<?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>S0186-23912016000100017</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Calidad de vida de los pacientes con inmunodeficiencias primarias de anticuerpos]]></article-title>
<article-title xml:lang="en"><![CDATA[Quality of life in patientes with antibody immunodeficiencies]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Muriel-Vizcaíno]]></surname>
<given-names><![CDATA[Rodolfo]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Treviño-Garza]]></surname>
<given-names><![CDATA[Gabriela]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Murata]]></surname>
<given-names><![CDATA[Chiharu]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Staines-Boone]]></surname>
<given-names><![CDATA[Aidé Tamara]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Yamazaki-Nakashimada]]></surname>
<given-names><![CDATA[Marco Antonio]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Espinosa-Padilla]]></surname>
<given-names><![CDATA[Sara Elba]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Espinosa-Rosales]]></surname>
<given-names><![CDATA[Francisco Javier]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Instituto Nacional de Pediatría  ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>México</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Hospital Regional Materno Infantil de Alta Especialidad Servicio de Inmunología ]]></institution>
<addr-line><![CDATA[Monterrey ]]></addr-line>
</aff>
<aff id="Af3">
<institution><![CDATA[,Instituto Nacional de Pediatría Departamento de Metodología de la Investigación ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>México</country>
</aff>
<aff id="Af4">
<institution><![CDATA[,Instituto Mexicano del Seguro Social Unidad Médica de Alta Especialidad 25 Servicio de Inmunología]]></institution>
<addr-line><![CDATA[Monterrey Nuevo León]]></addr-line>
<country>Mexico</country>
</aff>
<aff id="Af5">
<institution><![CDATA[,Instituto Nacional de Pediatría Servicio de Inmunología ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>México</country>
</aff>
<aff id="Af6">
<institution><![CDATA[,Instituto Nacional de Pediatría Unidad de Inmunología Clínica y Alergia ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>México</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>02</month>
<year>2016</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>02</month>
<year>2016</year>
</pub-date>
<volume>37</volume>
<numero>1</numero>
<fpage>17</fpage>
<lpage>25</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_arttext&amp;pid=S0186-23912016000100017&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_abstract&amp;pid=S0186-23912016000100017&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_pdf&amp;pid=S0186-23912016000100017&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[RESUMEN: Las inmunodeficiencias primarias son enfermedades genéticas caracterizadas, en la mayoría de los casos, por la predisposición del individuo afectado a infecciones recurrentes o graves con afectación de su calidad de vida. Las más frecuentes son las de defectos en la producción de anticuerpos. Cuando no se hace un diagnóstico y tratamiento oportunos puede ocurrir daño permanente a órganos blanco afectando aún más la calidad de vida.  Objetivo:  evaluar la calidad de vida de los pacientes con inmunodeficiencias primarias por defectos en la producción de anticuerpos y compararla con la de controles sanos.  Material y métodos:  estudio transversal analítico mediante la aplicación del instrumento "Pediatric Quality of Life Inventory" (Peds-QL) en su versión validada en español mexicano. La diferencia del puntaje de PedsQL entre niños con inmunodeficiencias primarias y controles sanos se determinó por la prueba de t de Student. El efecto del retraso en el diagnóstico fue evaluado por el modelo de análisis de covarianza. Se incluyeron 28 pacientes. La mediana de edad fue 5 años y 5 meses.  Resultados:  el promedio de edad al diagnóstico fue de 6 años con 3 meses. La mediana de retraso diagnóstico fue de 3 años 3 meses. El promedio de calidad de vida en los pacientes fue de 74.1 (DE ± 13.8) y de 83.3 (DE ± 10.1) para los controles (p = 0.005). La relación entre calidad de vida, retraso diagnóstico y presencia de complicaciones se analizó mediante un modelo lineal que fue marginalmente significativo (p = 0.056). Se encontró correlación negativa entre concentraciones de IgG en el último año y la calidad de vida. Se observó que, en los pacientes que presentaban bronquiectasias, conforme aumentan los años de retraso en el diagnóstico la calidad de vida disminuye de forma significativa (p = 0.007).  Conclusiones:  consideramos necesario evaluar la calidad de vida para conocer el estado de salud de los pacientes con inmunodeficiencias primarias y para su seguimiento durante el tratamiento con el objeto de incidir no sólo en la reducción del número de infecciones, hospitalizaciones y complicaciones, sino también en la mejoría de su calidad de vida. Deberá diseñarse un instrumento específico para evaluar la calidad de vida de los pacientes con inmunodeficiencias primarias.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[ABSTRACT Primary immunodeficiencies (PID) are genetic diseases affecting im-munological normal response. In the great majority of cases affected individual sare predisposed to recurrent and / or severe infections with impaired quality of life (QoL). The most frequent PID are defects in the production of antibodies. When diagnosis and treatment are not done properly, permanent damage may occur in affected organs and further affecting QoL.  Objective:  To evaluate QoL in patients with antibody PID compared with QoL of healthy controls.  Methods:  Cross-sectional study with the application of the instrument "Pediatric Quality of Life Inventory" (PedsQL) in its Mexican Spanish validated version. The PedsQL score difference between children with PID and healthy children was determined by the Student t test. The effect of a delayed in diagnosis was evaluated by analysis in a covari-ance model. 28 patients were included. The median age was 5 years and 5 months.  Results:  The average age at diagnosis was 6 years and 3 months. The median diagnostic delay was 3 years 3 months. The average QoL in the patients was 74.1 (SD ± 13.8) and 83.3 (SD ± 10.1) for controls (p = 0.005). The relationship between QoL, delayed diagnosis and presence of complications was analyzed using a linear model that was marginally significant (p = 0.056). Negative correlation between levels of IgG in the last year and the quality of life was found. It was observed that in patients with bronchiectasis, QoL decreased significantly as the duration of delayed diagnosis (p = 0.007).  Conclusions.  We consider necessary to assess QoL of patients with PID and to monitoring it during treatment in order to have an impact not only in reducing the number of infections, hospitalizations and complications, but also in improving their QoL. It should be designed a QoL measuring instrument specific for patients with PID]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[calidad de vida]]></kwd>
<kwd lng="es"><![CDATA[inmunodeficiencias de anticuerpos]]></kwd>
<kwd lng="es"><![CDATA[inmunodeficiencias primarias]]></kwd>
<kwd lng="en"><![CDATA[Quality of life]]></kwd>
<kwd lng="en"><![CDATA[Primary Immunodeficiencies]]></kwd>
<kwd lng="en"><![CDATA[Antibody immunodeficiencies]]></kwd>
</kwd-group>
</article-meta>
</front><back>
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