Caso Clínico/Case Report
Um caso raro de discinesia ciliar primária associada a heterotaxia
A rare case of primary ciliary dyskinesia with heterotaxy
1 Interna do Internato Complementar de Gastrenterologia, Centro Hospitalar de Lisboa Central – Hospital Santo António dos Capuchos, Lisboa.
2 Interna do Internato Complementar de Medicina Interna, Centro Hospitalar de Lisboa Central – Hospital Santo António dos Capuchos, Lisboa.
3 Assistente Graduada de Gastrenterologia, Centro Hospitalar de Lisboa Central – Hospital Santo António dos Capuchos, Lisboa.
4 Assistente de Medicina Interna, Centro Hospitalar de Lisboa Central – Hospital Santo António dos Capuchos, Lisboa.
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"etiqueta" => "1" "identificador" => "af0005" ] 1 => array:3 [ "entidad" => "Interna do Internato Complementar de Medicina Interna, Centro Hospitalar de Lisboa Central – Hospital Santo António dos Capuchos, Lisboa." "etiqueta" => "2" "identificador" => "af0010" ] 2 => array:3 [ "entidad" => "Assistente Graduada de Gastrenterologia, Centro Hospitalar de Lisboa Central – Hospital Santo António dos Capuchos, Lisboa." "etiqueta" => "3" "identificador" => "af0015" ] 3 => array:3 [ "entidad" => "Assistente de Medicina Interna, Centro Hospitalar de Lisboa Central – Hospital Santo António dos Capuchos, Lisboa." "etiqueta" => "4" "identificador" => "af0020" ] ] ] ] "titulosAlternativos" => array:1 [ "en" => array:1 [ "titulo" => "A rare case of primary ciliary dyskinesia with heterotaxy" ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2008-07-01" "fechaAceptado" => "2008-10-01" "PalabrasClave" => array:2 [ "pt" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palavras-chave" "identificador" => "xpalclavsec570511" "palabras" => array:2 [ 0 => "Discinesia ciliar primária" 1 => "heterotaxia" ] ] ] "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Key-words" "identificador" => "xpalclavsec570510" "palabras" => array:2 [ 0 => "Primary ciliary dyskinesia" 1 => "heterotaxy" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "pt" => array:2 [ "titulo" => "Resumo" "resumen" => "<span id="as0005" class="elsevierStyleSection elsevierViewall"><p id="sp0005" class="elsevierStyleSimplePara elsevierViewall">A discinesia ciliar primária é uma doença autossómica recessiva caracterizada pela história de infecções de repetição do aparelho respiratório superior e inferior, rinossinusite e bronquite associada a <span class="elsevierStyleItalic">situs inversus</span> completo ou parcial. Os autores apresentam um doente de 78 anos, eurocaucasiano, com rinossinusites, bronquite crónica e dispneia, otite média com défices auditivos, infertilidade, seguido em consulta de gastrenterologia por dispepsia e obstipação há vários anos. Realizou vários exames que mostraram: agenesia frontal direita, espessamento brônquico, bronquiectasias, cego e cólon ascendente localizados na fossa ilíaca esquerda. Excluiu-se imunodeficiência, alergias, fibrose quística e outros. No decurso da investigação concluímos que se tratava de um caso de discinesia ciliar primária. Pela raridade deste caso, decidimos apresentá-lo.</p><p id="sp0010" class="elsevierStyleSimplePara elsevierViewall"><span class="elsevierStyleBold">Rev Port Pneumol 2009; XV (1): 115-120</span></p></span>" ] "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<span id="as0010" class="elsevierStyleSection elsevierViewall"><p id="sp0015" class="elsevierStyleSimplePara elsevierViewall">Primary ciliary dyskinesia is an autosomal recessive disease with a clinical history of upper and lowers respiratory infections, rhinosinusitis and bronquitis associated with complete or partial situs inversus. The authors present a 78-year-old male caucasian patient with rhinosinusitis, lower respiratory tract infection and dyspnea, chronic otitis with hearing deficit and infertility followed in Gastroenterology for dyspepsia and constipation. The radiological studies revealed agenesis of right frontal sinus; bronchial wall thickening; bronchiectasis; cecum and ascending colon located on the left and small bowel occupies right side of abdomen. He had no immunodeficiency, allergies, cystic fibrosis and others. We concluded primary ciliary dyskinesia with heterotaxy. For the rarity of this case we decided to present it.</p><p id="sp0020" class="elsevierStyleSimplePara elsevierViewall"><span class="elsevierStyleBold">Rev Port Pneumol 2009; XV (1): 115-120</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:11 [ 0 => array:3 [ "identificador" => "bb0005" "etiqueta" => "1." 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Mauviel" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:6 [ "tituloSerie" => "West J Med" "fecha" => "1991" "volumen" => "155" "paginaInicial" => "280" "paginaFinal" => "283" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/1949776" "web" => "Medline" ] ] ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bb0015" "etiqueta" => "3." "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Heterotaxia, congenital heart disease, and primary ciliary diskinesia" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:1 [ 0 => "M. 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Hariri" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1136/adc.2006.096958" "Revista" => array:6 [ "tituloSerie" => "Arch Dis Child" "fecha" => "2007" "volumen" => "92" "paginaInicial" => "1136" "paginaFinal" => "1140" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/17634184" "web" => "Medline" ] ] ] ] ] ] ] ] 4 => array:3 [ "identificador" => "bb0025" "etiqueta" => "5." "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Primary ciliary dyskinesia: diagnosis and standards of care" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:7 [ 0 => "A. Bush" 1 => "P. Cole" 2 => "M. Hariri" 3 => "I. Mackay" 4 => "G. Phillips" 5 => "C. O´Callaghan" 6 => "R. 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Fulcher" 1 => "M.A. Turner" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1148/rg.226025016" "Revista" => array:6 [ "tituloSerie" => "Radiographics" "fecha" => "2002" "volumen" => "22" "paginaInicial" => "1439" "paginaFinal" => "1456" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/12432114" "web" => "Medline" ] ] ] ] ] ] ] ] 9 => array:3 [ "identificador" => "bb0050" "etiqueta" => "10." "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Discinesia ciliar primaria:tres nuevos casos" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:1 [ 0 => "R.M.B. Monge" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:6 [ "tituloSerie" => "An Esp Pediatr" "fecha" => "2001" "volumen" => "54" "paginaInicial" => "513" "paginaFinal" => "517" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/11333484" "web" => "Medline" ] ] ] ] ] ] ] ] 10 => array:3 [ "identificador" => "bb0055" "etiqueta" => "11." "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Discinesia ciliar primaria, a propósito de un caso atípico" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:5 [ 0 => "M.M. Albaladejo" 1 => "F.P. Albarracin" 2 => "C.O. Arqueros" 3 => "J.T. Alvaro" 4 => "B. Acin" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:5 [ "tituloSerie" => "An Med Interna (Madrid)" "fecha" => "2002" "volumen" => "19" "paginaInicial" => "460" "paginaFinal" => "462" ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "pt" "url" => "/08732159/0000001500000001/v1_201509151539/S0873215915301161/v1_201509151539/pt/main.assets" "Apartado" => array:4 [ "identificador" => "43360" "tipo" => "SECCION" "pt" => array:2 [ "titulo" => "Casos Clínicos/Cases Reports" "idiomaDefecto" => true ] "idiomaDefecto" => "pt" ] "PDF" => "https://static.elsevier.es/multimedia/08732159/0000001500000001/v1_201509151539/S0873215915301161/v1_201509151539/pt/main.pdf?idApp=UINPBA00004E&text.app=https://journalpulmonology.org/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0873215915301161?idApp=UINPBA00004E" ]
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ISSN: 08732159Original language: Portuguese
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| 2017 April | 23 | 2 | 25 |
| 2017 March | 13 | 40 | 53 |
| 2017 February | 11 | 3 | 14 |
| 2017 January | 14 | 4 | 18 |
| 2016 December | 15 | 10 | 25 |
| 2016 November | 9 | 9 | 18 |
| 2016 October | 11 | 7 | 18 |
| 2016 September | 4 | 10 | 14 |
| 2016 August | 3 | 3 | 6 |
| 2016 July | 2 | 6 | 8 |
| 2016 May | 4 | 9 | 13 |
| 2016 April | 1 | 1 | 2 |
| 2016 March | 3 | 3 | 6 |
| 2016 February | 4 | 7 | 11 |
| 2016 January | 6 | 4 | 10 |
| 2015 December | 6 | 3 | 9 |
| 2015 November | 3 | 1 | 4 |
| 2015 October | 3 | 3 | 6 |
Show all
