<?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>2448-8771</journal-id>
<journal-title><![CDATA[Anestesia en México]]></journal-title>
<abbrev-journal-title><![CDATA[Anest. Méx.]]></abbrev-journal-title>
<issn>2448-8771</issn>
<publisher>
<publisher-name><![CDATA[Federación Mexicana de Colegios de Anestesiología A.C.]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S2448-87712019000100043</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Manejo anestésico en paciente pediátrico con síndrome de Beckwithwiedemann asociado a síndrome de qt largo]]></article-title>
<article-title xml:lang="en"><![CDATA[Anesthetic management to a pediatric patient with Beckwithwiedemann syndrome associated with long qt syndrome]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Aguirre-Salazar]]></surname>
<given-names><![CDATA[José Juan]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Mancera-Elías]]></surname>
<given-names><![CDATA[Gabriel]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Ortiz-Marmolejo]]></surname>
<given-names><![CDATA[Delfina]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Instituto Nacional de Pediatría  ]]></institution>
<addr-line><![CDATA[CDM ]]></addr-line>
<country>Mexico</country>
</aff>
<aff id="Af3">
<institution><![CDATA[,Instituto Nacional de Pediatría Departamento de Anestesiología ]]></institution>
<addr-line><![CDATA[CDM ]]></addr-line>
<country>Mexico</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>04</month>
<year>2019</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>04</month>
<year>2019</year>
</pub-date>
<volume>31</volume>
<numero>1</numero>
<fpage>43</fpage>
<lpage>50</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_arttext&amp;pid=S2448-87712019000100043&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_abstract&amp;pid=S2448-87712019000100043&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_pdf&amp;pid=S2448-87712019000100043&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[Resumen El síndrome de QT largo (SQTL) es un desorden hereditario asociado con muerte súbita caracterizado por una repolarización anómala y prolongada, produciendo un sustrato potencialmente peligroso en la génesis de taquicardia ventricular, síncope e incluso muerte súbita; eventos que pueden ser detonados por el estrés emocional o físico, los síntomas pueden presentarse súbitamente mediante convulsiones, síncope o muerte súbita como primera manifestación, esta entidad puede verse asociada con otras condiciones genéticas como el síndrome de Bekwitt-Widemann que se caracteriza por macrosomia, macroglosia, anomalías urogenitales y defecto de la pared abdominal. Se presenta el caso de una paciente femenina de 14 años con SQTL y Síndrome de Becwitt- Widemann, sometida a un procedimiento ortopédico que requirió anestesia, se analizan las implicaciones de la anestesia y la patología presentada.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[Abstract Long QT syndrome is an inherited disorder associated with sudden death characterized by abnormal and prolonged repolarization producing a potentially dangerous substrate in the genesis of ventricular tachycardia, syncope and even sudden death; Events that can be triggered by emotional or physical stress, the symptoms can suddenly appear by convulsions, syncope or sudden death as the first manifestation, this entity may be associated with other genetic conditions such as the Bekwitt Widemann syndrome that is characterized by macrosomia, macroglossia, urogenital anomalies and abdominal wall defect. The case of a 14-year-old female patient with long Q-T syndrome and Becwitt Widemann's syndrome submitted to an orthopedic procedure requiring anesthesia, the implications of anesthesia and the pathology presented.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[Síndrome Q-T Largo]]></kwd>
<kwd lng="es"><![CDATA[Síndrome de Becwitt Wideman]]></kwd>
<kwd lng="es"><![CDATA[anestesia]]></kwd>
<kwd lng="en"><![CDATA[Long Q-T síndrome]]></kwd>
<kwd lng="en"><![CDATA[Becwitt Widemann syndrome]]></kwd>
<kwd lng="en"><![CDATA[anesthesia]]></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[Kaltenbach]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Capril]]></surname>
<given-names><![CDATA[Y]]></given-names>
</name>
<name>
<surname><![CDATA[Rossignol]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Denjoy]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Beckwith-Wiedemann syndrome and long QT syndrome due to familial-balanced translocation t(11;17)(p15.5;q21.3) involving the KNCQ1 gene]]></article-title>
<source><![CDATA[Clinical Genetics]]></source>
<year>2013</year>
<volume>84</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>78-81</page-range></nlm-citation>
</ref>
<ref id="B2">
<label>2</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Gurrieti]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
<name>
<surname><![CDATA[Zollino]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Oliva]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Mild Beckwith-Wiedemann and severe long QT syndrome due to deletion of the imprinting center 2 on chomosome 11p]]></article-title>
<source><![CDATA[European Journal of Human Genetics]]></source>
<year>2013</year>
<volume>21</volume>
<page-range>965-9</page-range></nlm-citation>
</ref>
<ref id="B3">
<label>3</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Krönauer]]></surname>
<given-names><![CDATA[T]]></given-names>
</name>
<name>
<surname><![CDATA[Frlederich]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Long-QTSyndrom Historie, GenetiK, Klinische Syntome, Ursachen und Therapie]]></article-title>
<source><![CDATA[Der Anaesthesist]]></source>
<year>2015</year>
<volume>8</volume>
<page-range>586-95</page-range></nlm-citation>
</ref>
<ref id="B4">
<label>4</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Levine]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Rosero S]]></surname>
<given-names><![CDATA[Z]]></given-names>
</name>
<name>
<surname><![CDATA[Budzikowski A]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Congenital long QT syndrome: considerations for primary care physicians]]></article-title>
<source><![CDATA[Cleve Clin J Med]]></source>
<year>2008</year>
<volume>75</volume>
<numero>8</numero>
<issue>8</issue>
<page-range>591-600</page-range></nlm-citation>
</ref>
<ref id="B5">
<label>5</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Goldenberg]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[Moss]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Long QT Syndrome]]></article-title>
<source><![CDATA[Journal of American Coll of Cardiol]]></source>
<year>2008</year>
<volume>51</volume>
<numero>24</numero>
<issue>24</issue>
<page-range>2291-300</page-range></nlm-citation>
</ref>
<ref id="B6">
<label>6</label><nlm-citation citation-type="book">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Ch]]></surname>
<given-names><![CDATA[Shumann]]></given-names>
</name>
<name>
<surname><![CDATA[Becwith]]></surname>
<given-names><![CDATA[BJ]]></given-names>
</name>
<name>
<surname><![CDATA[Wecksberg]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Beckwith-Widemann Syndrome]]></article-title>
<person-group person-group-type="editor">
<name>
<surname><![CDATA[Adam]]></surname>
<given-names><![CDATA[MP]]></given-names>
</name>
<name>
<surname><![CDATA[Ardinger]]></surname>
<given-names><![CDATA[HH]]></given-names>
</name>
<name>
<surname><![CDATA[Pagon]]></surname>
<given-names><![CDATA[RA]]></given-names>
</name>
</person-group>
<source><![CDATA[Gene Reviews]]></source>
<year>1998</year>
<publisher-name><![CDATA[University of Washington]]></publisher-name>
</nlm-citation>
</ref>
<ref id="B7">
<label>7</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Whizar-Lugo]]></surname>
<given-names><![CDATA[V]]></given-names>
</name>
<name>
<surname><![CDATA[Singler]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Ontiveros]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Anestesia para glosectomia en un lactante con síndrome de Beckwith- Wiedemann]]></article-title>
<source><![CDATA[Anestesia en México]]></source>
<year>2006</year>
<volume>18</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>158-64</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[Whyte]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Natham]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Myers]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[The Safety of Modern Anesthesia for Children Whit Long QT Syndrome]]></article-title>
<source><![CDATA[Pedriatric anestheasia]]></source>
<year>2015</year>
<volume>59</volume>
<numero>4</numero>
<issue>4</issue>
<page-range>182-3</page-range></nlm-citation>
</ref>
<ref id="B9">
<label>9</label><nlm-citation citation-type="">
<collab>Genetics Home Reference</collab>
<source><![CDATA[]]></source>
<year>2015</year>
</nlm-citation>
</ref>
</ref-list>
</back>
</article>
