Linfangioleiomiomatosis pulmonar: Caso clínico / Pulmonary lymphangioleiomyomatosis: Report of one case
Rev. méd. Chile
;
137(11): 1474-1477, nov. 2009. ilus
Article
Dans Espagnol
| 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.
Texte intégral:
Disponible
Indice:
LILAS (Amériques)
Sujet Principal:
Lymphangioléiomyomatose
/
Pneumopathies interstitielles
/
Tumeurs du poumon
Type d'étude:
Etude d'étiologie
Limites du sujet:
Adulte
/
Femelle
/
Humains
langue:
Espagnol
Texte intégral:
Rev. méd. Chile
Thème du journal:
Médicament
Année:
2009
Type:
Article
Pays d'affiliation:
Chili
Institution/Pays d'affiliation:
Hospital Base de Linares/CL
/
Hospital Regional de Talca/CL
/
Universidad de Talca/CL
Documents relatifs à ce sujet
MEDLINE
...
LILACS
LIS