Um caso raro de discinesia ciliar primária associada a heterotaxia
A rare case of primary ciliary dyskinesia with heterotaxy
Cátia Quintela1, Cláudia Meireles2, Maria João Bettencourt3, Augusto Ribeirinho4, Teresa Bentes3, Martins Neves5, António Santos Castro6, Moura Nunes7
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
5 Serviço de Gastrenterologia do Hospital Centro Hospitalar de Lisboa Central – Hospital Santo António dos Capuchos
6 Serviço de Medicina Interna do Centro Hospitalar de Lisboa Central – Hospital Santo António dos Capuchos
7 Serviço de Microscopia Electrónica Instituto Português de Oncologia de Lisboa
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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" => "xpalclavsec159924" "palabras" => array:2 [ 0 => "Discinesia ciliar primária" 1 => "heterotaxia" ] ] ] "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Key-words" "identificador" => "xpalclavsec159925" "palabras" => array:2 [ 0 => "Primary ciliary dyskinesia" 1 => "heterotaxy" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "pt" => array:2 [ "titulo" => "Resumo" "resumen" => "<p id="spar0005" 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 doen te 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>" ] "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<p id="spar0010" 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>" ] ] "bibliografia" => array:2 [ "titulo" => "Bibliografia" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:12 [ 0 => array:3 [ "identificador" => "bib1" "etiqueta" => "1." 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ISSN: 21735115Original language: Portuguese
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| 2023 August | 14 | 18 | 32 |
| 2023 July | 17 | 25 | 42 |
| 2023 June | 15 | 19 | 34 |
| 2023 May | 18 | 23 | 41 |
| 2023 April | 15 | 6 | 21 |
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