<?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>0034-7531</journal-id>
<journal-title><![CDATA[Revista Cubana de Pediatría]]></journal-title>
<abbrev-journal-title><![CDATA[Rev Cubana Pediatr]]></abbrev-journal-title>
<issn>0034-7531</issn>
<publisher>
<publisher-name><![CDATA[Centro Nacional de Información de Ciencias MédicasEditorial Ciencias Médicas]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S0034-75312023000100026</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Supervivencia en pacientes con fibrosis quística]]></article-title>
<article-title xml:lang="en"><![CDATA[Survival in Patients with Cystic Fibrosis]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Vázquez Gutiérrez]]></surname>
<given-names><![CDATA[Giselle Lucila]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Sariol González]]></surname>
<given-names><![CDATA[Pedro Alberto]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Calás Torres]]></surname>
<given-names><![CDATA[Jimmy Javier]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[de la Rosa Santana]]></surname>
<given-names><![CDATA[Jesús Daniel]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Martínez Marreiro]]></surname>
<given-names><![CDATA[Luis Armando]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Vázquez Palanco]]></surname>
<given-names><![CDATA[Julio Roberto]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Universidad de Ciencias Médicas de Granma  ]]></institution>
<addr-line><![CDATA[ Granma]]></addr-line>
<country>Cuba</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Hospital Clínico Quirúrgico &#8220;Celia Sánchez Manduley&#8221;  ]]></institution>
<addr-line><![CDATA[ Granma]]></addr-line>
<country>Cuba</country>
</aff>
<aff id="Af3">
<institution><![CDATA[,Policlínico Docente &#8220;René Vallejo Ortiz&#8221;  ]]></institution>
<addr-line><![CDATA[ Granma]]></addr-line>
<country>Cuba</country>
</aff>
<aff id="Af4">
<institution><![CDATA[,Hospital Pediátrico Docente &#8220;Hermanos Cordové&#8221;  ]]></institution>
<addr-line><![CDATA[ Granma]]></addr-line>
<country>Cuba</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>00</month>
<year>2023</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>00</month>
<year>2023</year>
</pub-date>
<volume>95</volume>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_arttext&amp;pid=S0034-75312023000100026&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_abstract&amp;pid=S0034-75312023000100026&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_pdf&amp;pid=S0034-75312023000100026&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[RESUMEN  Introducción: La fibrosis quística ha dejado de ser una enfermedad pediátrica para ser una enfermedad crónica del adulto. Existen múltiples factores que condicionan la supervivencia de estos enfermos.  Objetivo:  Determinar la sobrevida de pacientes pediátricos con fibrosis quística hospitalizados en un centro especializado.  Métodos: Estudio retrospectivo de cohorte cuyo universo lo conformaron 27 pacientes incluidos en el Registro Provincial de Fibrosis Quística de Granma, 2003-2018. Se analizaron variables: edad actual y sexo, variante genética, presencia de gérmenes, formas clínicas, y supervivencia a partir del diagnóstico y tratamiento. Se utilizó el método de Kaplan-Meier y Log-Rank Test, para un nivel de significación de 95 %.  Resultados:  El 40,7 % de los pacientes declaró tener actualmente más de 15 años, predominó el sexo masculino (63 %), y la mutación F508del en su variante genética heterocigótica (44,4 %). El promedio de supervivencia resultó en 21,4 años, no existieron diferencias significativas según sexo, formas clínicas y tipos de gérmenes.  Conclusiones: Los pacientes con fibrosis quística en Granma, Cuba mostraron un promedio de supervivencia de 21,4 años, inferior a lo documentado en países desarrollados. El Estado cubano garantiza recursos para la atención de estos pacientes, a pesar de las dificultades económicas del país.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[ABSTRACT  Introduction:  Cystic fibrosis is no longer a pediatric disease but a chronic adult disease. There are multiple factors that condition the survival of these patients.  Objective:  To determine the survival of pediatric patients with cystic fibrosis hospitalized in a specialized center.  Methods:  Retrospective cohort study whose universe consisted of 27 patients included in the Granma provincial cystic fibrosis registry, 2003-2018. Variables were analyzed: current age and gender, genetic variant, presence of germs, clinical forms and survival after diagnosis and treatment. The Kaplan-Meier method and Log-Rank Test were used, for a significance level of 95%.  Results:  40.7% of patients are currently over 15 years of age, male sex predominates (63%) and the F508del mutation in its heterozygous genetic variant (44.4%). The average survival is 21.4 years, there were no significant differences according to gender, clinical forms and types of germs.  Conclusions:  The average survival rate of patients with cystic fibrosis in Granma, Cuba, is 21.4 years, lower than that documented in developed countries. The Cuban State guarantees resources for the care of these patients, despite the country's economic difficulties.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[fibrosis quística]]></kwd>
<kwd lng="es"><![CDATA[enfermedades genéticas]]></kwd>
<kwd lng="es"><![CDATA[análisis de supervivencia]]></kwd>
<kwd lng="es"><![CDATA[sobrevida]]></kwd>
<kwd lng="es"><![CDATA[tiempo medio de supervivencia]]></kwd>
<kwd lng="es"><![CDATA[mutación]]></kwd>
<kwd lng="en"><![CDATA[cystic fibrosis]]></kwd>
<kwd lng="en"><![CDATA[genetic diseases]]></kwd>
<kwd lng="en"><![CDATA[survival analysis]]></kwd>
<kwd lng="en"><![CDATA[survival]]></kwd>
<kwd lng="en"><![CDATA[mean survival time]]></kwd>
<kwd lng="en"><![CDATA[mutation]]></kwd>
</kwd-group>
</article-meta>
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