<?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>2709-7927</journal-id>
<journal-title><![CDATA[Acta Médica del Centro]]></journal-title>
<abbrev-journal-title><![CDATA[Acta méd centro]]></abbrev-journal-title>
<issn>2709-7927</issn>
<publisher>
<publisher-name><![CDATA[Hospital Provincial Clínico Quirúrgico Universitario Arnaldo Milián Castro]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S2709-79272023000400783</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Anestesia y enfermedad de Von Hippel-Lindau. Infome de caso]]></article-title>
<article-title xml:lang="en"><![CDATA[Anesthesia and Von Hippel-Lindau disease. Case report]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Mejías Chao]]></surname>
<given-names><![CDATA[Tania]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Mejías Chao]]></surname>
<given-names><![CDATA[Lilliam María]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Amador Mejías]]></surname>
<given-names><![CDATA[Luis Miguel]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Hospital Provincial Oncológico Universitario &#8220;Dr. Celestino Hernández Robau&#8221;  ]]></institution>
<addr-line><![CDATA[Santa Clara Villa Clara]]></addr-line>
<country>Cuba</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Universidad de Ciencias Médicas de Villa Clara  ]]></institution>
<addr-line><![CDATA[Santa Clara Villa Clara]]></addr-line>
<country>Cuba</country>
</aff>
<aff id="Af3">
<institution><![CDATA[,Policlínico Universitario &#8220;Chiqui Gómez Lubián&#8221;  ]]></institution>
<addr-line><![CDATA[Santa Clara Villa Clara]]></addr-line>
<country>Cuba</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>12</month>
<year>2023</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>12</month>
<year>2023</year>
</pub-date>
<volume>17</volume>
<numero>4</numero>
<fpage>783</fpage>
<lpage>788</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_arttext&amp;pid=S2709-79272023000400783&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_abstract&amp;pid=S2709-79272023000400783&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_pdf&amp;pid=S2709-79272023000400783&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[RESUMEN  Introducción:  el manejo de las enfermedades genéticas constituye un reto por su baja incidencia, su etiología diversa, las enfermedades coexistentes y la variabilidad en las respuestas a los anestésicos. La presentación de este caso aporta una nueva visión de cómo abordar el manejo anestésico general multimodal en pacientes que padecen la enfermedad de Von Hippel-Lindau.  Información del paciente:  se presenta una paciente femenina de 49 años para cirugía ortopédica con herida abierta de tibia y antecedentes de asma bronquial y enfermedad de Von Hippel-Lindau, operada de hemangioblastoma cerebeloso y de carcinoma renal, con hemangioblastoma meningeo y cerebeloso izquierdo actual con seguimiento clínico e imagenológico, quistes renales y pancreáticos, lesión nodular suprarrenal derecha de reciente aparición (descarta la posibilidad de un feocromocitoma) y cefalea migrañosa reciente y no relacionada con hipertensión endocraneana a pesar del efecto de masa del tumor cerebeloso. Se realizó manejo anestésico general multimodal.  Conclusiones:  se puede plantear la posibilidad de realizar técnicas mínimamente invasivas multimodales en la intervención quirúrgica de un paciente de alto riesgo quirúrgico con enfermedad Von Hippel-Lindau con herida abierta de tibia con el fin de minimizar la morbilidad y la mortalidad transoperatorias y evitar complicaciones.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[ABSTRACT  Introduction:  the management of genetic diseases is challenging because of their low incidence, diverse etiology, coexisting diseases, and variability in responses to anesthetics. This case presentation provides new insight into how to approach multimodal general anesthetic management in patients with Von Hippel-Lindau disease.  Patient information:  a 49-year-old female patient is presented for orthopedic surgery with open tibial wound and history of bronchial asthma and Von Hippel-Lindau disease, operated for cerebellar hemangioblastoma and renal carcinoma, with current left meningeal and cerebellar hemangioblastoma with clinical and imaging follow-up, renal and pancreatic cysts, right adrenal nodular lesion of recent appearance (ruling out the possibility of pheochromocytoma) and recent migraine headache unrelated to endocranial hypertension despite the mass effect of the cerebellar tumor. Multimodal general anesthetic management was performed.  Conclusions:  The possibility of performing multimodal minimally invasive techniques in the surgical intervention of a high surgical risk patient with Von Hippel-Lindau disease with open tibial wound can be considered in order to minimize transoperative morbidity and mortality and to avoid complications.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[anestesia general multimodal]]></kwd>
<kwd lng="es"><![CDATA[enfermedad de von Hippel-Lindau]]></kwd>
<kwd lng="en"><![CDATA[multimodal general anesthesia]]></kwd>
<kwd lng="en"><![CDATA[von Hippel-Lindau Disease]]></kwd>
</kwd-group>
</article-meta>
</front><back>
<ref-list>
<ref id="B1">
<label>1</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[García Balaguer]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Arteche Arnaiz]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[Elgezabal Gomez]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[García-Serrano Fuertes]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Lopez Maseda]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Garmendia Zabaleta]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Enfermedad de Von Hippel-Lindau: sus manifestaciones más frecuentes y cómo reconocerlas]]></article-title>
<source><![CDATA[SERAM]]></source>
<year>2022</year>
<volume>1</volume>
<numero>1</numero>
<issue>1</issue>
</nlm-citation>
</ref>
<ref id="B2">
<label>2</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Calle]]></surname>
<given-names><![CDATA[X]]></given-names>
</name>
<name>
<surname><![CDATA[Jiménez-Gallegos]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
<name>
<surname><![CDATA[Muñoz-Córdova]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
<name>
<surname><![CDATA[Sánchez]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Lavandero]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Mecanismo sensor y de adaptación a los niveles de oxígeno y su implicancia en las enfermedades cardiovasculares: a propósito del Premio Nobel de Fisiología-Medicina 2019]]></article-title>
<source><![CDATA[Rev Chil Cardiol]]></source>
<year>2019</year>
<volume>38</volume>
<numero>3</numero>
<issue>3</issue>
<page-range>225-35</page-range></nlm-citation>
</ref>
<ref id="B3">
<label>3</label><nlm-citation citation-type="book">
<collab>MedlinePlus</collab>
<article-title xml:lang=""><![CDATA[Von Hippel-Lindau Disease]]></article-title>
<source><![CDATA[]]></source>
<year>25/0</year>
<month>4/</month>
<day>20</day>
<publisher-loc><![CDATA[Bethesda ]]></publisher-loc>
<publisher-name><![CDATA[National Library of Medicine]]></publisher-name>
</nlm-citation>
</ref>
<ref id="B4">
<label>4</label><nlm-citation citation-type="book">
<collab>Instituto Nacional del Cáncer</collab>
<article-title xml:lang=""><![CDATA[Enfermedad de Von Hippel-Lindau (PDQ(r))-Versión para profesionales de salud]]></article-title>
<source><![CDATA[]]></source>
<year>24/0</year>
<month>6/</month>
<day>20</day>
<publisher-loc><![CDATA[Bethesda ]]></publisher-loc>
<publisher-name><![CDATA[NCI]]></publisher-name>
</nlm-citation>
</ref>
<ref id="B5">
<label>5</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Sala-Hernández]]></surname>
<given-names><![CDATA[Á]]></given-names>
</name>
<name>
<surname><![CDATA[Montalvá-Orón]]></surname>
<given-names><![CDATA[EM]]></given-names>
</name>
<name>
<surname><![CDATA[Pareja-Ibars]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Ballester-Pla]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[López-Andújar]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Manejo del gastrinoma pancreático asociado a la enfermedad de Von Hippel-Lindau. A propósito de un caso]]></article-title>
<source><![CDATA[Rev Esp Enferm Dig]]></source>
<year>2017</year>
<volume>109</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>154-7</page-range></nlm-citation>
</ref>
<ref id="B6">
<label>6</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Salazar]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<name>
<surname><![CDATA[González-Castaño]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Rozas]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Castro]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Hemangioma capilar retiniano y enfermedad de von Hippel-Lindau: implicaciones diagnósticas y terapéuticas]]></article-title>
<source><![CDATA[Arch Soc Esp Oftalmol]]></source>
<year>2011</year>
<volume>86</volume>
<numero>7</numero>
<issue>7</issue>
<page-range>218-21</page-range></nlm-citation>
</ref>
<ref id="B7">
<label>7</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Fons Martínez]]></surname>
<given-names><![CDATA[MR]]></given-names>
</name>
<name>
<surname><![CDATA[España Gregori]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Aviñó Martínez]]></surname>
<given-names><![CDATA[JA]]></given-names>
</name>
<name>
<surname><![CDATA[Hernández Pardines]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Angioma retiniano como síndrome mascarada de tumor del nervio óptico en la enfermedad de Von Hippel-Lindau]]></article-title>
<source><![CDATA[Arch Soc Esp Oftalmol]]></source>
<year>2006</year>
<volume>81</volume>
<numero>5</numero>
<issue>5</issue>
<page-range>293-6</page-range></nlm-citation>
</ref>
<ref id="B8">
<label>8</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Villar Gómez de las Heras]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Descripción de la enfermedad de von Hippel-Lindau en tres familias españolas]]></article-title>
<source><![CDATA[An Med Interna (Madrid)]]></source>
<year>2002</year>
<volume>19</volume>
<numero>7</numero>
<issue>7</issue>
<page-range>34-8</page-range></nlm-citation>
</ref>
<ref id="B9">
<label>9</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Vallejo Desviat]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Anguita Martínez]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Villar Banco]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[Molina López-Nava]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Múltiples imágenes nodulares hipervasculares]]></article-title>
<source><![CDATA[Sanidad Mil]]></source>
<year>2018</year>
<volume>74</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>47-8</page-range></nlm-citation>
</ref>
<ref id="B10">
<label>10</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Regato Avilés]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Cimadevilla Calvo]]></surname>
<given-names><![CDATA[B]]></given-names>
</name>
<name>
<surname><![CDATA[Rábago Moriyón]]></surname>
<given-names><![CDATA[JL]]></given-names>
</name>
<name>
<surname><![CDATA[Ruiz Andrés]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Del Olmo Ruiloba]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Manejo anestésico de feocromocitoma en paciente con enfermedad de Von Hippel-Lindau]]></article-title>
<source><![CDATA[Rev Chil Anest]]></source>
<year>2022</year>
<volume>51</volume>
<numero>3</numero>
<issue>3</issue>
<page-range>335-8</page-range></nlm-citation>
</ref>
<ref id="B11">
<label>11</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Ene]]></surname>
<given-names><![CDATA[CI]]></given-names>
</name>
<name>
<surname><![CDATA[Marton]]></surname>
<given-names><![CDATA[RP]]></given-names>
</name>
<name>
<surname><![CDATA[Ferreira Jr]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Sekhar]]></surname>
<given-names><![CDATA[LN]]></given-names>
</name>
<name>
<surname><![CDATA[Kim]]></surname>
<given-names><![CDATA[LJ]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Spontaneous hemorrhage from central nervous system hemangioblastomas]]></article-title>
<source><![CDATA[World Neurosurg]]></source>
<year>2015</year>
<volume>83</volume>
<numero>6</numero>
<issue>6</issue>
<page-range>1180.e13-7</page-range></nlm-citation>
</ref>
<ref id="B12">
<label>12</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Shoseyov]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
<name>
<surname><![CDATA[Cohen-Cymberknoh]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Wilschanski]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Ataluren for the treatment of cystic fibrosis]]></article-title>
<source><![CDATA[Expert Rev Respir Med]]></source>
<year>2016</year>
<volume>10</volume>
<numero>4</numero>
<issue>4</issue>
<page-range>387-91</page-range></nlm-citation>
</ref>
<ref id="B13">
<label>13</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Jito]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Nozaki]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Treatment strategies for cerebellar hemangioblastomas: simple or further studies?]]></article-title>
<source><![CDATA[World Neurosurg]]></source>
<year>2014</year>
<volume>82</volume>
<numero>5</numero>
<issue>5</issue>
<page-range>619-20</page-range></nlm-citation>
</ref>
<ref id="B14">
<label>14</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Richards]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Aziz]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Bale]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Bick]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
<name>
<surname><![CDATA[Das]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Gastier-Foster]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Standards and guidelines for the interpretation of sequence variants: a joint consensus recommendation of the American College of Medical Genetics and Genomics and the Association for Molecular Pathology]]></article-title>
<source><![CDATA[Genet Med]]></source>
<year>2015</year>
<volume>17</volume>
<numero>5</numero>
<issue>5</issue>
<page-range>405-24</page-range></nlm-citation>
</ref>
<ref id="B15">
<label>15</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Binderup]]></surname>
<given-names><![CDATA[ML]]></given-names>
</name>
<name>
<surname><![CDATA[Budtz-Jørgensen]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Bisgaard]]></surname>
<given-names><![CDATA[ML]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Risk of new tumors in von Hippel-Lindau patients depends on age and genotype]]></article-title>
<source><![CDATA[Genet Med]]></source>
<year>2016</year>
<volume>18</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>89-97</page-range></nlm-citation>
</ref>
</ref-list>
</back>
</article>
