<?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>1665-1146</journal-id>
<journal-title><![CDATA[Boletín médico del Hospital Infantil de México]]></journal-title>
<abbrev-journal-title><![CDATA[Bol. Med. Hosp. Infant. Mex.]]></abbrev-journal-title>
<issn>1665-1146</issn>
<publisher>
<publisher-name><![CDATA[Instituto Nacional de Salud, Hospital Infantil de México Federico Gómez]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S1665-11462015000500325</article-id>
<article-id pub-id-type="doi">10.1016/j.bmhimx.2015.08.003</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Incontinencia pigmenti con defecto en la inmunidad celular]]></article-title>
<article-title xml:lang="en"><![CDATA[Incontinentia pigmenti with defect in cellular immunity]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Zamora-Chávez]]></surname>
<given-names><![CDATA[Antonio]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Escobar-Sánchez]]></surname>
<given-names><![CDATA[Argelia]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Sadowinski-Pine]]></surname>
<given-names><![CDATA[Stanislaw]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Saucedo-Ramírez]]></surname>
<given-names><![CDATA[Omar Josué]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Delgado-Barrera]]></surname>
<given-names><![CDATA[Palmira]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Enríquez-Quiñones]]></surname>
<given-names><![CDATA[Claudia G.]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Hospital Infantil de México Federico Gómez Departamento de Medicina Interna ]]></institution>
<addr-line><![CDATA[México D.F.]]></addr-line>
<country>MX</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Hospital Infantil de México Federico Gómez Departamento de Patología ]]></institution>
<addr-line><![CDATA[México D.F.]]></addr-line>
<country>MX</country>
</aff>
<aff id="Af3">
<institution><![CDATA[,Hospital Infantil de México Federico Gómez Departamento de Alergia e Inmunología Clínica ]]></institution>
<addr-line><![CDATA[México D.F.]]></addr-line>
<country>MX</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>10</month>
<year>2015</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>10</month>
<year>2015</year>
</pub-date>
<volume>72</volume>
<numero>5</numero>
<fpage>325</fpage>
<lpage>332</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_arttext&amp;pid=S1665-11462015000500325&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_abstract&amp;pid=S1665-11462015000500325&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_pdf&amp;pid=S1665-11462015000500325&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[ResumenIntroducción: La incontinencia pigmenti es una enfermedad genética rara ligada al cromosoma X, letal en el varón, que afecta a todos los tejidos derivados del ectodermo, como piel, faneras, ojos, dientes y sistema nervioso central, y presenta alteraciones de grado variable en la inmunidad celular. Se caracteriza por la disminución de la melanina en la epidermis y su incremento en la dermis.Caso clínico: Se presenta el caso de una lactante de dos meses de edad con incontinencia pigmenti grave, confirmada con estudio histopatológico de piel, que cursó con alteraciones neurológicas severas y crisis convulsivas. Además, presentó inmunodeficiencia celular grave que condicionó el desarrollo de infecciones que le ocasionaron la muerte.Conclusiones: Se resalta la importancia del diagnóstico clínico temprano, así como la importancia del manejo multidisciplinario de las alteraciones neurológicas y de las complicaciones infecciosas.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[AbstractBackground: Incontinentia pigmenti is a rare, X-linked genetic disease and affects all ectoderm-derived tissues such as skin, appendages, eyes, teeth and central nervous system as well as disorders of varying degree of cellular immunity characterized by decreasing melanin in the epidermis and increase in the dermis. When the condition occurs in males, it is lethal.Case report: We present the case of a 2-month-old infant with severe incontinentia pigmenti confirmed by histological examination of skin biopsy. The condition evolved with severe neurological disorders and seizures along with severe cellular immune deficiency, which affected the development of severe infections and caused the death of the patient.Conclusions: The importance of early clinical diagnosis is highlighted along with the importance of multidisciplinary management of neurological disorders and infectious complications.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[Incontinencia pigmenti]]></kwd>
<kwd lng="es"><![CDATA[Inmunodeficiencia celular]]></kwd>
<kwd lng="es"><![CDATA[Sepsis]]></kwd>
<kwd lng="en"><![CDATA[Incontinentia pigmenti]]></kwd>
<kwd lng="en"><![CDATA[Cellular immunodeficiency]]></kwd>
<kwd lng="en"><![CDATA[Sepsis]]></kwd>
</kwd-group>
</article-meta>
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