<?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>1683-9803</journal-id>
<journal-title><![CDATA[Pediatría (Asunción)]]></journal-title>
<abbrev-journal-title><![CDATA[Pediatr. (Asunción)]]></abbrev-journal-title>
<issn>1683-9803</issn>
<publisher>
<publisher-name><![CDATA[Sociedad Paraguaya de Pediatría]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S1683-98032017000100049</article-id>
<article-id pub-id-type="doi">10.18004/ped.2017.abril.49-55</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Estallido respiratorio de neutrófilos por las técnicas del nitroazul tetrazolio (NBT) y dihidrorodamina (DHR) en niños con sospecha clínica de enfermedad granulomatosa crónica (EGC)]]></article-title>
<article-title xml:lang="en"><![CDATA[Neutrophil`s respiratory burst by nitroblue tetrazolium (NBT) and dihydrorhodamine (DHR) techniques in children with clinical suspicion of chronic granulomatous disease (CGD)]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Sanabria]]></surname>
<given-names><![CDATA[Diana]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Giménez]]></surname>
<given-names><![CDATA[Vivian]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Carpinelli]]></surname>
<given-names><![CDATA[María Mercedes]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Martínez de Cuellar]]></surname>
<given-names><![CDATA[Celia]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Universidad Nacional de Asunción Instituto de Investigaciones en Ciencias de la Salud Departamento de Inmunología]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Paraguay</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Universidad Nacional de Asunción Facultad de Ciencias Médicas ]]></institution>
<addr-line><![CDATA[Asunción ]]></addr-line>
<country>Paraguay</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>04</month>
<year>2017</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>04</month>
<year>2017</year>
</pub-date>
<volume>44</volume>
<numero>1</numero>
<fpage>49</fpage>
<lpage>55</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.iics.una.py/scielo.php?script=sci_arttext&amp;pid=S1683-98032017000100049&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.iics.una.py/scielo.php?script=sci_abstract&amp;pid=S1683-98032017000100049&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.iics.una.py/scielo.php?script=sci_pdf&amp;pid=S1683-98032017000100049&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[RESUMEN  Introducción:  El estallido respiratorio (ER) de neutrófilos es fundamental para la defensa contra infecciones, proceso ausente o ineficaz en la EGC, una inmunodeficiencia primaria (IDP) diagnosticada mediante la prueba del NBT. Actualmente se destacan las técnicas por citometría de flujo como la DHR, realizada únicamente en el Instituto de Investigaciones en Ciencias de la Salud (IICS), habiendo sido aplicada sólo en niños sanos.  Objetivo:  Evaluar el ER de neutrófilos por las técnicas NBT y DHR en niños con sospecha clínica de EGC y describir sus características clínico-demográficas.  Materiales y Métodos:  Se incluyeron 36 niños de ambos sexos, menores de 17 años de edad, remitidos al IICS entre el 2014-2015 por médicos especialistas. Se extrajo sangre para evaluación del ER de neutrófilos y se aplicó un cuestionario.  Resultados:  La mediana de edad fue de 4 años, 56% varones. Predominaron los pacientes hospitalizados, la sepsis y forunculosis cutánea fueron las manifestaciones clínicas más frecuentes y un 72% presentó recurrencia de infecciones con mediana de 3 episodios/año. El promedio para el IE de neutrófilos fue de 38,1±13,7 en el ensayo DHR, y 87±17% de activación para la prueba del NBT. En 8 pacientes los valores fueron inferiores al considerado normal y en un niño se confirmó EGC, observándose un patrón de herencia ligada al X.  Conclusión:  La evaluación del ER de neutrófilos permitió detectar un caso de EGC, determinándose el patrón hereditario mediante la técnica DHR por primera vez en el país. Es esencial el empleo de herramientas diagnósticas disponibles en niños con sospecha clínica de IDPs, para la detección y tratamiento oportunos que mejoran su calidad de vida y reducen la mortalidad.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[ABSTRACT  Introduction: The neutrophil`s respiratory burst (RB) is essential for the defense against infections, this process is absent or ineffective in the CGD, a primary immunodeficiency (PID) diagnosed by the NBT test. Techniques that used flow cytometry such as DHR, performed only at the Instituto de Investigaciones en Ciencias de la Salud (IICS), currently stand out, having been applied only to healthy children.  Objective:  To evaluate the neutrophil`s RB using the NBT and DHR techniques in children with clinical suspicion of CGD and to describe their clinical and demographic characteristics.  Materials and methods:  36 children of both sexes, with less than 17 years of age, that were referred to the IICS by specialists physicians between the years 2014-2015 were included. A blood sample was obtained to evaluate the neutrophil`s RB and a questionnaire was applied.  Results:  The median age was of 4 years and 56 % were males. Predominantly the patients were hospitalized, being sepsis and cutanueos furunculosis the most frequent clinical manifestations and a 72 % presented recurrent infection with a median of 3 episodes/year. The average for the neutrophil´s stimulation index (EI) was 38,1±13,7 with the DHR test, and 87±17% of activation for the NBT test. In 8 patients the values obtained were below the ones considered as normal and in one child CGD was confirmed, in which an X-linked inheritance pattern was observed.  Conclusion:  The evaluation of the neutrophil`s RB allowed the detection of one case of CGD, and the inheritance pattern was determined by the DHR test for the first time in our country. The use of available diagnostic tools in children with clinical suspicion of PID is essential for the appropriate detection and treatment that improve the quality of life and reduce mortality.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[Estallido respiratorio]]></kwd>
<kwd lng="es"><![CDATA[neutrófilos]]></kwd>
<kwd lng="es"><![CDATA[NBT]]></kwd>
<kwd lng="es"><![CDATA[DHR]]></kwd>
<kwd lng="es"><![CDATA[citometría de flujo]]></kwd>
<kwd lng="es"><![CDATA[EGC]]></kwd>
<kwd lng="es"><![CDATA[herencia ligada al X.]]></kwd>
<kwd lng="en"><![CDATA[respiratory burst]]></kwd>
<kwd lng="en"><![CDATA[neutrophils]]></kwd>
<kwd lng="en"><![CDATA[NBT]]></kwd>
<kwd lng="en"><![CDATA[DHR]]></kwd>
<kwd lng="en"><![CDATA[flow cytometry]]></kwd>
<kwd lng="en"><![CDATA[CGD]]></kwd>
<kwd lng="en"><![CDATA[X-linked inheritance.]]></kwd>
</kwd-group>
</article-meta>
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