<?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>2448-9190</journal-id>
<journal-title><![CDATA[Revista alergia México]]></journal-title>
<abbrev-journal-title><![CDATA[Rev. alerg. Méx.]]></abbrev-journal-title>
<issn>2448-9190</issn>
<publisher>
<publisher-name><![CDATA[Colegio Mexicano de Inmunología Clínica y Alergia A.C.]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S2448-91902020000300268</article-id>
<article-id pub-id-type="doi">10.29262/ram.v67i3.738</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Registro de errores innatos de la inmunidad en un hospital pediátrico]]></article-title>
<article-title xml:lang="en"><![CDATA[Registry of Inborn errors of immunity in a pediatric hospital]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[García-Domínguez]]></surname>
<given-names><![CDATA[Miguel]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Valero-Gálvez]]></surname>
<given-names><![CDATA[Guadalupe del Carmen]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Velázquez-Ríos]]></surname>
<given-names><![CDATA[Carlos Alberto]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Blancas-Galicia]]></surname>
<given-names><![CDATA[Lizbeth]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Secretaría de Salud del Estado de Sinaloa Hospital Pediátrico de Sinaloa Departamento de Inmunología Clínica]]></institution>
<addr-line><![CDATA[ Sinaloa]]></addr-line>
<country>México</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Secretaría de Salud del Estado de Sinaloa Hospital Pediátrico de Sinaloa Departamento de Inmunogenética]]></institution>
<addr-line><![CDATA[ Sinaloa]]></addr-line>
<country>México</country>
</aff>
<aff id="Af3">
<institution><![CDATA[,Secretaría de Salud del Estado de Sinaloa Hospital Pediátrico de Sinaloa Departamento de Infectología]]></institution>
<addr-line><![CDATA[ Culiacán]]></addr-line>
<country>México</country>
</aff>
<aff id="Af4">
<institution><![CDATA[,Instituto Nacional de Pediatría Unidad de Investigación de Inmunodeficiencias ]]></institution>
<addr-line><![CDATA[ Ciudad de México]]></addr-line>
<country>México</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>09</month>
<year>2020</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>09</month>
<year>2020</year>
</pub-date>
<volume>67</volume>
<numero>3</numero>
<fpage>268</fpage>
<lpage>278</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_arttext&amp;pid=S2448-91902020000300268&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_abstract&amp;pid=S2448-91902020000300268&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_pdf&amp;pid=S2448-91902020000300268&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[Resumen El Hospital Pediátrico de Sinaloa (HPS) inició el registro de pacientes con inmunodeficiencia primaria o error innato de la inmunidad (EII) en la plataforma de la Sociedad Latinoamericana de Inmunodeficiencias Primarias (LASID) desde 2017. Durante ese periodo se han diagnosticado y tratado 12 casos en el hospital. El rango de edad al momento del diagnóstico fue de dos días a 16 años y el rango de inicio de los síntomas de 19 días a cuatro años, con predominio del sexo masculino (67 %). El EII más frecuente fue la deficiencia predominantemente de anticuerpos (33.3 %), seguida de defectos en fagocitos en número o función (16.6 %), desórdenes auto inflamatorios (16.6 %), inmunodeficiencias que afectan la inmunidad celular y humoral (16.6 %), inmunodeficiencias combinadas asociadas con los hallazgos sindromáticos (8.3 %) y defectos en la inmunidad intrínseca e innata (8.3 %). El 84 % recibió inmunoglobulina intravenosa; se identificó la variante patogénica en el gen WAS en un caso con síndrome de Wiskott-Aldrich; un paciente recibió trasplante de células progenitoras hematopoyética; 33.3 % falleció, 25 % por sepsis y 8.3 % por hemorragia masiva. El registro de las EII permite conocer datos epidemiológicos, incidencia, prevalencia, diagnósticos y tratamientos, lo que favorecerá al desarrollo de nuevas políticas sanitarias para la obtención de recursos y herramientas para mejorar los modelos de atención.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[Abstract In 2017, the Pediatric Hospital of Sinaloa (PHS) began its affiliation to the registry of patients with primary immunodeficiency or inborn errors of immunity (IEI) on the platform of the Latin American Society for Immunodeficiencies (LASID). During this period, twelve cases with IEI have been diagnosed and treated at the hospital. The age category at the time of diagnosis varied from two days to sixteen years old, and the range of the onset of the symptoms varied from nineteen days to four years, with a predominance of males (67%). The most frequent IEI was predominantly antibody deficiency (33.3%), followed by defects in the number or function of phagocytes (16.6%), autoinflammatory disorders (16.6%), immunodeficiencies that affect cellular and humoral immunity (16.6%), combined immunodeficiencies associated with syndromic findings (8.3%), and defects in intrinsic and inborn immunity (8.3%). 84% of patients received intravenous immunoglobulin and, in one case of a patient with Wiskott-Aldrich syndrome, a pathogenic variant in the WAS gene was identified; a patient received hematopoietic stem cell transplantation, 33.3% of patients died, of which 25% died of sepsis and 8.3% died of massive hemorrhage. The registry of IEI provides information about epidemiological data, incidences, prevalence, diagnoses, and treatments, which will favor the development of new health policies for obtaining resources and tools to improve the care models.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[Inmunodeficiencias primarias]]></kwd>
<kwd lng="es"><![CDATA[Errores innatos de la inmunidad]]></kwd>
<kwd lng="es"><![CDATA[Enfermedad de inmunodeficiencia]]></kwd>
<kwd lng="es"><![CDATA[Infecciones recurrentes]]></kwd>
<kwd lng="en"><![CDATA[Primary immunodeficiencies]]></kwd>
<kwd lng="en"><![CDATA[Inborn errors of immunity]]></kwd>
<kwd lng="en"><![CDATA[immunodeficiency disease]]></kwd>
<kwd lng="en"><![CDATA[recurrent infections]]></kwd>
</kwd-group>
</article-meta>
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