<?xml version="1.0" encoding="ISO-8859-1"?><article xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance">
<front>
<journal-meta>
<journal-id>0211-6995</journal-id>
<journal-title><![CDATA[Nefrología (Madrid)]]></journal-title>
<abbrev-journal-title><![CDATA[Nefrología (Madr.)]]></abbrev-journal-title>
<issn>0211-6995</issn>
<publisher>
<publisher-name><![CDATA[Sociedad Española de Nefrología]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S0211-69952017000100087</article-id>
<article-id pub-id-type="doi">10.1016/j.nefro.2016.04.007</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Sangrado de angiomiolipoma renal en paciente con síndrome de genes contiguos (TSC2/PKD1) tras 17 años de tratamiento renal sustitutivo]]></article-title>
<article-title xml:lang="en"><![CDATA[Renal angiomyolipoma bleeding in a patient with TSC2/PKD1 contiguous gene syndrome after 17 years of renal replacement therapy]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Furlano]]></surname>
<given-names><![CDATA[Mónica]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
<xref ref-type="aff" rid="A a"/>
<xref ref-type="aff" rid="A7 "/>
<xref ref-type="aff" rid="Af1"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Barreiro]]></surname>
<given-names><![CDATA[Yaima]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Martí]]></surname>
<given-names><![CDATA[Teresa]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Facundo]]></surname>
<given-names><![CDATA[Carme]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
<xref ref-type="aff" rid="A a"/>
<xref ref-type="aff" rid="A7 "/>
<xref ref-type="aff" rid="Af1"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Ruiz-García]]></surname>
<given-names><![CDATA[César]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[DaSilva]]></surname>
<given-names><![CDATA[Iara]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
<xref ref-type="aff" rid="A a"/>
<xref ref-type="aff" rid="A7 "/>
<xref ref-type="aff" rid="Af1"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Ayasreh]]></surname>
<given-names><![CDATA[Nadia]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
<xref ref-type="aff" rid="A a"/>
<xref ref-type="aff" rid="A7 "/>
<xref ref-type="aff" rid="Af1"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Cabrera-López]]></surname>
<given-names><![CDATA[Cristina]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
<xref ref-type="aff" rid="A a"/>
<xref ref-type="aff" rid="A10"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Ballarín]]></surname>
<given-names><![CDATA[José]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
<xref ref-type="aff" rid="A a"/>
<xref ref-type="aff" rid="A7 "/>
<xref ref-type="aff" rid="Af1"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Ars]]></surname>
<given-names><![CDATA[Elisabet]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
<xref ref-type="aff" rid="A a"/>
<xref ref-type="aff" rid="A8 "/>
<xref ref-type="aff" rid="Af1"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Torra]]></surname>
<given-names><![CDATA[Roser]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
<xref ref-type="aff" rid="A a"/>
<xref ref-type="aff" rid="A7 "/>
<xref ref-type="aff" rid="Af1"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Fundació Puigvert Servicio de Nefrología Enfermedades Renales Hereditarias]]></institution>
<addr-line><![CDATA[Barcelona ]]></addr-line>
<country>España</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Avericum S.L. Servicio de Hemodiálisis ]]></institution>
<addr-line><![CDATA[Las Palmas de Gran Canaria ]]></addr-line>
<country>España</country>
</aff>
<aff id="Af3">
<institution><![CDATA[,Fundació Puigvert Servicio de Radiología ]]></institution>
<addr-line><![CDATA[Barcelona ]]></addr-line>
<country>España</country>
</aff>
<aff id="Af4">
<institution><![CDATA[,Fundació Puigvert Servicio de Nefrología Unidad de Trasplante Renal]]></institution>
<addr-line><![CDATA[Barcelona ]]></addr-line>
<country>España</country>
</aff>
<aff id="Af5">
<institution><![CDATA[,Fundació Puigvert Servicio de Nefrología ]]></institution>
<addr-line><![CDATA[Barcelona ]]></addr-line>
<country>España</country>
</aff>
<aff id="Af6">
<institution><![CDATA[,Instituto de Investigaciones Biomédicas Sant Pau (IIB-Sant Pau)  ]]></institution>
<addr-line><![CDATA[Barcelona ]]></addr-line>
<country>España</country>
</aff>
<aff id="Af7">
<institution><![CDATA[,Universitat Autònoma de Barcelona  ]]></institution>
<addr-line><![CDATA[Barcelona ]]></addr-line>
<country>Spain</country>
</aff>
<aff id="A10">
<institution><![CDATA[,Instituto de Investigación Carlos III REDinREN ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
</aff>
<aff id="Af8">
<institution><![CDATA[,Fundació Puigvert Laboratorio de Biología Molecular ]]></institution>
<addr-line><![CDATA[Barcelona ]]></addr-line>
<country>España</country>
</aff>
<aff id="Af9">
<institution><![CDATA[,Hospital Moisés Broggi Servicio de Nefrología ]]></institution>
<addr-line><![CDATA[Sant Joan Despí Barcelona]]></addr-line>
<country>España</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>02</month>
<year>2017</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>02</month>
<year>2017</year>
</pub-date>
<volume>37</volume>
<numero>1</numero>
<fpage>87</fpage>
<lpage>92</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.isciii.es/scielo.php?script=sci_arttext&amp;pid=S0211-69952017000100087&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.isciii.es/scielo.php?script=sci_abstract&amp;pid=S0211-69952017000100087&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.isciii.es/scielo.php?script=sci_pdf&amp;pid=S0211-69952017000100087&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[Resumen Presentamos el caso de un varón de 32 años, con síndrome de genes contiguos TSC2/PKD1, que le ocasiona esclerosis tuberosa (ET) y poliquistosis renal autosómica dominante simultáneamente. Evolucionó a enfermedad renal terminal y se realizó trasplante renal a los 12 años. Los riñones presentaban angiomiolipomas (AML), que son tumores benignos frecuentes en pacientes con ET. A los 17 años postrasplante, presentó un cuadro de dolor abdominal, anemización y hematoma retroperitoneal. Dicho hematoma se produjo por el sangrado de los AML. Como tratamiento se realizó embolización selectiva. Nuestro paciente podría haberse beneficiado en el momento del trasplante renal del tratamiento con inhibidores de mTOR. Este fármaco actúa como inmunosupresor y reductor tumoral en la ET, al disminuir el riesgo de rotura y hemorragia. En este paciente no se administró porque cuando se trasplantó no se conocía la relación de los inhibidores de mTOR con la ET. Este caso confirma que, a pesar de tratarse de pacientes trasplantados o en diálisis, el riesgo de sangrado por los AML persiste, por lo cual se propone realizar controles periódicos de los riñones propios y valorar la nefrectomía.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[Abstract We report the case of a 32-year-old male diagnosed with TSC2/PKD1 contiguous gene syndrome, presenting with tuberous sclerosis (TS) and autosomal dominant polycystic kidney disease simultaneously. He progressed to end-stage renal disease and received a kidney transplant at the age of 12. The native kidneys presented angiomyolipomas (AML), which are common benign tumours in patients with TS. Seventeen years after transplantation, he presented with abdominal pain, anaemia and a retroperitoneal haematoma, the latter caused by renal AML bleeding. Selective embolisation was performed. Our patient could have benefited from the administration of mTOR inhibitors at transplant. This therapy is immunosuppressive and reduces the size of benign tumours in TS as well as the risk of rupture and bleeding. This patient did not receive mTOR inhibitors at the time of the transplant because the relationship between mTOR inhibitors and TS was unknown at that time. This case confirms the persistent risk of renal AML bleeding for both transplanted patients and patients on dialysis. As a result, we would recommend routine check-ups of native kidneys and nephrectomy assessment.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[Esclerosis tuberosa]]></kwd>
<kwd lng="es"><![CDATA[Poliquistosis renal autosómica dominante]]></kwd>
<kwd lng="es"><![CDATA[PQRAD]]></kwd>
<kwd lng="es"><![CDATA[Angiomiolipoma]]></kwd>
<kwd lng="es"><![CDATA[Inhibidores de mTOR]]></kwd>
<kwd lng="es"><![CDATA[Síndrome de genes contiguos]]></kwd>
<kwd lng="es"><![CDATA[TSC2/PKD1]]></kwd>
<kwd lng="en"><![CDATA[Tuberous sclerosis]]></kwd>
<kwd lng="en"><![CDATA[Autosomal dominant polycystic kidney disease]]></kwd>
<kwd lng="en"><![CDATA[ADPKD]]></kwd>
<kwd lng="en"><![CDATA[Angiomyolipoma]]></kwd>
<kwd lng="en"><![CDATA[mTOR inhibitors]]></kwd>
<kwd lng="en"><![CDATA[Contiguous gene syndrome]]></kwd>
<kwd lng="en"><![CDATA[TSC2/PKD1]]></kwd>
</kwd-group>
</article-meta>
</front><back>
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