<?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>1561-2953</journal-id>
<journal-title><![CDATA[Revista Cubana de Endocrinología]]></journal-title>
<abbrev-journal-title><![CDATA[Rev Cubana Endocrinol]]></abbrev-journal-title>
<issn>1561-2953</issn>
<publisher>
<publisher-name><![CDATA[Editorial Ciencias Médicas]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S1561-29532020000100007</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Pubertad en pacientes con hiperplasia adrenal congénita por déficit de 21hidroxilasa y asignación femenina]]></article-title>
<article-title xml:lang="en"><![CDATA[Puberty in patients with congenital adrenal hyperplasia due to a 21-hydroxylase deficit and female assignment]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Espinosa Reyes]]></surname>
<given-names><![CDATA[Tania M]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Leyva Cruz]]></surname>
<given-names><![CDATA[Daymí]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Domínguez Alonso]]></surname>
<given-names><![CDATA[Emma]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Vera Pérez]]></surname>
<given-names><![CDATA[Greter]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Agramonte Machado]]></surname>
<given-names><![CDATA[Adriana]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Instituto Nacional de Endocrinología (INEN)  ]]></institution>
<addr-line><![CDATA[La Habana ]]></addr-line>
<country>Cuba</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Hospital Militar Dr. Luis Díaz Soto  ]]></institution>
<addr-line><![CDATA[La Habana ]]></addr-line>
<country>Cuba</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>04</month>
<year>2020</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>04</month>
<year>2020</year>
</pub-date>
<volume>31</volume>
<numero>1</numero>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_arttext&amp;pid=S1561-29532020000100007&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_abstract&amp;pid=S1561-29532020000100007&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_pdf&amp;pid=S1561-29532020000100007&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[RESUMEN  Introducción:  En la hiperplasia adrenal congénita el aumento de los niveles de andrógenos suprarrenales en las pacientes no tratadas o mal controladas, puede alterar el inicio y/o la progresión puberal (progresión puberal/progresiones puberales?).  Objetivos:  Describir las características puberales de pacientes con hiperplasia adrenal congénita asignadas como femeninas e identificar si existe asociación entre elementos relacionados con la enfermedad y el inicio y progresión puberales.  Métodos:  Se incluyeron todas las pacientes con diagnóstico de hiperplasia adrenal congénita asignadas como femeninas, que fueron atendidas en el INEN de enero 2000 a mayo 2019.  Resultados:  Fueron estudiadas 47 pacientes, con una media de edad de 14,76 ± 7,04 años. Se comprobó un predominio de las formas clínicas clásicas en 25 pacientes (53,19 %), de ellas 11 (23,40 %) fueron formas virilizantes simples, 14 (29,78 %) perdedoras de sal y 22 (46,80 %) formas no clásicas. El inicio del vello pubiano fue a una edad promedio de 7,78 ± 3,2 años. El comienzo de la telarquia resultó en una media de 10,09 ± 2,4 años y la menarquia a los 12,2 ± 2,3 años como promedio. De las 29 pacientes que ya habían menstruado 16 (55,2 %) presentaban irregularidades menstruales. El tiempo entre el inicio puberal y la menarquia fue de 3,4 años en las formas no clásicas, 5,6 años en las perdedoras de sal y 7,0 años en las virilizantes simples. La edad al diagnóstico, la edad de inicio del tratamiento y la dosis de esteroides empleada se relacionaron con algunos aspectos puberales.  Conclusiones:  El diagnóstico oportuno y el ajuste cuidadoso del esquema esteroideo, constituyen pilares importantes en el inicio y progresión puberales, y en la consecución de ciclos ovulatorios regulares que aseguren desde la adolescencia, un inicio y desarrollo puberales normales y en edades reproductivas, la optimización de la fertilidad.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[ABSTRACT  Introduction:  In the congenital adrenal hyperplasia, the increased levels of adrenal androgens in patients untreated or poorly controlled can alter the start and/or pubertal progression (pubertal progression/pubertal progressions).  Objectives:  To describe the pubertal characteristics of patients with congenital adrenal hyperplasia assigned as females and to identify whether there is an association between elements related to the disease and the pubertal onset and progression.  Methods:  There were included all patients diagnosed with congenital adrenal hyperplasia assigned as females that were attended at the National Institute of Endocrinology from January 2000 to May 2019.  Results:  47 patients were studied, with an average age of 14.76 ± 7.04 years. It was found a predominance of classic clinical forms in 25 patients (53.19 %), of which 11 (23.40 %) had simple virilization forms, 14 (29.78 %) were salt-losers and 22 (46.80 %) had non-classical forms. The onset of the pubic hair was at an average age of 7.78 ± 3.2 years. The beginning of the thelarche resulted in an average of 10.09 ± 2.4 years and menarche at the 12.2 ± 2.3 years on average. Of the 29 patients who had menstruated, 16 (55.2 %) presented menstrual irregularities. The time between the puberty onset and menarche was 3.4 years in the non-classical forms, 5.6 years in the salt-losers, and 7.0 years in the simple virilizations. The age at initial diagnosis treatment and the dose of steroids used were related to some pubertal aspects.  Conclusions:  Early diagnosis and careful adjustment of the steroid scheme are important pillars in the pubertal onset and progression, the achievement of regular ovulatory cycles, and with it, in the optimization of fertility.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[pubertad]]></kwd>
<kwd lng="es"><![CDATA[hiperplasia adrenal congénita]]></kwd>
<kwd lng="en"><![CDATA[Puberty]]></kwd>
<kwd lng="en"><![CDATA[congenital adrenal hyperplasia]]></kwd>
</kwd-group>
</article-meta>
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