<?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>1561-3194</journal-id>
<journal-title><![CDATA[Revista de Ciencias Médicas de Pinar del Río]]></journal-title>
<abbrev-journal-title><![CDATA[Rev Ciencias Médicas]]></abbrev-journal-title>
<issn>1561-3194</issn>
<publisher>
<publisher-name><![CDATA[Editorial Ciencias Médicas]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S1561-31942023000600019</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Inmunodeficiencias primarias por déficit de IgA. Heterogeneidad clínica y reto diagnóstico]]></article-title>
<article-title xml:lang="en"><![CDATA[Primary immunodeficiencies due to IgA deficiency. Clinic heterogeneity and diagnostic challenge]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Pérez-Acevedo]]></surname>
<given-names><![CDATA[Lidia Cecilia]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[González-Costa]]></surname>
<given-names><![CDATA[Maricarmen]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Addine-Ramírez]]></surname>
<given-names><![CDATA[Bárbara de la Caridad]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Miló-Valdés]]></surname>
<given-names><![CDATA[Carlos Alfredo]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Marrón-González]]></surname>
<given-names><![CDATA[Reynel]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Universidad de Ciencias Médicas de La Habana. Instituto Superior de Ciencias Básicas y Preclínicas &#8220;Victoria de Girón&#8221; ]]></institution>
<addr-line><![CDATA[La Habana, ]]></addr-line>
<country>Cuba</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Universidad de Ciencias Médicas de Granma. Hospital Provincial Pediátrico Docente &#8220;General Milanés&#8221; de Bayamo. ]]></institution>
<addr-line><![CDATA[Granma ]]></addr-line>
<country>Cuba</country>
</aff>
<aff id="Af3">
<institution><![CDATA[,Universidad de Ciencias Médicas de Granma. Facultad de Ciencias Médicas de Bayamo ]]></institution>
<addr-line><![CDATA[Granma ]]></addr-line>
<country>Cuba</country>
</aff>
<aff id="Af4">
<institution><![CDATA[,Universidad de Ciencias Médicas de Pinar del Río. Hospital Pediátrico Provincial Docente &#8220;Pepe Portilla&#8221;. ]]></institution>
<addr-line><![CDATA[Pinar del Río, ]]></addr-line>
<country>Cuba</country>
</aff>
<aff id="Af5">
<institution><![CDATA[,Universidad de Ciencias Médicas de Granma. Hospital Provincial Universitario &#8220;Carlos Manuel de Céspedes&#8221; ]]></institution>
<addr-line><![CDATA[Granma ]]></addr-line>
<country>Cuba</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>10</month>
<year>2023</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>10</month>
<year>2023</year>
</pub-date>
<volume>27</volume>
<numero>5</numero>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_arttext&amp;pid=S1561-31942023000600019&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_abstract&amp;pid=S1561-31942023000600019&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_pdf&amp;pid=S1561-31942023000600019&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[RESUMEN  Introducción:  la deficiencia selectiva de inmunoglobulina A (IgA) es la inmunodeficiencia primaria (IDP) más frecuente; puede pasar desapercibida provocando falsos diagnósticos y reacciones inexplicables a hemoderivados. Los datos sobre las principales características de los pacientes con IDP por déficit de IgA (IDP-IgA) son relativamente escasos y su estudio es crucial para una mejor identificación y manejo de la enfermedad.  Objetivo:  caracterizar las IDP-IgA en pacientes atendidos en el servicio de Inmunología y Alergia del Hospital Provincial Universitario &#8220;Carlos Manuel de Céspedes&#8221; de Bayamo-Granma, en el periodo 2013-2022.  Métodos:  se realizó un estudio descriptivo, retrospectivo, en el total de pacientes diagnosticados con IDP-IgA parcial y total (22). Se estudiaron variables demográficas, manifestaciones clínicas, antecedentes patológicos familiares y tipo de déficit de IgA.  Resultados:  la edad media de aparición de los primeros síntomas fue tres años. El 54,5 % de los pacientes diagnosticados con IDP-IgA fueron del sexo femenino y el 68,2 % presentó un déficit parcial. El 77,2 %, 68,2 % y el 31,8 % del total de casos presentaron alergias, infecciones y autoinmunidad, respectivamente. Las alergias como antecedentes patológicos familiares se reportaron en el 59,1 % de los pacientes y las IDP en el 27,3 %.  Conclusiones:  las IDP-IgA en Granma se caracterizaron por el predominio de la variante de déficit parcial, mayor incidencia en el sexo femenino, el debut en edades pediátricas de los casos sintomáticos y el reporte de enfermedades alérgicas como manifestación clínica y antecedente patológico personal más frecuente.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[ABSTRACT  Introduction:  selective immunoglobulin A (IgA) deficiency is the most common primary immunodeficiency (PID); it can go unnoticed causing false diagnoses and unexplained reactions to blood products. Data on the main characteristics of patients with PID due to IgA deficiency (PID-IgA) are relatively scarce; hence, this knowledge is crucial for a better identification and management of this disease.  Objective:  to characterize patients with primary immunodeficiency due to IgA deficit in patients diagnosed with PID-IgA, assisted in the Immunology and Allergy service of "Carlos Manuel de Céspedes" Provincial University Hospital, in Bayamo-Granma, from 2013 to 2022.  Methods:  a descriptive, retrospective study was conducted in the total of patients (22) diagnosed with PID-IgA (partial and total). Demographic variables were studied, as well as other such as clinical features, familial antecedents and type of IgA deficiency.  Results:  mean age of symptoms onset was three years. 54,5 % of patients diagnosed with PID-IgA were female and 68,2 % suffered from a partial deficit. Allergies, infections and autoimmunity presented in 77,2 %, 68,2 % and 31,8 % of total cases, respectively. Family history of allergies was reported in 59,1 % of patients and familial PID in 27,3 %.  Conclusions:  The main features that characterized PID-IgA in Granma were a greater incidence in females, predominance of the partial deficiency type, clinical onset in childhood for symptomatic patients and the report of allergic diseases as the most frequently reported clinical feature and familial antecedent.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[DÉFICIT SELECTIVO DE IGA]]></kwd>
<kwd lng="es"><![CDATA[INMUNODEFICIENCIA PRIMARIA]]></kwd>
<kwd lng="es"><![CDATA[ERROR INNATO DE LLA INMUNIDAD]]></kwd>
<kwd lng="es"><![CDATA[DÉFICIT DE IGA]]></kwd>
<kwd lng="en"><![CDATA[SELECTIVE IGA DEFICIENCY]]></kwd>
<kwd lng="en"><![CDATA[PRIMARY IMMUNODEFICIENCY DISEASES]]></kwd>
<kwd lng="en"><![CDATA[INBORN ERROR OF IMMUNITY]]></kwd>
<kwd lng="en"><![CDATA[IGA DEFICIENCY]]></kwd>
</kwd-group>
</article-meta>
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