Statistics

 array:24 [
  "pii" => "S1579212918303951"
  "issn" => "15792129"
  "doi" => "10.1016/j.arbr.2018.03.010"
  "estado" => "S300"
  "fechaPublicacion" => "2018-12-01"
  "aid" => "1879"
  "copyright" => "SEPAR"
  "copyrightAnyo" => "2018"
  "documento" => "simple-article"
  "crossmark" => 1
  "subdocumento" => "crp"
  "cita" => "Arch Bronconeumol. 2018;54:634-5"
  "abierto" => array:3 [
    "ES" => false
    "ES2" => false
    "LATM" => false
  ]
  "gratuito" => false
  "lecturas" => array:2 [
    "total" => 382
    "formatos" => array:3 [
      "EPUB" => 78
      "HTML" => 192
      "PDF" => 112
    ]
  ]
  "Traduccion" => array:1 [
    "en" => array:19 [
      "pii" => "S0300289618301534"
      "issn" => "03002896"
      "doi" => "10.1016/j.arbres.2018.03.009"
      "estado" => "S300"
      "fechaPublicacion" => "2018-12-01"
      "aid" => "1879"
      "copyright" => "SEPAR"
      "documento" => "simple-article"
      "crossmark" => 1
      "subdocumento" => "crp"
      "cita" => "Arch Bronconeumol. 2018;54:634-5"
      "abierto" => array:3 [
        "ES" => true
        "ES2" => true
        "LATM" => true
      ]
      "gratuito" => true
      "lecturas" => array:2 [
        "total" => 543
        "formatos" => array:3 [
          "EPUB" => 66
          "HTML" => 304
          "PDF" => 173
        ]
      ]
      "en" => array:11 [
        "idiomaDefecto" => true
        "cabecera" => "<span class="elsevierStyleTextfn">Scientific Letter</span>"
        "titulo" => "ABCA3 Deficiency in a Newborn with Respiratory Failure"
        "tienePdf" => "en"
        "tieneTextoCompleto" => "en"
        "paginas" => array:1 [
          0 => array:2 [
            "paginaInicial" => "634"
            "paginaFinal" => "635"
          ]
        ]
        "titulosAlternativos" => array:1 [
          "es" => array:1 [
            "titulo" => "Deficiencia de ABCA3 en un reci&#233;n nacido con insuficiencia respiratoria"
          ]
        ]
        "contieneTextoCompleto" => array:1 [
          "en" => true
        ]
        "contienePdf" => array:1 [
          "en" => true
        ]
        "resumenGrafico" => array:2 [
          "original" => 0
          "multimedia" => array:7 [
            "identificador" => "fig0005"
            "etiqueta" => "Fig&#46; 1"
            "tipo" => "MULTIMEDIAFIGURA"
            "mostrarFloat" => true
            "mostrarDisplay" => false
            "figura" => array:1 [
              0 => array:4 [
                "imagen" => "gr1.jpeg"
                "Alto" => 459
                "Ancho" => 604
                "Tamanyo" => 25684
              ]
            ]
            "descripcion" => array:1 [
              "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">CT with bilateral granular opacities and ground-glass opacification&#46;</p>"
            ]
          ]
        ]
        "autores" => array:1 [
          0 => array:2 [
            "autoresLista" => "M&#170; Carmen L&#243;pez Castillo, Estela P&#233;rez Ruiz, Pilar Caro Aguilera, Mar&#237;a Adela Rodr&#237;guez Vives"
            "autores" => array:4 [
              0 => array:2 [
                "nombre" => "M&#170; Carmen"
                "apellidos" => "L&#243;pez Castillo"
              ]
              1 => array:2 [
                "nombre" => "Estela"
                "apellidos" => "P&#233;rez Ruiz"
              ]
              2 => array:2 [
                "nombre" => "Pilar"
                "apellidos" => "Caro Aguilera"
              ]
              3 => array:2 [
                "nombre" => "Mar&#237;a Adela"
                "apellidos" => "Rodr&#237;guez Vives"
              ]
            ]
          ]
        ]
      ]
      "idiomaDefecto" => "en"
      "Traduccion" => array:1 [
        "en" => array:9 [
          "pii" => "S1579212918303951"
          "doi" => "10.1016/j.arbr.2018.03.010"
          "estado" => "S300"
          "subdocumento" => ""
          "abierto" => array:3 [
            "ES" => false
            "ES2" => false
            "LATM" => false
          ]
          "gratuito" => false
          "lecturas" => array:1 [
            "total" => 0
          ]
          "idiomaDefecto" => "en"
          "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S1579212918303951?idApp=UINPBA00003Z"
        ]
      ]
      "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0300289618301534?idApp=UINPBA00003Z"
      "url" => "/03002896/0000005400000012/v1_201812020626/S0300289618301534/v1_201812020626/en/main.assets"
    ]
  ]
  "itemSiguiente" => array:19 [
    "pii" => "S157921291830329X"
    "issn" => "15792129"
    "doi" => "10.1016/j.arbr.2018.10.001"
    "estado" => "S300"
    "fechaPublicacion" => "2018-12-01"
    "aid" => "1881"
    "copyright" => "SEPAR"
    "documento" => "simple-article"
    "crossmark" => 1
    "subdocumento" => "crp"
    "cita" => "Arch Bronconeumol. 2018;54:635-7"
    "abierto" => array:3 [
      "ES" => false
      "ES2" => false
      "LATM" => false
    ]
    "gratuito" => false
    "lecturas" => array:2 [
      "total" => 442
      "formatos" => array:3 [
        "EPUB" => 71
        "HTML" => 226
        "PDF" => 145
      ]
    ]
    "en" => array:11 [
      "idiomaDefecto" => true
      "cabecera" => "<span class="elsevierStyleTextfn">Scientific Letter</span>"
      "titulo" => "Tuberculosis and Microscopic Polyangiitis&#46; A Rare Combination"
      "tienePdf" => "en"
      "tieneTextoCompleto" => "en"
      "paginas" => array:1 [
        0 => array:2 [
          "paginaInicial" => "635"
          "paginaFinal" => "637"
        ]
      ]
      "titulosAlternativos" => array:1 [
        "es" => array:1 [
          "titulo" => "Tuberculosis y poliangitis microsc&#243;pica&#46; Una asociaci&#243;n muy poco frecuente"
        ]
      ]
      "contieneTextoCompleto" => array:1 [
        "en" => true
      ]
      "contienePdf" => array:1 [
        "en" => true
      ]
      "resumenGrafico" => array:2 [
        "original" => 0
        "multimedia" => array:7 [
          "identificador" => "fig0005"
          "etiqueta" => "Fig&#46; 1"
          "tipo" => "MULTIMEDIAFIGURA"
          "mostrarFloat" => true
          "mostrarDisplay" => false
          "figura" => array:1 [
            0 => array:4 [
              "imagen" => "gr1.jpeg"
              "Alto" => 3684
              "Ancho" => 3000
              "Tamanyo" => 1894464
            ]
          ]
          "descripcion" => array:1 [
            "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">&#40;A&#41; Chest high-resolution computed tomography &#40;HRCT&#41;&#46; No evidence of disease or pleural effusion observed in middle and lower fields&#46; &#40;B&#41; Lung biopsy&#46; Granulomatous inflammation with extensive areas of necrosis and multinucleated Langhans giant cells&#46; &#40;C&#41; Chest HRCT&#46; Diffuse increase of pulmonary radiodensity&#44; mainly ground glass opacities with moderate left loculated pleural effusion&#44; and a fissural component&#44; interpreted as diffuse alveolar hemorrhage&#46; &#40;D&#41; Renal biopsy&#46; Renal glomerulus with area of focal necrosis and karyorrhexis&#46; &#40;E&#41; Renal biopsy with fibrinoid necrosis of the small arteries affecting more than 50&#37; of the circumference&#44; associated with transmural inflammation&#46; &#40;F&#41; Chest HRCT&#46; Reduction of pleural effusion and slight improvement of the ground glass opacities&#46;</p>"
          ]
        ]
      ]
      "autores" => array:1 [
        0 => array:2 [
          "autoresLista" => "Vanessa Riveiro, Jorge Ricoy, Mar&#237;a E&#46; Toubes, Luis Vald&#233;s"
          "autores" => array:4 [
            0 => array:2 [
              "nombre" => "Vanessa"
              "apellidos" => "Riveiro"
            ]
            1 => array:2 [
              "nombre" => "Jorge"
              "apellidos" => "Ricoy"
            ]
            2 => array:2 [
              "nombre" => "Mar&#237;a E&#46;"
              "apellidos" => "Toubes"
            ]
            3 => array:2 [
              "nombre" => "Luis"
              "apellidos" => "Vald&#233;s"
            ]
          ]
        ]
      ]
    ]
    "idiomaDefecto" => "en"
    "Traduccion" => array:1 [
      "es" => array:9 [
        "pii" => "S0300289618301558"
        "doi" => "10.1016/j.arbres.2018.03.011"
        "estado" => "S300"
        "subdocumento" => ""
        "abierto" => array:3 [
          "ES" => true
          "ES2" => true
          "LATM" => true
        ]
        "gratuito" => true
        "lecturas" => array:1 [
          "total" => 0
        ]
        "idiomaDefecto" => "es"
        "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0300289618301558?idApp=UINPBA00003Z"
      ]
    ]
    "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S157921291830329X?idApp=UINPBA00003Z"
    "url" => "/15792129/0000005400000012/v1_201812020625/S157921291830329X/v1_201812020625/en/main.assets"
  ]
  "itemAnterior" => array:19 [
    "pii" => "S157921291830394X"
    "issn" => "15792129"
    "doi" => "10.1016/j.arbr.2018.02.031"
    "estado" => "S300"
    "fechaPublicacion" => "2018-12-01"
    "aid" => "1878"
    "copyright" => "SEPAR"
    "documento" => "simple-article"
    "crossmark" => 1
    "subdocumento" => "crp"
    "cita" => "Arch Bronconeumol. 2018;54:632-3"
    "abierto" => array:3 [
      "ES" => false
      "ES2" => false
      "LATM" => false
    ]
    "gratuito" => false
    "lecturas" => array:2 [
      "total" => 453
      "formatos" => array:3 [
        "EPUB" => 61
        "HTML" => 255
        "PDF" => 137
      ]
    ]
    "en" => array:11 [
      "idiomaDefecto" => true
      "cabecera" => "<span class="elsevierStyleTextfn">Scientific Letter</span>"
      "titulo" => "Pulmonary Foreign Body Granulomatosis 11 Years After Injection of a Cosmetic Dermal Filler"
      "tienePdf" => "en"
      "tieneTextoCompleto" => "en"
      "paginas" => array:1 [
        0 => array:2 [
          "paginaInicial" => "632"
          "paginaFinal" => "633"
        ]
      ]
      "titulosAlternativos" => array:1 [
        "es" => array:1 [
          "titulo" => "Granulomatosis Pulmonar De Cuerpo Extra&#241;o 11 A&#241;os Despu&#233;s De La Inyecci&#243;n De Un Relleno D&#233;rmico Cosm&#233;tico"
        ]
      ]
      "contieneTextoCompleto" => array:1 [
        "en" => true
      ]
      "contienePdf" => array:1 [
        "en" => true
      ]
      "resumenGrafico" => array:2 [
        "original" => 0
        "multimedia" => array:7 [
          "identificador" => "fig0005"
          "etiqueta" => "Fig&#46; 1"
          "tipo" => "MULTIMEDIAFIGURA"
          "mostrarFloat" => true
          "mostrarDisplay" => false
          "figura" => array:1 [
            0 => array:4 [
              "imagen" => "gr1.jpeg"
              "Alto" => 907
              "Ancho" => 1200
              "Tamanyo" => 243911
            ]
          ]
          "descripcion" => array:1 [
            "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">&#40;A&#41; Patient with erythema and edema at the inter-ciliary region and naso-genian sulc&#59; &#40;B&#41; the skin biopsy revealed a deep and severe inflammatory process&#44; depicting lipogranulomas with multinucleated giant cells of the foreign body type &#40;H&#38;E&#44; 40&#215; and 200&#215;&#41;&#59; &#40;C&#41; HRCT-L showing a micronodular milliary pattern of the upper lobes&#44; ground glass areas and slight thickening of interlobular septae&#59; &#40;D&#41; microphotograph of the lung biopsy showing sarcoid-like epithelioid granulomas with multinucleated giant cells of the foreign body type &#40;H&#38;E&#44; 200&#215;&#41;&#46;</p>"
          ]
        ]
      ]
      "autores" => array:1 [
        0 => array:2 [
          "autoresLista" => "Francisca Teixeira-Lopes, Ana Dias, Rita Lu&#237;s, Leonardo Ferreira"
          "autores" => array:4 [
            0 => array:2 [
              "nombre" => "Francisca"
              "apellidos" => "Teixeira-Lopes"
            ]
            1 => array:2 [
              "nombre" => "Ana"
              "apellidos" => "Dias"
            ]
            2 => array:2 [
              "nombre" => "Rita"
              "apellidos" => "Lu&#237;s"
            ]
            3 => array:2 [
              "nombre" => "Leonardo"
              "apellidos" => "Ferreira"
            ]
          ]
        ]
      ]
    ]
    "idiomaDefecto" => "en"
    "Traduccion" => array:1 [
      "en" => array:9 [
        "pii" => "S0300289618301522"
        "doi" => "10.1016/j.arbres.2018.02.024"
        "estado" => "S300"
        "subdocumento" => ""
        "abierto" => array:3 [
          "ES" => true
          "ES2" => true
          "LATM" => true
        ]
        "gratuito" => true
        "lecturas" => array:1 [
          "total" => 0
        ]
        "idiomaDefecto" => "en"
        "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0300289618301522?idApp=UINPBA00003Z"
      ]
    ]
    "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S157921291830394X?idApp=UINPBA00003Z"
    "url" => "/15792129/0000005400000012/v1_201812020625/S157921291830394X/v1_201812020625/en/main.assets"
  ]
  "en" => array:14 [
    "idiomaDefecto" => true
    "cabecera" => "<span class="elsevierStyleTextfn">Scientific Letter</span>"
    "titulo" => "ABCA3 Deficiency in a Newborn with Respiratory Failure"
    "tieneTextoCompleto" => true
    "saludo" => "Dear Editor&#44;"
    "paginas" => array:1 [
      0 => array:2 [
        "paginaInicial" => "634"
        "paginaFinal" => "635"
      ]
    ]
    "autores" => array:1 [
      0 => array:4 [
        "autoresLista" => "M&#170; Carmen L&#243;pez Castillo, Estela P&#233;rez Ruiz, Pilar Caro Aguilera, Mar&#237;a Adela Rodr&#237;guez Vives"
        "autores" => array:4 [
          0 => array:4 [
            "nombre" => "M&#170; Carmen"
            "apellidos" => "L&#243;pez Castillo"
            "email" => array:1 [
              0 => "mcarmen&#46;lopez123&#64;gmail&#46;com"
            ]
            "referencia" => array:2 [
              0 => array:2 [
                "etiqueta" => "<span class="elsevierStyleSup">a</span>"
                "identificador" => "aff0005"
              ]
              1 => array:2 [
                "etiqueta" => "<span class="elsevierStyleSup">&#42;</span>"
                "identificador" => "cor0005"
              ]
            ]
          ]
          1 => array:3 [
            "nombre" => "Estela"
            "apellidos" => "P&#233;rez Ruiz"
            "referencia" => array:1 [
              0 => array:2 [
                "etiqueta" => "<span class="elsevierStyleSup">a</span>"
                "identificador" => "aff0005"
              ]
            ]
          ]
          2 => array:3 [
            "nombre" => "Pilar"
            "apellidos" => "Caro Aguilera"
            "referencia" => array:1 [
              0 => array:2 [
                "etiqueta" => "<span class="elsevierStyleSup">a</span>"
                "identificador" => "aff0005"
              ]
            ]
          ]
          3 => array:3 [
            "nombre" => "Mar&#237;a Adela"
            "apellidos" => "Rodr&#237;guez Vives"
            "referencia" => array:1 [
              0 => array:2 [
                "etiqueta" => "<span class="elsevierStyleSup">b</span>"
                "identificador" => "aff0010"
              ]
            ]
          ]
        ]
        "afiliaciones" => array:2 [
          0 => array:3 [
            "entidad" => "Hospital Materno Infantil de M&#225;laga&#44; Spain"
            "etiqueta" => "a"
            "identificador" => "aff0005"
          ]
          1 => array:3 [
            "entidad" => "Facultad de Medicina de M&#225;laga&#44; Hospital Materno Infantil de M&#225;laga&#44; Spain"
            "etiqueta" => "b"
            "identificador" => "aff0010"
          ]
        ]
        "correspondencia" => array:1 [
          0 => array:3 [
            "identificador" => "cor0005"
            "etiqueta" => "&#8270;"
            "correspondencia" => "Corresponding author&#46;"
          ]
        ]
      ]
    ]
    "titulosAlternativos" => array:1 [
      "es" => array:1 [
        "titulo" => "Deficiencia de ABCA3 en un reci&#233;n nacido con insuficiencia respiratoria"
      ]
    ]
    "resumenGrafico" => array:2 [
      "original" => 0
      "multimedia" => array:7 [
        "identificador" => "fig0005"
        "etiqueta" => "Fig&#46; 1"
        "tipo" => "MULTIMEDIAFIGURA"
        "mostrarFloat" => true
        "mostrarDisplay" => false
        "figura" => array:1 [
          0 => array:4 [
            "imagen" => "gr1.jpeg"
            "Alto" => 459
            "Ancho" => 604
            "Tamanyo" => 25684
          ]
        ]
        "descripcion" => array:1 [
          "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">CT with bilateral granular opacities and ground-glass opacification&#46;</p>"
        ]
      ]
    ]
    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Congenital surfactant deficiencies are rare conditions&#44; including mutation in the surfactant protein B &#40;SP-B&#41;&#44; surfactant protein C &#40;SP-C&#41; and ABCA3 &#40;ATP-binding cassette member A3&#41; genes&#46; They may present with respiratory failure and pulmonary hypertension &#40;PH&#41; in the newborn&#46; Long-term outcomes are different according to the mutations&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">We present an infrequent case&#44; diagnosed in a tertiary hospital&#44; who has survived&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">A term female Arabian infant was born via spontaneous vaginal delivery&#46; Mother and father were consanguineous&#46; Immediately after birth&#44; the infant developed respiratory distress and was initially managed with continuous positive airway pressure&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">Her physical examination was notable for bilateral coarse breath sounds and generalized thoracic retractions&#46; Chest radiograph demonstrated diffuse bilateral granular opacities&#46; An echocardiogram revealed no evidence of anatomic heart disease with suprasystemic levels of pulmonary artery pressure&#46; Over the next days&#44; her gas exchange worsened&#44; needing intubation and mechanical ventilation&#46; She developed progressive hypoxic respiratory failure that needed high frequency oscillatory ventilation&#44; and nitric oxide administration&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">The infant was treated with antibiotics but infectious causes for PH were ruled out with negative blood cultures&#46; Chest computer tomography &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41; at 15 days of life showed bilateral granular opacities and ground-glass opacification&#59; two doses of surfactant were administered without improvement&#46; Bronchoscopic bronchoalveolar lavage detected PAS positive material&#46; With this information&#44; a lung biopsy trough video thoracoscopy was performed&#46; There were marked alveolar epithelial hyperplasia and mild widening of alveolar walls and the suspicion of a genetic disorder of surfactant dysfunction was considered&#46; She still needed mechanical ventilation and take away a treatment with monthly high intravenous doses of methylprednisolone in association with oral daily hydroxychloroquine and every other day azithromycin&#46; Genetic testing showed a nonsense mutation in ABCA3 gene&#44; c&#46;4681C&#62;T or p&#46;R1561X&#46; This mutation was present on both maternal and paternal alleles&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">At 7 months of age the infant was transferred to a pediatric lung transplant unit where she underwent bilateral lung transplantation at 10 months of age&#46; Currently she is 2 years old needing home mechanical ventilation support because of tracheal and right main bronchus malacia&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">Interstitial lung diseases &#40;ILD&#41; are a heterogeneous group of pathological processes that affect pulmonary parenchyma and&#44; in most cases&#44; lead to an impairment of gas transfer and reduction of the lung capacity&#46; There are no reliable estimates&#44; but prevalence is likely &#60;1 per 100<span class="elsevierStyleHsp" style=""></span>000&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">1</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">The definition requires at least three of the four following criteria in the absence of other know lung disorders&#58; &#40;1&#41; respiratory symptoms &#40;cough&#44; rapid and&#47;or difficult breathing&#44; or exercise intolerance&#41;&#44; &#40;2&#41; signs &#40;resting tachypnea&#44; adventitious sounds&#44; retractions&#44; digital clubbing&#44; failure to thrive&#44; or respiratory failure&#41;&#44; &#40;3&#41; hypoxemia&#44; and &#40;4&#41; diffuse abnormalities on chest X-ray or CT scan&#46; Thus&#44; establishing 3 of 4 criteria is a sensitivity method for recognizing patients that could benefit from and ILD evaluation&#46;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">2</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">The earliest presentation of ILD is shortly after birth&#44; with unexplained respiratory distress in a term neonate&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">An organized classification scheme for ILD in children less was published by the chILD Research Network<a class="elsevierStyleCrossRefs" href="#bib0075"><span class="elsevierStyleSup">3&#8211;5</span></a> &#40;<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>&#41;&#46;</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0055" class="elsevierStylePara elsevierViewall">Given the non-specific presentation of ILD&#44; difficulty is frequently experienced in discerning ILD&#46; Excluding these conditions prior to proceeding to more invasive test is important<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">1&#44;2&#44;6</span></a>&#58;<ul class="elsevierStyleList" id="lis0005"><li class="elsevierStyleListItem" id="lsti0005"><span class="elsevierStyleLabel">-</span><p id="par0060" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleBold">Infection screen</span>&#46;</p></li><li class="elsevierStyleListItem" id="lsti0010"><span class="elsevierStyleLabel">-</span><p id="par0065" class="elsevierStylePara elsevierViewall">An <span class="elsevierStyleBold">echocardiography</span> to rule out structural cardiovascular disease and pulmonary hypertension&#46;</p></li><li class="elsevierStyleListItem" id="lsti0015"><span class="elsevierStyleLabel">-</span><p id="par0070" class="elsevierStylePara elsevierViewall">Baseline <span class="elsevierStyleBold">chest X-ray</span>&#46;</p></li><li class="elsevierStyleListItem" id="lsti0020"><span class="elsevierStyleLabel">-</span><p id="par0075" class="elsevierStylePara elsevierViewall">Thin-<span class="elsevierStyleBold">section CT</span> scanning&#46; Ground glass opacification and air trapping are classical features detected&#46;</p></li><li class="elsevierStyleListItem" id="lsti0025"><span class="elsevierStyleLabel">-</span><p id="par0080" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleBold">Flexible bronchoscopy with BAL</span> to exclude infection or airway abnormalities&#46;</p></li><li class="elsevierStyleListItem" id="lsti0030"><span class="elsevierStyleLabel">-</span><p id="par0085" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleBold">Genetic testing</span>&#46; Surfactant protein mutations produce recognizable clinical phenotypes of varying severity&#46;</p></li><li class="elsevierStyleListItem" id="lsti0035"><span class="elsevierStyleLabel">-</span><p id="par0090" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleBold">Lung biopsy</span> is the gold standard&#46;</p></li></ul></p><p id="par0095" class="elsevierStylePara elsevierViewall">The gene for ABCA3 is expressed in alveolar type II cells&#44; and the protein is localized to lamellar bodies&#46; ABCA3 mutations have been associated with lethal neonatal respiratory distress and surfactant metabolism dysfunction&#46; Outcomes in patients with ABCA3 mutations are variable&#44; ranging from severe irreversible respiratory failure in early infancy to chronic static or progressive ILD&#46;<a class="elsevierStyleCrossRefs" href="#bib0095"><span class="elsevierStyleSup">7&#44;8</span></a></p><p id="par0100" class="elsevierStylePara elsevierViewall">There have been no controlled trials of therapeutic interventions in ILD syndrome&#46; Case reports of improvement have been recorded with use of glucocorticoids&#44; hydroxychloroquine&#44; azathioprine&#44; bronchodilators&#44; mycophenolate&#44; and other immune modulators&#46;<a class="elsevierStyleCrossRefs" href="#bib0105"><span class="elsevierStyleSup">9&#44;10</span></a> Lung transplantation is an option in end-stage lung disease&#46;<a class="elsevierStyleCrossRef" href="#bib0115"><span class="elsevierStyleSup">11</span></a></p><p id="par0105" class="elsevierStylePara elsevierViewall">The North American Children study<a class="elsevierStyleCrossRef" href="#bib0120"><span class="elsevierStyleSup">12</span></a> found a mortality rate of 30&#37;&#44; with 50&#37; of patients experiencing on-going morbidity&#46; It has become clear that some ILD entities are associated with very high mortality&#44; whereas others have a favorable outcome&#46;</p></span>"
    "pdfFichero" => "main.pdf"
    "tienePdf" => true
    "multimedia" => array:2 [
      0 => array:7 [
        "identificador" => "fig0005"
        "etiqueta" => "Fig&#46; 1"
        "tipo" => "MULTIMEDIAFIGURA"
        "mostrarFloat" => true
        "mostrarDisplay" => false
        "figura" => array:1 [
          0 => array:4 [
            "imagen" => "gr1.jpeg"
            "Alto" => 459
            "Ancho" => 604
            "Tamanyo" => 25684
          ]
        ]
        "descripcion" => array:1 [
          "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">CT with bilateral granular opacities and ground-glass opacification&#46;</p>"
        ]
      ]
      1 => array:8 [
        "identificador" => "tbl0005"
        "etiqueta" => "Table 1"
        "tipo" => "MULTIMEDIATABLA"
        "mostrarFloat" => true
        "mostrarDisplay" => false
        "detalles" => array:1 [
          0 => array:3 [
            "identificador" => "at1"
            "detalle" => "Table "
            "rol" => "short"
          ]
        ]
        "tabla" => array:1 [
          "tablatextoimagen" => array:1 [
            0 => array:2 [
              "tabla" => array:1 [
                0 => """
                  <table border="0" frame="\n
                  \t\t\t\t\tvoid\n
                  \t\t\t\t" class=""><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Diffuse developmental disorders&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Acinar dysplasia&#44; congenital alveolar dysplasia&#44; alveolar-capillary dysplasia&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Growth abnormalities&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Pulmonary hypoplasia&#44; structural pulmonary changes with chromosomal abnormalities&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Specific conditions of undefined etiology&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Pulmonary interstitial glycogenosis&#44; neuroendocrine cell hyperplasia of infancy&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Surfactant dysfunction mutations&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">SPFTB&#44; SPFTC&#44; ABCA3 genetic mutations&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Disorders of the normal host&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Infectious processes&#44; environmental agents&#44; aspiration syndromes&#44; eosinophilic pneumonia&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Disorders related to systemic disease processes&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Immune-related disorders&#44; storage disease&#44; Langerhans cell histiocytosis&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Disorders masquerading as interstitial disease&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">Arterial hypertensive vasculopathy&#44; congestive vasculopathy&#44; lymphatic disorders&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
                  """
              ]
              "imagenFichero" => array:1 [
                0 => "xTab1912000.png"
              ]
            ]
          ]
        ]
        "descripcion" => array:1 [
          "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Classification for ILD in Children&#46;</p>"
        ]
      ]
    ]
    "bibliografia" => array:2 [
      "titulo" => "References"
      "seccion" => array:1 [
        0 => array:2 [
          "identificador" => "bibs0015"
          "bibliografiaReferencia" => array:12 [
            0 => array:3 [
              "identificador" => "bib0065"
              "etiqueta" => "1"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "European protocols for the diagnosis and initial treatment of interstitial lung disease in children"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "A&#46; Bush"
                            1 => "S&#46; Cunningham"
                            2 => "J&#46; Blic"
                            3 => "A&#46; Barbato"
                            4 => "A&#46; Clement"
                            5 => "R&#46; Epaud"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:5 [
                        "tituloSerie" => "Thorax"
                        "fecha" => "2015"
                        "volumen" => "70"
                        "paginaInicial" => "1078"
                        "paginaFinal" => "1084"
                      ]
                    ]
                  ]
                ]
              ]
            ]
            1 => array:3 [
              "identificador" => "bib0070"
              "etiqueta" => "2"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "An official American thoracic society clinical practice guideline&#58; classification&#44; evaluation&#44; and management of childhood interstitial lung disease in infancy"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "G&#46; Kurland"
                            1 => "R&#46;R&#46; Deterding"
                            2 => "J&#46;S&#46; Hagood"
                            3 => "L&#46;R&#46; Young"
                            4 => "A&#46;S&#46; Brody"
                            5 => "R&#46;G&#46; Castile"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:6 [
                        "tituloSerie" => "Am J Respir Crit Care Med"
                        "fecha" => "2013"
                        "volumen" => "188"
                        "paginaInicial" => "376"
                        "paginaFinal" => "394"
                        "itemHostRev" => array:3 [
                          "pii" => "S0140673600033171"
                          "estado" => "S300"
                          "issn" => "01406736"
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            2 => array:3 [
              "identificador" => "bib0075"
              "etiqueta" => "3"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Defining the spectrum of diffuse lung disease in infancy&#58; a working classification of the pediatric interstitial lung disease cooperative"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "G&#46;H&#46; Deutsch"
                            1 => "E&#46; Albright"
                            2 => "P&#46;M&#46; Chou"
                            3 => "C&#46;D&#46; Cool"
                            4 => "S&#46; Coventry"
                            5 => "M&#46;M&#46; Davis"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:4 [
                        "tituloSerie" => "Mod Pathol"
                        "fecha" => "2005"
                        "volumen" => "18"
                        "paginaInicial" => "304"
                      ]
                    ]
                  ]
                ]
              ]
            ]
            3 => array:3 [
              "identificador" => "bib0080"
              "etiqueta" => "4"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Infant lung biopsy&#58; clarifying the pathologic spectrum"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:2 [
                            0 => "C&#46; Langston"
                            1 => "M&#46; Dishop"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:6 [
                        "tituloSerie" => "Pathol Int"
                        "fecha" => "2004"
                        "volumen" => "54"
                        "paginaInicial" => "S419"
                        "paginaFinal" => "S421"
                        "itemHostRev" => array:3 [
                          "pii" => "S0140673600045712"
                          "estado" => "S300"
                          "issn" => "01406736"
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            4 => array:3 [
              "identificador" => "bib0085"
              "etiqueta" => "5"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "ChILD Research Co-operative Diffuse lung disease in young children&#58; application of a novel classification scheme"
                      "autores" => array:1 [
                        0 => array:3 [
                          "colaboracion" => "Pathology Cooperative Group"
                          "etal" => true
                          "autores" => array:6 [
                            0 => "G&#46;H&#46; Deutsch"
                            1 => "L&#46;R&#46; Young"
                            2 => "R&#46;R&#46; Deterding"
                            3 => "L&#46;L&#46; Fan"
                            4 => "S&#46;D&#46; Dell"
                            5 => "J&#46;A&#46; Bean"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:5 [
                        "tituloSerie" => "Am J Respir Crit Care Med"
                        "fecha" => "2007"
                        "volumen" => "176"
                        "paginaInicial" => "1120"
                        "paginaFinal" => "1128"
                      ]
                    ]
                  ]
                ]
              ]
            ]
            5 => array:3 [
              "identificador" => "bib0090"
              "etiqueta" => "6"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Interstitial lung disease in infancy&#58; a general approach"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:5 [
                            0 => "E&#46; Hines"
                            1 => "M&#46; Walsh"
                            2 => "J&#46; Armes"
                            3 => "T&#46; Douglas"
                            4 => "J&#46; Chawla"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:6 [
                        "tituloSerie" => "J Paediatr Child Health"
                        "fecha" => "2016"
                        "volumen" => "52"
                        "paginaInicial" => "370"
                        "paginaFinal" => "376"
                        "itemHostRev" => array:3 [
                          "pii" => "S0140673601063905"
                          "estado" => "S300"
                          "issn" => "01406736"
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            6 => array:3 [
              "identificador" => "bib0095"
              "etiqueta" => "7"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Clinical&#44; radiological and pathological features of ABCA3 mutations in children"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "M&#46;L&#46; Doan"
                            1 => "R&#46;P&#46; Guillerman"
                            2 => "M&#46;K&#46; Dishop"
                            3 => "L&#46;M&#46; Nogee"
                            4 => "C&#46; Langston"
                            5 => "G&#46;B&#46; Mallory"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:5 [
                        "tituloSerie" => "Thorax"
                        "fecha" => "2008"
                        "volumen" => "63"
                        "paginaInicial" => "366"
                        "paginaFinal" => "373"
                      ]
                    ]
                  ]
                ]
              ]
            ]
            7 => array:3 [
              "identificador" => "bib0100"
              "etiqueta" => "8"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Unexplained neonatal respiratory distress due to congenital surfactant deficiency"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "M&#46; Somaschini"
                            1 => "L&#46; Nogee"
                            2 => "I&#46; Sassi"
                            3 => "O&#46; Danhaive"
                            4 => "S&#46; Presi"
                            5 => "R&#46; Boldrini"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:5 [
                        "tituloSerie" => "J Pediatr"
                        "fecha" => "2007"
                        "volumen" => "150"
                        "paginaInicial" => "537"
                        "paginaFinal" => "649"
                      ]
                    ]
                  ]
                ]
              ]
            ]
            8 => array:3 [
              "identificador" => "bib0105"
              "etiqueta" => "9"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "ABCA3 gene mutations in newborns with fatal surfactant deficiency"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:6 [
                            0 => "Shulenin"
                            1 => "L&#46;M&#46; Nogee"
                            2 => "T&#46; Annilo"
                            3 => "S&#46;S&#46;E&#46; Wert"
                            4 => "J&#46;A&#46; Whitsett"
                            5 => "M&#46; Dean"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:5 [
                        "tituloSerie" => "N Engl J Med"
                        "fecha" => "2004"
                        "volumen" => "350"
                        "paginaInicial" => "1296"
                        "paginaFinal" => "1303"
                      ]
                    ]
                  ]
                ]
              ]
            ]
            9 => array:3 [
              "identificador" => "bib0110"
              "etiqueta" => "10"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "ABCA3 mutations associated with pediatric intertitial lung disease"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:5 [
                            0 => "J&#46;E&#46; Bullard"
                            1 => "S&#46;E&#46; Wert"
                            2 => "J&#46;A&#46; Whitsett"
                            3 => "M&#46; Dean"
                            4 => "L&#46;M&#46; Nogee"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:5 [
                        "tituloSerie" => "Am J Respir Crit Care Med"
                        "fecha" => "2005"
                        "volumen" => "172"
                        "paginaInicial" => "1026"
                        "paginaFinal" => "1031"
                      ]
                    ]
                  ]
                ]
              ]
            ]
            10 => array:3 [
              "identificador" => "bib0115"
              "etiqueta" => "11"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Lung transplantation in young infants with interstitial pneumonia"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "A&#46; Moreno"
                            1 => "J&#46; Maestre"
                            2 => "J&#46; Balcells"
                            3 => "C&#46; Marhuenda"
                            4 => "N&#46; Cobos"
                            5 => "A&#46; Roman"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:5 [
                        "tituloSerie" => "Transpl Proc"
                        "fecha" => "2003"
                        "volumen" => "35"
                        "paginaInicial" => "1951"
                        "paginaFinal" => "1953"
                      ]
                    ]
                  ]
                ]
              ]
            ]
            11 => array:3 [
              "identificador" => "bib0120"
              "etiqueta" => "12"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Lung disease caused by ABCA3 mutations"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "C&#46; Kr&#246;ner"
                            1 => "T&#46; Wittmann"
                            2 => "S&#46; Reu"
                            3 => "V&#46; Teusch"
                            4 => "M&#46; Klemme"
                            5 => "D&#46; Rauch"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:5 [
                        "tituloSerie" => "Thorax"
                        "fecha" => "2017"
                        "volumen" => "72"
                        "paginaInicial" => "213"
                        "paginaFinal" => "220"
                      ]
                    ]
                  ]
                ]
              ]
            ]
          ]
        ]
      ]
    ]
  ]
  "idiomaDefecto" => "en"
  "url" => "/15792129/0000005400000012/v1_201812020625/S1579212918303951/v1_201812020625/en/main.assets"
  "Apartado" => array:4 [
    "identificador" => "49861"
    "tipo" => "SECCION"
    "en" => array:2 [
      "titulo" => "Scientific letters"
      "idiomaDefecto" => true
    ]
    "idiomaDefecto" => "en"
  ]
  "PDF" => "https://static.elsevier.es/multimedia/15792129/0000005400000012/v1_201812020625/S1579212918303951/v1_201812020625/en/main.pdf?idApp=UINPBA00003Z&text.app=https://archbronconeumol.org/"
  "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S1579212918303951?idApp=UINPBA00003Z"
]