999"
                          "volumen" => "13"
                          "paginaInicial" => "51"
                          "paginaFinal" => "247"
                        ]
                      ]
                    ]
                  ]
                ]
              ]
              14 => array:3 [
                "identificador" => "bb0075"
                "etiqueta" => "15&#46;"
                "referencia" => array:1 [
                  0 => array:2 [
                    "contribucion" => array:1 [
                      0 => array:2 [
                        "titulo" => "Airways obstruction and two year survival in patients with severe alpha1-antitrypsin deficiency"
                        "autores" => array:1 [
                          0 => array:2 [
                            "etal" => false
                            "autores" => array:3 [
                              0 => "N&#46; Seersholm"
                              1 => "A&#46; Dirksen"
                              2 => "A&#46; Kok-Jensen"
                            ]
                          ]
                        ]
                      ]
                    ]
                    "host" => array:1 [
                      0 => array:1 [
                        "Revista" => array:5 [
                          "tituloSerie" => "Eur Respir J"
                          "fecha" => "1994"
                          "volumen" => "7"
                          "paginaInicial" => "7"
                          "paginaFinal" => "1985"
                        ]
                      ]
                    ]
                  ]
                ]
              ]
              15 => array:3 [
                "identificador" => "bb0080"
                "etiqueta" => "16&#46;"
                "referencia" => array:1 [
                  0 => array:2 [
                    "contribucion" => array:1 [
                      0 => array:2 [
                        "titulo" => "Antitrypsin and emphysema&#46; Perspective and prospects"
                        "autores" => array:1 [
                          0 => array:2 [
                            "etal" => false
                            "autores" => array:1 [
                              0 => "J&#46;A&#46; Pierce"
                            ]
                          ]
                        ]
                      ]
                    ]
                    "host" => array:1 [
                      0 => array:1 [
                        "Revista" => array:6 [
                          "tituloSerie" => "JAMA"
                          "fecha" => "1988"
                          "volumen" => "259"
                          "paginaInicial" => "5"
                          "paginaFinal" => "2890"
                          "itemHostRev" => array:3 [
                            "pii" => "S0140673601067289"
                            "estado" => "S300"
                            "issn" => "01406736"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
              16 => array:3 [
                "identificador" => "bb0085"
                "etiqueta" => "17&#46;"
                "referencia" => array:1 [
                  0 => array:1 [
                    "referenciaCompleta" => "Talecris Biotherapeutics&#44; Inc&#46;&#59; Alpha1-Proteinase Inhibitor &#40;Human&#41;&#46; Prolastin&#174;&#46; Resumo das caracter&#237;sticas do medicamento&#46;"
                  ]
                ]
              ]
            ]
          ]
        ]
      ]
    ]
  ]
  "idiomaDefecto" => "pt"
  "url" => "/08732159/0000001400000002/v1_201509151520/S0873215915302385/v1_201509151520/pt/main.assets"
  "Apartado" => array:4 [
    "identificador" => "43360"
    "tipo" => "SECCION"
    "pt" => array:2 [
      "titulo" => "Casos Cl&#237;nicos&#47;Cases Reports"
      "idiomaDefecto" => true
    ]
    "idiomaDefecto" => "pt"
  ]
  "PDF" => "https://static.elsevier.es/multimedia/08732159/0000001400000002/v1_201509151520/S0873215915302385/v1_201509151520/pt/main.pdf?idApp=UINPBA00004E&text.app=https://journalpulmonology.org/"
  "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0873215915302385?idApp=UINPBA00004E"
]
Share
Journal Information
Vol. 14. Issue 2.
Pages 295-302 (March - April 2008)
Share
Share
Download PDF
More article options
Vol. 14. Issue 2.
Pages 295-302 (March - April 2008)
Caso Clínico/Clinical Case
Open Access
Défice de alfa-1 antitripsina. A propósito de dois casos clínicos
Alpha-1 antitrypsin deficiency. Two cases report
Visits
6542
Sónia Serra1, Graça Banha2
1 Interna do Complementar de Medicina Interna, Serviço de Pneumologia, Centro Hospitalar de Setúbal, EPE, Rua Camilo Castelo Branco, 2910-446 Setúbal – Portugal
2 Assistente Hospitalar Graduada de Pneumologia, Serviço de Pneumologia, Centro Hospitalar de Setúbal, EPE, Rua Camilo Castelo Branco, 2910-446 Setúbal – Portugal
This item has received

Under a Creative Commons license
Article information
Resumo

O défice de alfa-1 antitripsina é uma doença hereditária autossómica codominante. O fenótipo Pi ZZ está associado com maior frequência à doença pulmonar, sendo responsável pelo aparecimento precoce de enfisema, sobretudo nos fumadores. Os autores apresentam dois casos de diagnóstico tardio e em que estiveram presentes outros factores que terão contribuído para o aparecimento de manifestações clínicas.

Rev Port Pneumol 2007; XIV (2): 295-302

Palavras-chave:
Défice de alfa-1 antitripsina
fenótipo Pi ZZ
enfisema
Abstract

The alpha-1 antitrypsin deficiency is an hereditary autosomic codominant disease. The phenotype Pi ZZ is associated more frequently with pulmonary disease and is responsible for the presence of emphysema early in life, particularly in smokers. The author’s present two cases which diagnosis were performed later in life and in which other factors could be also responsible for clinical manifestations.

Rev Port Pneumol 2007; XIV (2): 295-302

Key-words:
Alpha-1 antitrypsin deficiency
phenotype Pi ZZ
emphysema
Full text is only aviable in PDF
Bibliografia
[1.]
American Thoracic Society/European Respiratory Society Statement.
Standards for the Diagnosis and Management of Individuals with Antitrypsin Deficiency.
Am J Respir Crit Care Med, 168 (2003), pp. 818-900
[2.]
F.J. de Serres.
Worldwide racial and ethnic distribution of alpha-1-antitrypsin deficiency: details of an analysis of published genetic epidemiological surveys.
Chest, 122 (2002), pp. 29-1818
[3.]
D.C. Hutchison.
α1-Antitrypsin deficiency in Europe: geographical distribution of Pi types S and Z.
Respir Med, 92 (1998), pp. 77-367
[4.]
F.J. de Serres, I. Blanco, E.F. Bustillo.
Genetic epidemiology of alpha-1 antitrypsin deficiency: France, Italy, Portugal and Spain.
Clin Genet, 63 (2003), pp. 490-509
[5.]
C. Larsson.
Natural history and life expectancy in severe Alpha1-antitrypsin deficiency, PiZ.
Acta Med Scand, 204 (1978), pp. 345-351
[6.]
M.L. Brantly, L.D. Paul, B.H. Miller, R.T. Falk, M. Wu, R.G. Crystal.
Clinical features and history of the destructive lung disease associated with alpha-1 antitrypsin deficiency of adults with pulmonary symptoms.
Am Rev Respir Dis, 138 (1988), pp. 327-336
[7.]
S.N. Kelada, D.L. Eaton, S.S. Wang, N.R. Rothman, M.J. Khoury.
The role of genetic polymorphisms in environmental health.
Environ Health Perspect, 111 (2003), pp. 1055-1064
[8.]
E. Eden, D. Mitchell, B. Mehlman, H. Khouli, M. Mejat, M.H. Grieco, G.M. Turino.
Atopy, asthma and emphysema in patients with severe alpha-1-antitrypsin deficiency.
Am J Respir Crit Care Med, 156 (1997), pp. 68-74
[9.]
M. Needhmam.
RA Stockley. α1-antitrypsin deficiency. Clinical manifestations and natural history.
Thorax, 59 (2004), pp. 441-445
[10.]
R. Mahadeva, D.A. Lomas.
Genetics and respiratory disease. 2. Alpha1-antitrypsin deficiency, cirrhosis and emphysema.
Thorax, 53 (1998), pp. 501-505
[11.]
Michael A. Campos, Adam Wanner, Guoyam Zhang, Robert A. Sandhaus.
Trends in the diagnosis of symptomatic patients with α1- Antitrypsin deficiency between 1968 and 2003.
Chest, 128 (2005), pp. 86-1179
[12.]
James K. Stoller, Robert A. Sandhaus, Gerard Turino, Ryan Dickson, Keith Rodgers, Charlie Strange.
Delay in diagnosis of α1- Antitrypsin deficiency. A continuing problem.
Chest, 128 (2005), pp. 94-1989
[13.]
N. Seersholm, A. Kok-Jensen, A. Dirksen.
Survival of patients with severe α1-antitrypsin deficiency with special reference to non-index cases.
Thorax, 49 (1994), pp. 8-695
[14.]
E. Piitulainen, S. Eriksson.
Decline in FEV1 rotated to smoking status in individuals with severe alpha1–antitrypsin deficiency (PiZZ).
Eur Respir J, 13 (1999), pp. 51-247
[15.]
N. Seersholm, A. Dirksen, A. Kok-Jensen.
Airways obstruction and two year survival in patients with severe alpha1-antitrypsin deficiency.
Eur Respir J, 7 (1994), pp. 7-1985
[16.]
J.A. Pierce.
Antitrypsin and emphysema. Perspective and prospects.
JAMA, 259 (1988), pp. 5-2890
[17.]
Talecris Biotherapeutics, Inc.; Alpha1-Proteinase Inhibitor (Human). Prolastin®. Resumo das características do medicamento.
Copyright © 2008. Sociedade Portuguesa de Pneumologia/SPP
Download PDF
Pulmonology
Article options
Tools

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