<?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>S0186-23912017000500322</article-id>
<article-id pub-id-type="doi">10.18233/apm38no5pp322-3291472</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Crisis neuropática por suspensión de nitisinona en una paciente con tirosinemia: informe de un caso]]></article-title>
<article-title xml:lang="en"><![CDATA[Neurologic crises after interruption of nitisinone in a tyrosinemia patient: case report]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Ibarra-González]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Belmont-Martínez]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Cervantes-Bustamante]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Zárate-Mondragón]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Guillén-López]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Fernández-Lainez]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Revilla-Estivil]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Vela-Amieva]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Instituto de Investigaciones Biomédicas Unidad de genética de la Nutrición ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Mexico</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Instituto Nacional de Pediatría Laboratorio de Errores Innatos del Metabolismo y Tamiz ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Mexico</country>
</aff>
<aff id="Af3">
<institution><![CDATA[,Instituto Nacional de Pediatría Servicio de Gastronutrición ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Mexico</country>
</aff>
<aff id="Af4">
<institution><![CDATA[,Instituto Nacional de Pediatría Departamento de urgencias ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Mexico</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>10</month>
<year>2017</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>10</month>
<year>2017</year>
</pub-date>
<volume>38</volume>
<numero>5</numero>
<fpage>322</fpage>
<lpage>329</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_arttext&amp;pid=S0186-23912017000500322&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_abstract&amp;pid=S0186-23912017000500322&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_pdf&amp;pid=S0186-23912017000500322&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[Resumen Se presenta el caso de una paciente con tirosinemia hepatorrenal (TYR-1) que, debido a la interrupción por cuatro semanas de tratamiento con nitisinona, tuvo una grave crisis neurológica de pseudoporfiria caracterizada por vómito, dolor abdominal, irritabilidad e hipertensión arterial, con gran elevación de la succinilacetona y alfafetoproteína. La crisis requirió tratamiento intrahospitalario. Una semana después de reiniciar la administración del fármaco revirtió totalmente. Este caso enfatiza la importancia de mantener el tratamiento ininterrumpido con nitisinona en pacientes con tirosinemia hepatorrenal. También es útil para reconocer el cuadro clínico y la fisiopatología de las crisis neurológicas características de esta enfermedad.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[Abstract We present the case of a female patient with hepatorenal tyrosinemia who presented a severe porphyria-like neurologic crisis four weeks after interrupting nitisinone treatment. The crisis was characterized by vomiting, abdominal pain, irritability, and high blood pressure, with elevation of blood succinylacetone and alpha-fetoprotein. The crisis required hospitalization and reverted totally one week after the reintroduction of nitisinone. This clinical case emphasizes the importance of maintaining nitisinone treatment uninterruptedly. This paper is useful for recognizing the clinical picture of the neurologic crises of TYR-1 and its physiopathology.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[tirosinemia hepatorrenal]]></kwd>
<kwd lng="es"><![CDATA[nitisinona]]></kwd>
<kwd lng="es"><![CDATA[succinilacetona]]></kwd>
<kwd lng="es"><![CDATA[crisis neurológica]]></kwd>
<kwd lng="en"><![CDATA[hepatorenal tyrosinemia]]></kwd>
<kwd lng="en"><![CDATA[nitisinone]]></kwd>
<kwd lng="en"><![CDATA[NTBC]]></kwd>
<kwd lng="en"><![CDATA[succinylacetone]]></kwd>
<kwd lng="en"><![CDATA[neurologic crises]]></kwd>
</kwd-group>
</article-meta>
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