Tratamiento con sirolimus y prednisona en enfermedad de Erdheim-Chester con compromiso cardiaco: Caso clínico

Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytic disorder. We report a 76-years-old man who suffered a cardiac tamponade secondary to ECD. A pericardial window was made and during the operation the surgeons observed that the myocardium was diffusely infiltrated. Twenty-eight months...

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Autores principales:	Vega,Jorge, Santamarina,Mario
Lenguaje:	Spanish / Castilian
Publicado:	Sociedad Médica de Santiago 2016
Materias:	Cardiac Tamponade Erdheim-Chester Disease Heart Failure Sirolimus
Acceso en línea:	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872016000300017
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spelling	oai:scielo:S0034-988720160003000172016-06-13Tratamiento con sirolimus y prednisona en enfermedad de Erdheim-Chester con compromiso cardiaco: Caso clínicoVega,JorgeSantamarina,Mario Cardiac Tamponade Erdheim-Chester Disease Heart Failure Sirolimus Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytic disorder. We report a 76-years-old man who suffered a cardiac tamponade secondary to ECD. A pericardial window was made and during the operation the surgeons observed that the myocardium was diffusely infiltrated. Twenty-eight months before, ECD was clinically diagnosed and prednisone and methotrexate were prescribed. Due to disease progression which culminated in the cardiac tamponade, methotrexate was changed to sirolimus aiming to obtain plasma levels between 5-8 ng/ml. This treatment stabilized his cardiac function allowing a survival of 52 months after its initiation, with fewer side effects.info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.144 n.3 20162016-03-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872016000300017es10.4067/S0034-98872016000300017
institution	Scielo Chile
collection	Scielo Chile
language	Spanish / Castilian
topic	Cardiac Tamponade Erdheim-Chester Disease Heart Failure Sirolimus
spellingShingle	Cardiac Tamponade Erdheim-Chester Disease Heart Failure Sirolimus Vega,Jorge Santamarina,Mario Tratamiento con sirolimus y prednisona en enfermedad de Erdheim-Chester con compromiso cardiaco: Caso clínico
description	Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytic disorder. We report a 76-years-old man who suffered a cardiac tamponade secondary to ECD. A pericardial window was made and during the operation the surgeons observed that the myocardium was diffusely infiltrated. Twenty-eight months before, ECD was clinically diagnosed and prednisone and methotrexate were prescribed. Due to disease progression which culminated in the cardiac tamponade, methotrexate was changed to sirolimus aiming to obtain plasma levels between 5-8 ng/ml. This treatment stabilized his cardiac function allowing a survival of 52 months after its initiation, with fewer side effects.
author	Vega,Jorge Santamarina,Mario
author_facet	Vega,Jorge Santamarina,Mario
author_sort	Vega,Jorge
title	Tratamiento con sirolimus y prednisona en enfermedad de Erdheim-Chester con compromiso cardiaco: Caso clínico
title_short	Tratamiento con sirolimus y prednisona en enfermedad de Erdheim-Chester con compromiso cardiaco: Caso clínico
title_full	Tratamiento con sirolimus y prednisona en enfermedad de Erdheim-Chester con compromiso cardiaco: Caso clínico
title_fullStr	Tratamiento con sirolimus y prednisona en enfermedad de Erdheim-Chester con compromiso cardiaco: Caso clínico
title_full_unstemmed	Tratamiento con sirolimus y prednisona en enfermedad de Erdheim-Chester con compromiso cardiaco: Caso clínico
title_sort	tratamiento con sirolimus y prednisona en enfermedad de erdheim-chester con compromiso cardiaco: caso clínico
publisher	Sociedad Médica de Santiago
publishDate	2016
url	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872016000300017
work_keys_str_mv	AT vegajorge tratamientoconsirolimusyprednisonaenenfermedaddeerdheimchesterconcompromisocardiacocasoclinico AT santamarinamario tratamientoconsirolimusyprednisonaenenfermedaddeerdheimchesterconcompromisocardiacocasoclinico
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Tratamiento con sirolimus y prednisona en enfermedad de Erdheim-Chester con compromiso cardiaco: Caso clínico

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