<?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>0212-1611</journal-id>
<journal-title><![CDATA[Nutrición Hospitalaria]]></journal-title>
<abbrev-journal-title><![CDATA[Nutr. Hosp.]]></abbrev-journal-title>
<issn>0212-1611</issn>
<publisher>
<publisher-name><![CDATA[Grupo Arán]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S0212-16112024000300025</article-id>
<article-id pub-id-type="doi">10.20960/nh.04867</article-id>
<title-group>
<article-title xml:lang="en"><![CDATA[Two pregnancies of an ornithine carbamoyltransferase deficiency disease carrier and review of the literature]]></article-title>
<article-title xml:lang="es"><![CDATA[Dos embarazos de una portadora de la enfermedad por deficiencia de ornitina transcarbamilasa (OTC) y revisión de la literatura]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Arhip]]></surname>
<given-names><![CDATA[Loredana]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Agreda]]></surname>
<given-names><![CDATA[Javier]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Serrano-Moreno]]></surname>
<given-names><![CDATA[Clara]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Motilla]]></surname>
<given-names><![CDATA[Marta]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Carrascal]]></surname>
<given-names><![CDATA[M.ª Luisa]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Bielza]]></surname>
<given-names><![CDATA[Atocha]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Velasco]]></surname>
<given-names><![CDATA[Cristina]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Camblor]]></surname>
<given-names><![CDATA[Miguel]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Bretón]]></surname>
<given-names><![CDATA[Irene]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Cuerda]]></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="A3"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Hospital General Universitario Gregorio Marañón Nutrition Unit ]]></institution>
<addr-line><![CDATA[Madrid ]]></addr-line>
<country>Spain</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Instituto de Investigación Sanitaria Gregorio Marañón  ]]></institution>
<addr-line><![CDATA[Madrid ]]></addr-line>
<country>Spain</country>
</aff>
<aff id="Af3">
<institution><![CDATA[,Universidad Complutense de Madrid Department of Medicine ]]></institution>
<addr-line><![CDATA[Madrid ]]></addr-line>
<country>Spain</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>04</month>
<year>2024</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>04</month>
<year>2024</year>
</pub-date>
<volume>41</volume>
<numero>2</numero>
<fpage>489</fpage>
<lpage>509</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.isciii.es/scielo.php?script=sci_arttext&amp;pid=S0212-16112024000300025&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.isciii.es/scielo.php?script=sci_abstract&amp;pid=S0212-16112024000300025&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.isciii.es/scielo.php?script=sci_pdf&amp;pid=S0212-16112024000300025&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="en"><p><![CDATA[Abstract  Background: the underlying cause of the deficiency of ornithine carbamoyltransferase (OTCD) is a gene mutation on the X chromosome. In females, the phenotype is highly variable, ranging from asymptomatic to neurologic compromise secondary to hyperammonemia and it can be prompted by numerous triggers, including pregnancy.  Objective: the objective of this article is to report a case of two pregnancies of an OTCD-carrier, and to review the literature describing OTCD and pregnancy, parturition and postpartum.  Methods: an extensive search in PubMed in December 2021 was conducted using different search terms. After screening all abstracts, 23 papers that corresponded to our inclusion criteria were identified.  Results: the article focuses on the management of OTCD during pregnancy, parturition, and the postpartum period in terms of clinical presentation, ammonia levels and treatment.  Conclusions: females with OTCD can certainly plan a pregnancy, but they need a careful management during delivery and particularly during the immediate postpartum period. If possible, a multidisciplinary team of physicians, dietitians, obstetrician-gynecologist, neonatologists, pharmacists, etc. with expertise in this field should participate in the care of women with OTCD and their children during this period and in their adult life.]]></p></abstract>
<abstract abstract-type="short" xml:lang="es"><p><![CDATA[Resumen  Antecedentes: la causa subyacente de la deficiencia de ornitina transcarbamilasa (OTC) es una mutación genética en el cromosoma X. En las mujeres, el fenotipo es muy variable, desde asintomático hasta presentar un compromiso neurológico secundario a hiperamonemia, y puede ser provocado por numerosos factores desencadenantes, incluido el embarazo.  Objetivo: el objetivo de este artículo es reportar un caso de dos embarazos de una portadora de OTC, y revisar la literatura que describe OTC y embarazo, parto y posparto.  Métodos: se realizó una búsqueda exhaustiva en PubMed en diciembre de 2021 utilizando diferentes términos de búsqueda. Después de examinar todos los resúmenes, identificamos 23 artículos que correspondían a nuestros criterios de inclusión.  Resultados: el artículo se centra en el manejo de la OTC durante el embarazo, el parto y el posparto en términos de presentación clínica, niveles de amonio y tratamiento.  Conclusiones: las mujeres con OTC pueden planificar un embarazo, pero necesitan un manejo cuidadoso durante el parto, y particularmente, durante el posparto inmediato. Si es posible, un equipo multidisciplinar de médicos, dietistas, ginecólogos-obstetras, neonatólogos, farmacéuticos, etc., con experiencia en este campo, debe participar en el cuidado de las mujeres con OTC y sus hijos durante este periodo y en su vida adulta.]]></p></abstract>
<kwd-group>
<kwd lng="en"><![CDATA[Hyperammonemia]]></kwd>
<kwd lng="en"><![CDATA[OTC deficiency]]></kwd>
<kwd lng="en"><![CDATA[Pregnancy]]></kwd>
<kwd lng="en"><![CDATA[Parturition]]></kwd>
<kwd lng="en"><![CDATA[Treatment]]></kwd>
<kwd lng="es"><![CDATA[Hiperamonemia]]></kwd>
<kwd lng="es"><![CDATA[Deficiencia de OTC]]></kwd>
<kwd lng="es"><![CDATA[Embarazo]]></kwd>
<kwd lng="es"><![CDATA[Parto]]></kwd>
<kwd lng="es"><![CDATA[Tratamiento]]></kwd>
</kwd-group>
</article-meta>
</front><back>
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