<?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>0138-6557</journal-id>
<journal-title><![CDATA[Revista Cubana de Medicina Militar]]></journal-title>
<abbrev-journal-title><![CDATA[Rev. cuban. med. mil.]]></abbrev-journal-title>
<issn>0138-6557</issn>
<publisher>
<publisher-name><![CDATA[Centro Nacional de Información de Ciencias MédicasEditorial Ciencias Médicas]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S0138-65572023000200006</article-id>
<title-group>
<article-title xml:lang="en"><![CDATA[Clinical-demographic characteristics of patient with primary immunodeficiencies]]></article-title>
<article-title xml:lang="es"><![CDATA[Características clínico-demográficas de pacientes con inmunodeficiencias primarias]]></article-title>
</title-group>
<contrib-group>
<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[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[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[Jiménez Hernández]]></surname>
<given-names><![CDATA[Edel]]></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[,Facultad de Ciencias Médicas de Bayamo Hospital Provincial Pediátrico Docente General Milanés ]]></institution>
<addr-line><![CDATA[ Granma]]></addr-line>
<country>Cuba</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Instituto de Ciencias Básicas y Preclínicas &#8220;Victoria de Girón&#8221;  ]]></institution>
<addr-line><![CDATA[ La Habana]]></addr-line>
<country>Cuba</country>
</aff>
<aff id="Af3">
<institution><![CDATA[,Facultad de Ciencias Médicas de Bayamo  ]]></institution>
<addr-line><![CDATA[ Granma]]></addr-line>
<country>Cuba</country>
</aff>
<aff id="Af4">
<institution><![CDATA[,Hospital Clínico Quirúrgico "Hermanos Ameijeiras"  ]]></institution>
<addr-line><![CDATA[ La Habana]]></addr-line>
<country>Cuba</country>
</aff>
<aff id="Af5">
<institution><![CDATA[,Hospital Provincial Universitario &#8220;Carlos Manuel de Céspedes&#8221; Centro Médico Ambulatorio ]]></institution>
<addr-line><![CDATA[Bayamo Granma]]></addr-line>
<country>Cuba</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>06</month>
<year>2023</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>06</month>
<year>2023</year>
</pub-date>
<volume>52</volume>
<numero>2</numero>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_arttext&amp;pid=S0138-65572023000200006&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_abstract&amp;pid=S0138-65572023000200006&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_pdf&amp;pid=S0138-65572023000200006&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="en"><p><![CDATA[ABSTRACT  Introduction: Primary immunodeficiencies are a heterogeneous group of genetic disorders that affect the functioning of the immune system. Primary immunodeficiencies can present with variable clinical features in early childhood and adulthood.  Objective:  To characterize patients diagnosed with primary immunodeficiencies clinically and demographically.  Methods:  A cross-sectional, descriptive, retrospective case series study was carried out with 39 patients of both sexes, diagnosed with primary immunodeficiencies, according to the latest International Union of Immunological Societies classification; attended from January 2012 to December 2021 in Granma. Age (at onset of symptoms, diagnosis, and delay in diagnosis), sex, family history, and clinical manifestations were evaluated. Data from medical records were collected. They were processed using descriptive statistics.  Results:  The age of onset of symptoms ranged from one month of life to 37 years, with a mean of 6.31 years; the average age of diagnosis was 11.89 years. Humoral deficiencies represented 69.23% of all primary immunodeficiencies and relative IgA deficiency predominated (11 cases). A family history of primary immunodeficiencies was reported by 12 patients. Recurrent infections (71.79%) and allergies (61.53%) were the most frequent manifestations; the mortality rate was 2.56%.  Conclusions:  Primary immunodeficiencies were more frequent in childhood, with the same behavior in both sexes. Infections, allergies, and a family history of primary immunodeficiency were important findings. Humoral immunodeficiencies were the most prevalent.]]></p></abstract>
<abstract abstract-type="short" xml:lang="es"><p><![CDATA[RESUMEN  Introducción:  Las inmunodeficiencias primarias son un grupo heterogéneo de trastornos genéticos que afectan el funcionamiento del sistema inmunológico. Pueden presentarse con características clínicas variables en la primera infancia y en la edad adulta.  Objetivo:  Caracterizar clínica y demográficamente a pacientes diagnosticados con inmunodeficiencias primarias.  Métodos:  Se realizó un estudio de serie de casos, transversal, descriptivo, retrospectivo, con 39 pacientes de ambos sexos, con diagnóstico de inmunodeficiencias primarias, según la última clasificación de la International Union of Immunological Societies; atendidos de enero de 2012 a diciembre de 2021 en Granma. Se evaluó la edad (de inicio de los síntomas, diagnóstico y retraso en el diagnóstico), sexo, antecedentes familiares y manifestaciones clínicas. Se recolectaron los datos de las historias clínicas. Se utilizó estadística descriptiva.  Resultados:  La edad de inicio de los síntomas osciló entre el mes de vida y los 37 años, con una media de 6,31 años; la edad media de diagnóstico fue de 11,89 años. Las deficiencias humorales representaron el 69,23 % de todas las inmunodeficiencias primarias y predominó el déficit relativo de IgA (11 casos). Reportaron antecedentes familiares de inmunodeficiencias primarias 12 pacientes. Las infecciones recurrentes (71,79 %) y las alergias (61,53 %) fueron las manifestaciones más frecuentes; la tasa de mortalidad fue de 2,56 %.  Conclusiones:  Las inmunodeficiencias primarias fueron más frecuentes en la infancia, con el mismo comportamiento en ambos sexos. Las infecciones, las alergias y los antecedentes familiares de inmunodeficiencia primaria fueron hallazgos importantes. Las inmunodeficiencias humorales fueron las más prevalentes.]]></p></abstract>
<kwd-group>
<kwd lng="en"><![CDATA[primary immune deficiency]]></kwd>
<kwd lng="en"><![CDATA[inborn errors of immunity]]></kwd>
<kwd lng="en"><![CDATA[primary immune deficiency registry]]></kwd>
<kwd lng="es"><![CDATA[inmunodeficiencia primaria]]></kwd>
<kwd lng="es"><![CDATA[errores congénitos de la inmunidad]]></kwd>
<kwd lng="es"><![CDATA[registro de inmunodeficiencia primaria]]></kwd>
</kwd-group>
</article-meta>
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