<?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>0871-9721</journal-id>
<journal-title><![CDATA[Revista Portuguesa de Imunoalergologia]]></journal-title>
<abbrev-journal-title><![CDATA[Rev Port Imunoalergologia]]></abbrev-journal-title>
<issn>0871-9721</issn>
<publisher>
<publisher-name><![CDATA[Sociedade Portuguesa de Alergologia e Imunologia Clínica]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S0871-97212023000300217</article-id>
<article-id pub-id-type="doi">10.32932/rpia.2023.08.117</article-id>
<title-group>
<article-title xml:lang="pt"><![CDATA[Infeção por SARS-CoV-2 nos doentes com agamaglobulinemia ligada ao X]]></article-title>
<article-title xml:lang="en"><![CDATA[SARS-CoV-2 infection in X-linked agammaglobulinemia patients]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Varandas]]></surname>
<given-names><![CDATA[Cláudia]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
<xref ref-type="aff" rid="A a"/>
<xref ref-type="aff" rid="A3"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Presa]]></surname>
<given-names><![CDATA[Ana Rita]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Ferreira]]></surname>
<given-names><![CDATA[Ruben Duarte]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
<xref ref-type="aff" rid="A a"/>
<xref ref-type="aff" rid="A5"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Silva]]></surname>
<given-names><![CDATA[Sara P.]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
<xref ref-type="aff" rid="A a"/>
<xref ref-type="aff" rid="A3"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Silva]]></surname>
<given-names><![CDATA[Susana L.]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
<xref ref-type="aff" rid="A a"/>
<xref ref-type="aff" rid="A3 "/>
<xref ref-type="aff" rid="Af6"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Centro Hospitalar Universitário Lisboa Norte Serviço de Imunoalergologia ]]></institution>
<addr-line><![CDATA[Lisboa ]]></addr-line>
<country>Portugal</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Centro Académico de Medicina Centro de Imunodeficiências Primárias ]]></institution>
<addr-line><![CDATA[Lisboa ]]></addr-line>
<country>Portugal</country>
</aff>
<aff id="Af3">
<institution><![CDATA[,Universidade de Lisboa Faculdade de Medicina Instituto de Medicina Molecular João Lobo Antunes]]></institution>
<addr-line><![CDATA[Lisboa ]]></addr-line>
<country>Portugal</country>
</aff>
<aff id="Af4">
<institution><![CDATA[,Centro Hospitalar de Vila Nova de Gaia e Espinho Serviço de Imunoalergologia ]]></institution>
<addr-line><![CDATA[Vila Nova de Gaia ]]></addr-line>
<country>Portugal</country>
</aff>
<aff id="Af5">
<institution><![CDATA[,Centro Hospitalar do Oeste Unidade de Imunoalergologia ]]></institution>
<addr-line><![CDATA[Caldas da Rainha ]]></addr-line>
<country>Portugal</country>
</aff>
<aff id="Af6">
<institution><![CDATA[,Universidade de Lisboa Faculdade de Medicina Clínica Universitária de Imunoalergologia]]></institution>
<addr-line><![CDATA[Lisboa ]]></addr-line>
<country>Portugal</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>09</month>
<year>2023</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>09</month>
<year>2023</year>
</pub-date>
<volume>31</volume>
<numero>3</numero>
<fpage>217</fpage>
<lpage>226</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_arttext&amp;pid=S0871-97212023000300217&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_abstract&amp;pid=S0871-97212023000300217&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_pdf&amp;pid=S0871-97212023000300217&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="pt"><p><![CDATA[RESUMO  Fundamentos:  A patogénese da infeção por SARS-CoV-2 tem sido extensamente investigada, constituindo um desafio à escala global. É sabido que a eficiência da resposta da imunidade inata e adquirida à infeção por SARS-CoV-2 condiciona a gravidade da apresentação clínica. É ainda escassa a literatura sobre a evolução da infeção por SARS-CoV-2 nos doentes com agamaglobulinemia ligada ao X.  Objetivo:  Reportar o espetro de manifestações clínicas associadas à infeção por SARS-CoV-2 em doentes com agamaglobulinemia ligada ao X e sua gravidade.  Métodos: Estudo observacional retrospetivo dos doentes com agamaglobulinemia ligada ao X em seguimento num hospital terciário e com infeção por SARS-CoV-2, diagnosticada entre março de 2020 e julho de 2022.  Resultados:  De um total de 13 doentes em seguimento com agamaglobulinemia ligada ao X, foram incluídos 9, com média de idades de 32±6,1 anos, entre os quais se identificaram 2 doentes com uma segunda infeção por SARS-CoV-2 durante o período em estudo. Todos os doentes estavam sob terapêutica de substituição com imunoglobulina polivalente G. Sete doentes apresentavam fatores de risco para doença COVID-19 grave: bronquiectasias (n=5), diabetes insulinodependente (n=1), tabagismo ativo (n=1), obesidade (n=1), doença cerebrovascular (n=1) e terapêutica imunossupressora (n=1). A infeção associou-se a manifestações clínicas de baixa gravidade na maioria dos doentes (infeção assintomática n=1, ligeira n=9, moderada n=1). Os sistemas mais afetados foram as vias áreas superiores (n=6) e inferiores (n=5), sendo a febre (n=7) e a obstrução nasal (n=6) as manifestações mais frequentes. Registou-se sobreinfeção bacteriana em 4 doentes. Um doente apresentou COVID-19 de gravidade moderada e duração prolongada, possivelmente em relação com comorbilidades médicas, tendo apresentado um curso clínico favorável com a administração de plasma de convalescentes.  Conclusão:  Reporta-se nesta série de doentes com agamaglobulinemia ligada ao X elevada prevalência de infeção por SARS-CoV-2, destacando-se a evolução de gravidade ligeira na quase totalidade dos doentes.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[ABSTRACT  Background:  The pathogenesis of infection triggered by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has been intensively investigated, constituting a challenge on a global scale. It is known that the effectiveness and magnitude of both innate and acquired immune responses to SARS-CoV-2 infection determine the severity of the clinical presentation. Literature on the evolution of SARS-CoV-2 infection in patients with X-linked agammaglobulinemia is still scarce.  Objective:  To report the spectrum of clinical manifestations associated with SARS-CoV-2 infection in patients with X-linked followed in a tertiary hospital.  Methods:  This is an observational retrospective study of patients with X-linked agammaglobulinemia with SARS-CoV-2 infection diagnosed between March 2020 and July 2022.  Results:  Among 13 patients under follow-up, nine patients were included, with a mean age of 32 ± 6.1 years. Two patients reported a second SARS-CoV-2 infection during the study. All patients were on polyvalent immunoglobulin G replacement therapy. Seven patients presented risk factors for severe COVID-19 disease: bronchiectasis (n=5), insulin-dependent diabetes (n=1), active smoking (n=1), obesity (n=1), cerebrovascular disease (n=1), immunosuppressive therapy (n=1). COVID-19 infection was mild in most patients (asymptomatic infection n=1, mild n=9, moderate n=1). The most frequently affected systems were the upper (n=6) and lower (n=5) respiratory airways, with fever (n=7) and nasal congestion (n=6) being the most frequent manifestations. Bacterial infection was reported in 4 patients. One patient underwent therapy with convalescent plasma, with tolerance and a favorable clinical course.  Conclusion:  In this series of patients with X-linked agammaglobulinemia, a high prevalence of SARS-CoV-2 infection is reported, highlighting a mild severity outcome in almost all patients.]]></p></abstract>
<kwd-group>
<kwd lng="pt"><![CDATA[Agamaglobulinemia ligada ao X]]></kwd>
<kwd lng="pt"><![CDATA[COVID-19]]></kwd>
<kwd lng="pt"><![CDATA[SARS-CoV-2]]></kwd>
<kwd lng="en"><![CDATA[COVID-19]]></kwd>
<kwd lng="en"><![CDATA[SARS-CoV-2]]></kwd>
<kwd lng="en"><![CDATA[X-Linked Agammaglobulinemia]]></kwd>
</kwd-group>
</article-meta>
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