Caso Clínico/Case Report
Linfangioleiomiomatose – A propósito de três casos clínicos
Lymphangioleiomyomatosis – report of three cases
Carla Valente1,
, Sónia André1, Alexandra Catarino2, Fátima Fradinho2, Fernanda Gamboa2, Mário Loureiro3, M. Fontes Baganha4
Corresponding author
carlavalente77@yahoo.com.br
Departamento de Ciências Pneumológicas e Alergológicas dos Hospitais da Universidade de Coimbra, Av. Bissaya Barreto e Praceta Prof. Mota Pinto, 3000-075 Coimbra
Departamento de Ciências Pneumológicas e Alergológicas dos Hospitais da Universidade de Coimbra, Av. Bissaya Barreto e Praceta Prof. Mota Pinto, 3000-075 Coimbra
1 Interna Complementar de Pneumologia/Resident, Pulmonology
2 Assistente Hospitalar de Pneumologia/Consultant, Pulmonology
3 Director do Serviço de Pneumologia dos HUC/Head, Pulomonology Unit, HUC
4 Director do Departamento de Ciências Pneumológicas e Alergológicas dos HUC/Director, Dept. of Pulmonology and Allergological Sciences, HUC
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Bissaya Barreto e Praceta Prof. Mota Pinto, 3000-075 Coimbra" ] ] ] ] "titulosAlternativos" => array:1 [ "en" => array:1 [ "titulo" => "Lymphangioleiomyomatosis – report of three cases" ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2009-05-04" "fechaAceptado" => "2009-06-23" "PalabrasClave" => array:2 [ "pt" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palavras-chave" "identificador" => "xpalclavsec570096" "palabras" => array:2 [ 0 => "Linfangioleiomiomatose" 1 => "complexo esclerose tuberosa" ] ] ] "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Key-words" "identificador" => "xpalclavsec570097" "palabras" => array:2 [ 0 => "Lymphangioleiomyomatosis" 1 => "tuberous sclerosis complex" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "pt" => array:2 [ "titulo" => "Resumo" "resumen" => "<span id="as0005" class="elsevierStyleSection elsevierViewall"><p id="sp0005" class="elsevierStyleSimplePara elsevierViewall">A linfangioleiomiomatose (LAM) é uma doença rara, de etiologia desconhecida, caracterizada pela proliferação anormal de células musculares lisas nas regiões perilinfática, perivascular e peribrônquica.</p><p id="sp0010" class="elsevierStyleSimplePara elsevierViewall">A LAM pode ocorrer esporadicamente ou associada ao complexo esclerose tuberosa (CET) e hamartose hereditária multiorgânica<span class="elsevierStyleSup">1</span>.</p><p id="sp0015" class="elsevierStyleSimplePara elsevierViewall">Em ambas as situações a LAM afecta principalmente mulheres jovens em idade fértil, sendo que aproximadamente 1/3 das mulheres com CET têm LAM<span class="elsevierStyleSup">2</span>.</p><p id="sp0020" class="elsevierStyleSimplePara elsevierViewall">A propósito desta patologia, os autores elaboram uma revisão da literatura e descrevem os casos clínicos de três doentes do sexo feminino com o diagnóstico de LAM com base nos achados clínicos e imagiológicos.</p><p id="sp0025" class="elsevierStyleSimplePara elsevierViewall"><span class="elsevierStyleBold">Rev Port Pneumol 2010; XVI (1): 187-196</span></p></span>" ] "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<span id="as0010" class="elsevierStyleSection elsevierViewall"><p id="sp0030" class="elsevierStyleSimplePara elsevierViewall">Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease of unknown aetiology. It is characterized by proliferation of abnormal smooth-muscle cells throughout the peribronchial, perivascular and perilymphatic regions of the lung.</p><p id="sp0035" class="elsevierStyleSimplePara elsevierViewall">LAM may occur sporadically, in association with tuberous sclerosis complex (TSC) or inheritable multiorgan hamartomatosis <span class="elsevierStyleSup">1</span>.</p><p id="sp0040" class="elsevierStyleSimplePara elsevierViewall">In either situation, LAM occurs almost exclusively in women of reproductive age, and approximately one third of the patients with TSC have LAM<span class="elsevierStyleSup">2</span>.</p><p id="sp0045" class="elsevierStyleSimplePara elsevierViewall">The authors review the cases of three female patients diagnosed with LAM based on clinical and radiological findings.</p><p id="sp0050" class="elsevierStyleSimplePara elsevierViewall">A brief review of the disease is then presented.</p><p id="sp0055" class="elsevierStyleSimplePara elsevierViewall"><span class="elsevierStyleBold">Rev Port Pneumol 2010; XVI (1): 187-196</span></p></span>" ] ] ] "idiomaDefecto" => "pt" "url" => "/08732159/0000001600000001/v1_201509151437/S0873215915300179/v1_201509151437/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/08732159/0000001600000001/v1_201509151437/S0873215915300179/v1_201509151437/pt/main.pdf?idApp=UINPBA00004E&text.app=https://journalpulmonology.org/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0873215915300179?idApp=UINPBA00004E" ]
Article information
ISSN: 08732159Original language: Portuguese
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