<?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>S1683-98032018000200127</article-id>
<article-id pub-id-type="doi">10.31698/ped.45022018004</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Características clínicas y manifestaciones infecciosas en pacientes con Enfermedad Granulomatosa Crónica (EGC) en Paraguay]]></article-title>
<article-title xml:lang="en"><![CDATA[Clinical characteristics and infectious manifestations in patients with Chronic Granulomatous Disease (CGD) in Paraguay]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Martínez de Cuellar]]></surname>
<given-names><![CDATA[Celia]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Lovera]]></surname>
<given-names><![CDATA[Dolores]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Amarilla]]></surname>
<given-names><![CDATA[Sara]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Gatti]]></surname>
<given-names><![CDATA[Luis]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Apodaca]]></surname>
<given-names><![CDATA[Silvio]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<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[Arbo]]></surname>
<given-names><![CDATA[Antonio]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Instituto de Medicina Tropical Servicio de Pediatría ]]></institution>
<addr-line><![CDATA[Asunción ]]></addr-line>
<country>Paraguay</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Universidad Nacional de Asunción  ]]></institution>
<addr-line><![CDATA[San Lorenzo ]]></addr-line>
<country>Paraguay</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>08</month>
<year>2018</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>08</month>
<year>2018</year>
</pub-date>
<volume>45</volume>
<numero>2</numero>
<fpage>127</fpage>
<lpage>134</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.iics.una.py/scielo.php?script=sci_arttext&amp;pid=S1683-98032018000200127&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.iics.una.py/scielo.php?script=sci_abstract&amp;pid=S1683-98032018000200127&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.iics.una.py/scielo.php?script=sci_pdf&amp;pid=S1683-98032018000200127&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[RESUMEN  Introducción:  La enfermedad granulomatosa crónica (EGC) es causada por un defecto en una de las subunidades de nicotinamida fosfato (NADPH) oxidasa, produciendo en los fagocitos una falla en la producción del superóxido. Como resultado, los pacientes presentan infecciones bacterianas y fúngicas recurrentes.  Objetivos:  analizar las características clínicas, manifestaciones infecciosas, los microrganismos aislados, así como la evolución de los pacientes que fueron diagnosticados en el Instituto de Medicina Tropical desde en el periodo 1991 - 2017.  Materiales y Métodos:  fueron incluidos pacientes con diagnóstico de EGC. Se revisaron los expedientes clínicos de pacientes con EGC hospitalizados, se determinaron las características clínico-demográficas, incluyendo la edad del inicio de las manifestaciones clínicas, edad de diagnóstico, antecedentes de consanguinidad en la familia, historia familiar de cuadros similares, manifestaciones clínicas, frecuencia de cuadros infecciosos, complicaciones asociadas a la vacuna BCG, días de hospitalización y evolución de la enfermedad.  Resultados:  se identificaron 11 pacientes (8 varones y 3 mujeres). Todos los pacientes pertenecían a 8 familias, se identificaron 2 familias con más de un miembro afectado (1 familia con 2 hermanas y otra con 2 primos y una tía afectados). Dos de los 11 pacientes tenían antecedentes de familiares con muerte prematura dentro de las 3 generaciones. La edad media de inicio de síntomas fue de 14.5±10,3 meses y al momento del diagnóstico de 32.4±25,06 meses. En un paciente se identificó antecedentes de consanguinidad, aunque todos recibieron la vacuna BCG, ninguno presento historia de becegeitis. La media de cuadros infecciosos que requirieron hospitalización fue de 3±1,7. Durante el periodo de estudio, los pacientes presentaron 38 cuadros infecciosos. Las infecciones pulmonares fueron las más frecuentemente observadas 42,1% (16/38), incluyendo las neumonías (9/38), absceso pulmonar (1/38), tuberculosis pulmonar (1/38) y bronquiolitis (14/38). Otros cuadros infecciosos que requirieron hospitalización fueron las sepsis 18,4% (7/38), uno de ellos con endocarditis, adenitis 13,2% (5/38), infecciones de piel y partes blandas 13,2% (5/38), meningitis bacteriana y osteomielitis 2,6% (1/38), respectivamente. En 19 de los cuadros infecciosos se aisló el microrganismo causante, siendo el más frecuente el S. aureus (n=9), pero en 10 casos se aisló un microrganismo inusual [Aspergillus (n = 4), Chromobacterium violaceum, Burkholderia cepacia, Enterobacter cloacae, Serratia marcescens, Mycobacterium tuberculosis (uno en cada caso]. El tiempo medio de hospitalización fue de 23,3±14,1 días y la mortalidad acumulada de 3/11.  Conclusiones:  Coincidente con otras series la mayoría de los casos de EGC, en Paraguay, se observó en varones, y la edad de inicio de síntomas fue antes de los 2 años de edad. El diagnóstico de la enfermedad fue relativamente precoz. El sitio infeccioso más frecuente fue el pulmonar, siendo el microorganismo más frecuentemente aislado el S. aureus, aunque se aislaron microorganismos inusuales en una proporción significativa. Es fundamental considerar esta ID en pacientes con cuadros infecciosos de evolución tórpida y por microorganismos inusuales.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[ABSTRACT  Introduction:  Chronic granulomatous disease (CGD) is caused by a defect in one of the subunits of nicotinamide phosphate (NADPH) oxidase, producing a failure in the production of superoxide in phagocytes. As a result, patients have recurrent bacterial and fungal infections.  Objectives:  to analyze the clinical characteristics, infectious manifestations, the isolated microorganisms, as well as the evolution of patients that were diagnosed at the Institute of Tropical Medicine from 1991 to 2017.  Materials and Methods:  patients with a diagnosis of CGD were included. Clinical records of hospitalized CGD patients were reviewed, clinical-demographic characteristics were determined, including the age of onset of clinical manifestations, age of diagnosis, family history of consanguinity, family history of similar conditions, clinical manifestations, frequency of infectious diseases, complications associated with the BCG vaccine, hospitalization days and disease evolution.  Results:  11 patients were identified (8 men and 3 women). All the patients belonged to 8 families, 2 families were identified who had more than one affected member (1 family with 2 sisters and another with 2 affected cousins and one aunt). Two of the 11 patients had a family history of premature death within 3 generations. The mean age of symptom onset was 14.5 ± 10.3 months and age at the time of diagnosis was 32.4 ± 25.06 months. In one patient, a history of consanguinity was identified; although all patients received the BCG vaccine, none presented a history of BCG adenitis. The mean number of infectious cases that required hospitalization was 3 ± 1.7. During the study period, patients presented with 38 infectious conditions. Pulmonary infections were the most frequently observed 42.1% (16/38), including pneumonia (9/38), lung abscess (1/38), pulmonary tuberculosis (1/38) and bronchiolitis (14/38). Other infectious conditions that required hospitalization were sepsis 18.4% (7/38), one of them with endocarditis, adenitis 13.2% (5/38), skin and soft tissue infections 13.2% (5/38), bacterial meningitis and osteomyelitis 2.6% (1/38), respectively. In 19 of the infectious conditions, the causative microorganism was isolated, the most frequent being S. aureus (n = 9), but in 10 cases an unusual microorganism was isolated [Aspergillus (n = 4), Chromobacterium violaceum, Burkholderia cepacia, Enterobacter cloacae, Serratia marcescens, Mycobacterium tuberculosis (one in each case.)] The mean hospitalization time was 23.3 ± 14.1 days and the accumulated mortality of 3/11.  Conclusions:  Coinciding with other series, most cases of CGD in Paraguay were observed in males, and the age of onset of symptoms was before 2 years of age.The diagnosis of the disease was made relatively early. The most frequent infectious site were the lungs, with the microorganism most frequently isolated being S. aureus, although unusual microorganisms were isolated in a significant number of cases. It is essential to consider this diagnosis in patients with recurrent bouts of infectious conditions and caused by unusual microorganisms.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[Enfermedad granulomatosa crónica]]></kwd>
<kwd lng="es"><![CDATA[inmunodeficiencias Primarias]]></kwd>
<kwd lng="en"><![CDATA[Chronic granulomatous disease]]></kwd>
<kwd lng="en"><![CDATA[primary immunodeficiencies]]></kwd>
</kwd-group>
</article-meta>
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