Linfangioleiomiomatosis pulmonar: Caso clínico

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...

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Autores principales:	Silva O,Rafael, Puelma C,Felipe, Retamal P,Víctor, Rojas SM,Pedro, Cruzat C,Claudio, Reyes G,Cecilia
Lenguaje:	Spanish / Castilian
Publicado:	Sociedad Médica de Santiago 2009
Materias:	Lymphangioleiomyomatosis Lung diseases Pneumothorax
Acceso en línea:	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872009001100010
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spelling	oai:scielo:S0034-988720090011000102010-01-13Linfangioleiomiomatosis pulmonar: Caso clínicoSilva O,RafaelPuelma C,FelipeRetamal P,VíctorRojas SM,PedroCruzat C,ClaudioReyes G,Cecilia Lymphangioleiomyomatosis Lung diseases Pneumothorax 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).info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.137 n.11 20092009-11-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872009001100010es10.4067/S0034-98872009001100010
institution	Scielo Chile
collection	Scielo Chile
language	Spanish / Castilian
topic	Lymphangioleiomyomatosis Lung diseases Pneumothorax
spellingShingle	Lymphangioleiomyomatosis Lung diseases Pneumothorax Silva O,Rafael Puelma C,Felipe Retamal P,Víctor Rojas SM,Pedro Cruzat C,Claudio Reyes G,Cecilia Linfangioleiomiomatosis pulmonar: Caso clínico
description	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).
author	Silva O,Rafael Puelma C,Felipe Retamal P,Víctor Rojas SM,Pedro Cruzat C,Claudio Reyes G,Cecilia
author_facet	Silva O,Rafael Puelma C,Felipe Retamal P,Víctor Rojas SM,Pedro Cruzat C,Claudio Reyes G,Cecilia
author_sort	Silva O,Rafael
title	Linfangioleiomiomatosis pulmonar: Caso clínico
title_short	Linfangioleiomiomatosis pulmonar: Caso clínico
title_full	Linfangioleiomiomatosis pulmonar: Caso clínico
title_fullStr	Linfangioleiomiomatosis pulmonar: Caso clínico
title_full_unstemmed	Linfangioleiomiomatosis pulmonar: Caso clínico
title_sort	linfangioleiomiomatosis pulmonar: caso clínico
publisher	Sociedad Médica de Santiago
publishDate	2009
url	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872009001100010
work_keys_str_mv	AT silvaorafael linfangioleiomiomatosispulmonarcasoclinico AT puelmacfelipe linfangioleiomiomatosispulmonarcasoclinico AT retamalpvictor linfangioleiomiomatosispulmonarcasoclinico AT rojassmpedro linfangioleiomiomatosispulmonarcasoclinico AT cruzatcclaudio linfangioleiomiomatosispulmonarcasoclinico AT reyesgcecilia linfangioleiomiomatosispulmonarcasoclinico
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Linfangioleiomiomatosis pulmonar: Caso clínico

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