<?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>0186-2391</journal-id>
<journal-title><![CDATA[Acta pediátrica de México]]></journal-title>
<abbrev-journal-title><![CDATA[Acta pediatr. Méx]]></abbrev-journal-title>
<issn>0186-2391</issn>
<publisher>
<publisher-name><![CDATA[Instituto Nacional de Pediatría]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S0186-23912018000100033</article-id>
<article-id pub-id-type="doi">10.18233/apm1no1pp33-411538</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Síndrome de Meckel-Gruber en un lactante menor con sobrevida prolongada. Reporte de Caso]]></article-title>
<article-title xml:lang="en"><![CDATA[Gruber Meckel Syndrome in a young infant with prolonged survival. Case Report]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Ramírez-Izcoa]]></surname>
<given-names><![CDATA[AE.]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Sánchez-Sierra]]></surname>
<given-names><![CDATA[LE.]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Alvarenga-Calidonio]]></surname>
<given-names><![CDATA[RH.]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Varela-González]]></surname>
<given-names><![CDATA[D.]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Universidad Nacional Autónoma de Honduras  ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Honduras</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Universidad Nacional Autónoma de Honduras  ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Honduras</country>
</aff>
<aff id="Af3">
<institution><![CDATA[,Universidad Nacional Autónoma de Honduras  ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Honduras</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>02</month>
<year>2018</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>02</month>
<year>2018</year>
</pub-date>
<volume>39</volume>
<numero>1</numero>
<fpage>33</fpage>
<lpage>41</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_arttext&amp;pid=S0186-23912018000100033&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_abstract&amp;pid=S0186-23912018000100033&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_pdf&amp;pid=S0186-23912018000100033&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[Resumen El síndrome de Meckel-Gruber es una ciliopatía debida a la disfunción del cilio celular; un trastorno autosómico recesivo letal que se caracteriza por una triada clásica de manifestaciones: displasia renal quística bilateral, meningoencefalocele occipital y polidactilia postaxial. Se presenta el caso de una lactante que al nacer presentó la triada del síndrome de Meckel-Gruber y otras alteraciones, entre las que destacó la aplasia bilateral del nervio óptico. A las 72 horas de vida fue sometida a exéresis de meningoencefalocele occipital con evolución satisfactoria, continuó con tratamiento por infecciones urinarias a repetición debido a displasia quística renal, también padeció infecciones respiratorias y falleció a los 11 meses de vida. Pese a la alta mortalidad del síndrome de Meckel-Gruber en algunos casos, poco frecuentes como éste, la sobrevida de extiende, por lo que es necesario un tratamiento multidisciplinario para tratar las complicaciones.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[Abstract Meckel-Gruber Syndrome is a ciliopathy caused by dysfunction of primary cilia, is a lethal autosomal recessive disorder that is diagnosed by the presence of a classical triad manifestations: bilateral cystic renal dysplasia, occipital meningoencephalocele and postaxial polydactyly. We present a case of a female infant, who at birth presented the triad Syndrome Meckel Gruber and other conditions, among which highlighted the bilateral aplasia of the optic nerve. The patient underwent exceresis of occipital meningoencephalocele at 72 hours of life, with satisfactory evolution; she continued with treatment for urinary secondary to the renal cystic dysplasia infections, also presented respiratory infections and died at 11 months. Despite the high mortality Meckel Gruber syndrome, in unusual cases like this, they have longer survival, so it is necessary to establish a multidisciplinary approach to treat complications.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[meningoencefalocele]]></kwd>
<kwd lng="es"><![CDATA[polidactilia]]></kwd>
<kwd lng="es"><![CDATA[ciliopatía]]></kwd>
<kwd lng="es"><![CDATA[síndrome de Meckel-Gruber]]></kwd>
<kwd lng="en"><![CDATA[meningoencephalocele]]></kwd>
<kwd lng="en"><![CDATA[polydactylylism]]></kwd>
<kwd lng="en"><![CDATA[ciliopathy]]></kwd>
</kwd-group>
</article-meta>
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