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Ciliopathies" "tieneTextoCompleto" => 0 "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "149" "paginaFinal" => "159" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "Miguel Armengot Carceller, Manuel Mata Roig, Xavier Milara Payá, Julio Cortijo Gimeno" "autores" => array:4 [ 0 => array:4 [ "nombre" => "Miguel" "apellidos" => "Armengot Carceller" "email" => array:1 [ 0 => "miguel.armengot@gmail.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">¿</span>" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "Manuel" "apellidos" => "Mata Roig" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 2 => array:3 [ "nombre" => "Xavier" "apellidos" => "Milara Payá" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 3 => array:3 [ "nombre" => "Julio" "apellidos" => "Cortijo Gimeno" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0015" ] ] ] ] "afiliaciones" => array:3 [ 0 => array:3 [ "entidad" => "Servicio de Otorrinolaringología, Hospital General Universitario, Facultat de Medicina, Universitat de València, Valencia, Spain" "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Fundación para la Investigación, Hospital General Universitario, Valencia, Spain" "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Facultat de Medicina, Universitat de València, Fundación para la Investigación, Hospital General Universitario, Valencia, Spain" "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0015" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Discinesia ciliar primaria. Ciliopatías" ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2008-12-04" "fechaAceptado" => "2009-01-12" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec81288" "palabras" => array:7 [ 0 => "Kartagener's syndrome" 1 => "Sinusitis" 2 => "Bronchiectasis" 3 => "Situs inversus" 4 => "Usher's syndrome" 5 => "Retinitis pigmentosa" 6 => "Oncogenesis" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec81287" "palabras" => array:7 [ 0 => "Sindrome de Kartagener" 1 => "Sinusitis" 2 => "Bronquiectasias" 3 => "Situs inversus" 4 => "Síndrome de Usher" 5 => "Retinitis pigmentosa" 6 => "Oncogénesis" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Primary ciliary dyskinesia is a genetically inherited syndrome characterised by ciliary immotility or dysmotility. Deficiency in mucociliary clearance produces chronic respiratory infections from birth, male sterility by spermatozoid immotility and situs inversus in 40%–50% of patients (Kartagener's syndrome). Diagnosis is made by analysing ciliary motility with high-speed digital video and ciliary ultrastructure. The wide distribution and functions of the cilia in the body mean that this dysfunction can generate other ciliopathies apart from primary ciliary dyskinesia.</p>" ] "es" => array:2 [ "titulo" => "Resumen" "resumen" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">La discinesia ciliar primaria es un trastorno genéticamente determinado que se caracteriza por un movimiento ciliar alterado o ausente. Genera un déficit en el aclaramiento mucociliar que se manifiesta clínicamente como infecciones crónicas de vías aéreas constantes desde el nacimiento, así como esterilidad masculina por inmovilidad del espermatozoide y situs inversus en el 40–50% de los pacientes (síndrome de Kartagener). El diagnóstico se basa en el estudio de la movilidad ciliar mediante vídeo de alta resolución digital y alta velocidad, complementado con el estudio de la ultraestructura ciliar. La amplia distribución ciliar en el organismo y sus numerosas funciones hacen que su patología origine, además de la discinesia ciliar primaria, otras ciliopatías.</p>" ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:60 [ 0 => array:3 [ "identificador" => "bib1" "etiqueta" => "1." 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Journal Information
Primary ciliary dyskinesia. Ciliopathies
Discinesia ciliar primaria. Ciliopatías
Miguel Armengot Carcellera,
, Manuel Mata Roigb, Xavier Milara Payáb, Julio Cortijo Gimenoc
Corresponding author
a Servicio de Otorrinolaringología, Hospital General Universitario, Facultat de Medicina, Universitat de València, Valencia, Spain
b Fundación para la Investigación, Hospital General Universitario, Valencia, Spain
c Facultat de Medicina, Universitat de València, Fundación para la Investigación, Hospital General Universitario, Valencia, Spain