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Resolución de los linfangioleiomiomas torácicos y abdominales en una paciente con linfangioleiomiomatosis tratada con sirolimus
Resolution of Thoracic and Abdominal Lymphangioleiomyomas in a Patient With Lymphangioleiomyomatosis Treated With Sirolimus
Luis Cabeza Osorioa, María Ángeles Ruiz Cobosb, Álvaro Casanova Espinosab,
Corresponding author
acasanova1977@yahoo.es

Autor para correspondencia.
a Servicio de Medicina Interna, Hospital Universitario del Henares, Coslada, Madrid, España
b Servicio de Neumología, Hospital Universitario del Henares, Coslada, Madrid, España
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        "titulo" => "Resolution of Thoracic and Abdominal Lymphangioleiomyomas in a Patient With Lymphangioleiomyomatosis Treated With Sirolimus"
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          "es" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Tomograf&#237;a axial computarizada &#40;TAC&#41; tor&#225;cica &#40;A&#41; donde se observa una lesi&#243;n qu&#237;stica paravertebral derecha de 3&#44;2<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>1&#44;9<span class="elsevierStyleHsp" style=""></span>cm&#44; compatible con un linfangioleiomioma retrocrural&#46; TAC abdominal &#40;B&#41; donde se aprecia una tumoraci&#243;n de 6&#44;8<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>3&#44;9<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>5&#44;1<span class="elsevierStyleHsp" style=""></span>cm de aspecto qu&#237;stico retroperitoneal&#44; de localizaci&#243;n paraa&#243;rtica izquierda que se extiende hasta la pelvis&#44; compatible con un linfangioleiomioma abdominal&#46; TAC toraco-abdominal al a&#241;o &#40;C y D&#41; donde se observa una resoluci&#243;n pr&#225;cticamente completa de las lesiones descritas&#46;</p>"
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Mujer de 53 a&#241;os diagnosticada en el a&#241;o 2010 de linfangioleiomiomatosis &#40;LAM&#41;&#44; con afectaci&#243;n pulmonar difusa con m&#250;ltiples quistes de paredes finas&#44; linfangioleiomiomas qu&#237;sticos retrocrurales derechos y m&#250;ltiples tumoraciones de aspecto qu&#237;stico retroperitoneales compatibles con linfangioleiomiomas abdominales&#44; la de mayor tama&#241;o de 6&#44;8<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>3&#44;9<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>5&#44;1<span class="elsevierStyleHsp" style=""></span>cm de di&#225;metro &#40;<a class="elsevierStyleCrossRef" href="#fig0005">fig&#46; 1</a> A y B&#41;&#46; La funci&#243;n pulmonar al diagn&#243;stico mostraba una capacidad vital forzada &#40;FVC&#41; de 3&#46;300<span class="elsevierStyleHsp" style=""></span>ml &#40;98&#37;&#41;&#44; un volumen espiratorio forzado en el primer segundo &#40;FEV<span class="elsevierStyleInf">1</span>&#41; de 2&#46;370<span class="elsevierStyleHsp" style=""></span>ml &#40;82&#37;&#41; y un cociente FEV<span class="elsevierStyleInf">1</span>&#47;FVC del 69&#37;&#46; La capacidad de difusi&#243;n de mon&#243;xido de carbono &#40;DLCO&#41; era del 89&#37;&#46; Cl&#237;nicamente&#44; refer&#237;a disnea de grandes esfuerzos y episodios intermitentes de dolor tor&#225;cico y abdominal de predominio catamenial&#46; Tras 2 a&#241;os de seguimiento se observa un empeoramiento significativo de la funci&#243;n pulmonar siendo el FEV<span class="elsevierStyleInf">1</span> de 2&#46;040<span class="elsevierStyleHsp" style=""></span>ml &#40;71&#37;&#41; y la DLCO de un 72&#37;&#44; un aumento de la disnea basal y persistencia de la cl&#237;nica catamenial&#46; Dada la progresi&#243;n cl&#237;nica y funcional se inicia tratamiento con el inhibidor de m-TOR &#40;del ingl&#233;s <span class="elsevierStyleItalic">&#171;mammalian target of rapamycin&#187;</span>&#41; sirolimus a dosis de 2<span class="elsevierStyleHsp" style=""></span>mg&#47;d&#237;a<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">1</span></a>&#46; Un a&#241;o despu&#233;s del inicio del tratamiento se realiza un control radiol&#243;gico evidenci&#225;ndose una resoluci&#243;n pr&#225;cticamente completa de los linfangioleiomiomas tor&#225;cicos y abdominales<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">2</span></a> &#40;<a class="elsevierStyleCrossRef" href="#fig0005">fig&#46; 1</a>C y D&#41;&#46; Se observa una mejor&#237;a cl&#237;nica y estabilidad de la funci&#243;n pulmonar con un FEV<span class="elsevierStyleInf">1</span> de 1&#46;980<span class="elsevierStyleHsp" style=""></span>ml &#40;70&#37;&#41; y una DLCO del 82&#37;&#46; Esta imagen demuestra el efecto beneficioso de sirolimus en la reducci&#243;n del tama&#241;o de los linfangioleiomiomas tor&#225;cicos y abdominales&#44; y en la estabilizaci&#243;n de la funci&#243;n pulmonar en pacientes con LAM&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span>"
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          "es" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Tomograf&#237;a axial computarizada &#40;TAC&#41; tor&#225;cica &#40;A&#41; donde se observa una lesi&#243;n qu&#237;stica paravertebral derecha de 3&#44;2<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>1&#44;9<span class="elsevierStyleHsp" style=""></span>cm&#44; compatible con un linfangioleiomioma retrocrural&#46; TAC abdominal &#40;B&#41; donde se aprecia una tumoraci&#243;n de 6&#44;8<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>3&#44;9<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>5&#44;1<span class="elsevierStyleHsp" style=""></span>cm de aspecto qu&#237;stico retroperitoneal&#44; de localizaci&#243;n paraa&#243;rtica izquierda que se extiende hasta la pelvis&#44; compatible con un linfangioleiomioma abdominal&#46; TAC toraco-abdominal al a&#241;o &#40;C y D&#41; donde se observa una resoluci&#243;n pr&#225;cticamente completa de las lesiones descritas&#46;</p>"
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Article information
ISSN: 03002896
Original language: Spanish
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