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Vol. 16. Issue 1.
Pages 187-195 (January - February 2010)
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Vol. 16. Issue 1.
Pages 187-195 (January - February 2010)
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Linfangioleiomiomatose – A propósito de três casos clínicos
Lymphangioleiomyomatosis – report of three cases
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Carla Valente1,
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.
, Sónia André1, Alexandra Catarino2, Fátima Fradinho2, Fernanda Gamboa2, Mário Loureiro3, M Fontes Baganha4
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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Resumo

A linfangioleiomiomatose (LAM) é uma doença rara, de etiologia desconhecida, caracterizada pela proliferação anormal de células musculares lisas nas regiões perilinfática, perivascular e peribrônquica.

A LAM pode ocorrer esporadicamente ou associada ao complexo esclerose tuberosa (CET) e hamartose hereditária multiorgânica1.

Em ambas as situações a LAM afecta principalmente mulheres jovens em idade fértil, sendo que aproximadamente 1/3 das mulheres com CET têm LAM2.

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.

Palavras-chave:
Linfangioleiomiomatose
complexo esclerose tuberosa
Abstract

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.

LAM may occur sporadically, in association with tuberous sclerosis complex (TSC) or inheritable multiorgan hamartomatosis 1.

In either situation, LAM occurs almost exclusively in women of reproductive age, and approximately one third of the patients with TSC have LAM2. The authors review the cases of three female patients diagnosed with LAM based on clinical and radiological findings.

A brief review of the disease is then presented.

Key-words:
Lymphangioleiomyomatosis
tuberous sclero sis complex
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