Linfangioleiomiomatosis pulmonar: Caso clínico / Pulmonary lymphangioleiomyomatosis: Report of one case
Rev. méd. Chile
;
137(11): 1474-1477, nov. 2009. ilus
Artículo
en Español
| LILACS
| ID: lil-537011
ABSTRACT
Lymphangioleiomyomatosis (LAM) is a rare interstitial lung disease, of unknown etiology, affecting almost exclusively women. Microscopically LAM consists of a diffuse proliferation of smooth muscle cells. LAM can occur without evidence of other diseases (sporadic LAM) or in conjunction with tuberous sclerosis complex (TSC). It presents with progressive breathlessness or with recurrent pneumothorax or chylothorax. We report a 33 year-old woman with a history of recurrent pneumothorax. Computed tomography (CT) scans showed numerous thin-walled cysts throughout the lungs, a characteristic finding in LAM. A pulmonary biopsy was compatible with the diagnosis and HMB-45 monoclonal antibodies were positive. Treatment with Sirolimus was started.
Texto completo:
Disponible
Índice:
LILACS (Américas)
Asunto principal:
Linfangioleiomiomatosis
/
Enfermedades Pulmonares Intersticiales
/
Neoplasias Pulmonares
Tipo de estudio:
Estudio de etiología
Límite:
Adulto
/
Femenino
/
Humanos
Idioma:
Español
Revista:
Rev. méd. Chile
Asunto de la revista:
Medicina
Año:
2009
Tipo del documento:
Artículo
País de afiliación:
Chile
Institución/País de afiliación:
Hospital Base de Linares/CL
/
Hospital Regional de Talca/CL
/
Universidad de Talca/CL
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