<?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>2413-4341</journal-id>
<journal-title><![CDATA[Revista Paraguaya de Reumatología]]></journal-title>
<abbrev-journal-title><![CDATA[Rev. parag. reumatol.]]></abbrev-journal-title>
<issn>2413-4341</issn>
<publisher>
<publisher-name><![CDATA[Sociedad Paraguaya de Reumatología]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S2413-43412025000100029</article-id>
<article-id pub-id-type="doi">10.18004/rpr/2025.11.01.29</article-id>
<title-group>
<article-title xml:lang="en"><![CDATA[Severe pericarditis as the first manifestation of a combination of genetic autoinflammatory disease and genetic anemia. Case report: Pericarditis and NLRP3 and G6PD]]></article-title>
<article-title xml:lang="es"><![CDATA[Pericarditis severa como primera manifestación de una combinación de enfermedad autoinflamatoria genética y anemia genética. Reporte de caso: Pericarditis y NLRP3 y G6PD]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Benítez]]></surname>
<given-names><![CDATA[Irene]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Ayala]]></surname>
<given-names><![CDATA[Zoilo Morel]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Lacarrubba]]></surname>
<given-names><![CDATA[Dora]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Rios]]></surname>
<given-names><![CDATA[Jorge]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Jiménez]]></surname>
<given-names><![CDATA[Hassel Jimmy]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Universidad Católica &#8220;Nuestra Señora de la Asunción Instituto de Previsión Social Hospital Central, Departamento de Pediatría]]></institution>
<addr-line><![CDATA[Asunción ]]></addr-line>
<country>Paraguay</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Universidad Católica &#8220;Nuestra Señora de la Asunción Instituto de Previsión Social Hospital Central, Departamento de Reumatología]]></institution>
<addr-line><![CDATA[Asunción ]]></addr-line>
<country>Paraguay</country>
</aff>
<aff id="Af3">
<institution><![CDATA[,Consultorio de Genética Centro Integral Dermatológico ]]></institution>
<addr-line><![CDATA[Asunción ]]></addr-line>
<country>Paraguay</country>
</aff>
<aff id="Af4">
<institution><![CDATA[,Sanatorio Británico  ]]></institution>
<addr-line><![CDATA[Asunción ]]></addr-line>
<country>Paraguay</country>
</aff>
<aff id="Af5">
<institution><![CDATA[,Universidad Nacional de Asunción Facultad de Ciencias Médicas, Hospital de Clínicas, Cátedra de Pediatría]]></institution>
<addr-line><![CDATA[San Lorenzo ]]></addr-line>
<country>Paraguay</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>06</month>
<year>2025</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>06</month>
<year>2025</year>
</pub-date>
<volume>11</volume>
<numero>1</numero>
<fpage>29</fpage>
<lpage>32</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.iics.una.py/scielo.php?script=sci_arttext&amp;pid=S2413-43412025000100029&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.iics.una.py/scielo.php?script=sci_abstract&amp;pid=S2413-43412025000100029&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.iics.una.py/scielo.php?script=sci_pdf&amp;pid=S2413-43412025000100029&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="en"><p><![CDATA[ ABSTRACT Pericarditis is one of the most common pericardial diseases. The activation of the NLRP3 (nucleotide-binding andoligomerization (NOD) like receptor (NLR) containing pyrin domain (NLRP) 3) inflammasome is central in the genesis of autoinflammatory pericarditis. Cryopyrin-associated periodic syndrome (CAPS) is a rare, heterogeneous disease associated with NLRP3 gene pathogenic variants and increased interleukin-1 (IL-1) secretion. Glucose-6-phosphate dehydrogenase (G6PD) deficiency in erythrocytes causes acute haemolytic anaemia during distress or chronic non responsive to iron anemia X-linked. We present the case of a male 1-year-9-months-old patient, with pericarditis and hemolytic anemia, diagnosed as inherited X-linked Glucose 6 Phosphate Dehydrogenase Deficiency p.[Val68Met;Asn126Asp] haplotype, and a heterozygous pathogenic variant in the NLRP3 gene: c.1315G&gt;A/p.Ala439Thr compatible with Autosomal Dominant Cryopyrin Associated Periodic Syndrome. This is the first Paraguayan patient with a &#8220;de novo&#8221; autoinflammatory condition coexisting with a glucose 6-phosphate dehydrogenase deficiency.]]></p></abstract>
<abstract abstract-type="short" xml:lang="es"><p><![CDATA[Resumen La pericarditis es una de las enfermedades pericárdicas más frecuentes. La activación del inflamasoma NLRP3 (receptor de unión a nucleótidos y oligomerización (NOD) similar a (NLR) que contiene el dominio pirina (NLRP) 3) es central en la génesis de la pericarditis autoinflamatoria. El síndrome periódico asociado a la criopirina (CAPS) es una enfermedad heterogénea y poco frecuente asociada con variantes patogénicas del gen NLRP3 y aumento de la secreción de interleucina-1 (IL-1). La deficiencia de glucosa-6-fosfato deshidrogenasa (G6PD) en los eritrocitos causa anemia hemolítica aguda durante algún distrés, o anemia crónica no respondedora a hierro, ligada al cromosoma X. Presentamos el caso de un paciente de sexo masculino de 1 año y 9 meses de edad, con pericarditis y anemia hemolítica, diagnosticado de deficiencia de glucosa 6 fosfato deshidrogenasa ligada al cromosoma X, haplotipo p.[Val68Met;Asn126Asp], y variante patogénica heterocigota en el gen NLRP3: c.1315G&gt;A/p.Ala439Thr, compatible con síndrome periódico autosómico dominante asociado a criopirina. Se trata del primer paciente paraguayo con un cuadro autoinflamatorio &#8220;de novo&#8221;, coexistente con una deficiencia de glucosa 6 fosfato deshidrogenasa.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[Deficiencia de glucosa 6 fosfato deshidrogenasa (G6PD)]]></kwd>
<kwd lng="es"><![CDATA[Síndrome periódico asociado a criopirina (CAPS)]]></kwd>
<kwd lng="es"><![CDATA[Pericarditis]]></kwd>
<kwd lng="es"><![CDATA[anemia hemolítica]]></kwd>
<kwd lng="en"><![CDATA[Glucose 6 Phosphate Dehydrogenase (G6PD) Deficiency]]></kwd>
<kwd lng="en"><![CDATA[Cryopyrin-associated periodic syndrome (CAPS)]]></kwd>
<kwd lng="en"><![CDATA[Pericarditis]]></kwd>
<kwd lng="en"><![CDATA[hemolytic anemia]]></kwd>
</kwd-group>
</article-meta>
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