<?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>S1561-31942021000600018</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Forma clásica virilizante simple grado IV de Hiperplasia suprarrenal congénita: reporte de un caso]]></article-title>
<article-title xml:lang="en"><![CDATA[Simple classical virilizing form grade-IV congenital adrenal hyperplasia: case report]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Suárez-García]]></surname>
<given-names><![CDATA[Nuvia]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Piloña-Ruiz]]></surname>
<given-names><![CDATA[Sergio Germán]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Universidad de Ciencias Médicas de Pinar del Río Hospital General Docente Abel Santamaría Cuadrado ]]></institution>
<addr-line><![CDATA[Pinar del Río ]]></addr-line>
<country>Cuba</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>12</month>
<year>2021</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>12</month>
<year>2021</year>
</pub-date>
<volume>25</volume>
<numero>6</numero>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_arttext&amp;pid=S1561-31942021000600018&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_abstract&amp;pid=S1561-31942021000600018&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_pdf&amp;pid=S1561-31942021000600018&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[RESUMEN  Introducción: la hiperplasia suprarrenal congénita es la causa más frecuente de alteraciones en la diferenciación sexual.  Presentación de caso:  se presenta el caso clínico de un recién nacido a término, buen peso, sin antecedentes perinatales patológicos. Nace en buenas condiciones y por método clínico se diagnóstica precozmente anomalía en el desarrollo de los genitales (ambiguos). Por lo que requiere interconsulta multidisciplinaria con genética, endocrinología, neonatología, urología, ginecología, psicología y cirugía, previo consentimiento informado familiar. En su evolución presenta: íctero flavínico de piel y mucosas a las 72 horas, en relación con íctero fisiológico, dosificación de 17 hidroxiprogesterona en suero de 200 ng/ml (elevado) y una pérdida fisiológica de peso del 10 %, límite máximo de la normalidad, según madurez. Por Programa de Tamizaje Neonatal se corroboró diagnóstico de HSC 21-hidroxilasa, a forma clásica virilizante simple grado IV, que lleva asociado hiponatremia e hiperpotasemia en estudios hemoquímicos. En cuidados especiales neonatales requirió estabilización, corrección de trastornos electrolíticos así como tratamiento con Fludrocortisona y acetato de cortisona. Evolucionó estable. Es egresado con seguimiento por consulta de Endocrinología y Genética. En la actualidad en espera de cirugía correctiva. La pareja involucrada requirió estudio, asesoramiento genético para la prevención de próximos nacimientos y se siguió en consulta multidisciplinaria.  Conclusiones:  la asistencia médica inmediata y la prontitud en el diagnóstico de hiperplasia suprarrenal congénita forma clásica virilizante simple grado IV, debutante en el período neonatal, permitió anticiparse a una pérdida salina aguda grave, lo que redujo el impacto biopsicosocial y mejoró la supervivencia infantil.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[ABSTRACT  Introduction:  congenital adrenal hyperplasia is the most frequent cause of alterations in sexual differentiation.  Case report:  the clinical case of a full-term newborn, good-weight, with no pathological perinatal history is presented. He was born in good condition and by clinical method an early diagnosis of anomalies in the development of genitalia (ambiguous) was made, requiring multidisciplinary consultation with genetics, endocrinology, neonatology, urology, gynecology, psychology and surgery, with prior informed consent of the family. During the evolution he presented: flavinic jaundice of skin and mucous membranes at 72 hours, in relation to physiological jaundice, levels of 17-hydroxyprogesterone serum in 200ng/ml (elevated) and a physiological weight loss of 10%, maximum limit of normality, according to maturity. The Neonatal Screening Program corroborated the diagnosis of HSC 21-hydroxylase, in the simple classical virilizing form IV-grade, with associated hyponatremia and hyperkalemia in hemochemical studies. In special neonatal care he required stabilization, correction of electrolyte disorders and treatment with Fludrocortisone and cortisone acetate. He evolved stable. He was discharged with follow-up by Endocrinology and Genetics consultations. Currently awaiting corrective surgery, the couple involved required studies, genetic counseling for the prevention of future births and was followed-up in a medical multidisciplinary consultation.  Conclusions:  immediate medical assistance and timely diagnosis of congenital adrenal hyperplasia, simple classical virilizing form grade- IV, debuting in the neonatal period, allowed anticipating severe acute saline loss, reducing the biopsychosocial impact and improving infant survival.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[HIPERPLASIA SUPRARRENAL CONGÉNITA]]></kwd>
<kwd lng="es"><![CDATA[FENILCETONURIA MATERNA]]></kwd>
<kwd lng="es"><![CDATA[TAMIZAJE NEONATAL]]></kwd>
<kwd lng="en"><![CDATA[ADRENAL HYPERPLASIA, CONGENITAL]]></kwd>
<kwd lng="en"><![CDATA[PHENYLKETONURIA, MATERNAL]]></kwd>
<kwd lng="en"><![CDATA[NEONATAL SCREENING]]></kwd>
</kwd-group>
</article-meta>
</front><back>
<ref-list>
<ref id="B1">
<label>1</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Latorre]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Garzón]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Manosalva]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Merchán]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Jacomussi]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Maldonado]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Hiperplasia adrenal congénita por déficit de 21 hidroxilasa: un reto diagnóstico y terapéutico]]></article-title>
<source><![CDATA[Repert Med Cir]]></source>
<year>2016</year>
<volume>25</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>79-88</page-range></nlm-citation>
</ref>
<ref id="B2">
<label>2</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[González]]></surname>
<given-names><![CDATA[EC]]></given-names>
</name>
<name>
<surname><![CDATA[Castells]]></surname>
<given-names><![CDATA[EM]]></given-names>
</name>
<name>
<surname><![CDATA[Frómeta]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Arteaga]]></surname>
<given-names><![CDATA[AL]]></given-names>
</name>
<name>
<surname><![CDATA[Del Río]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Tejeda]]></surname>
<given-names><![CDATA[Y]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[SUMA Technology and Newborn Screening Tests for Inherited Metabolic Diseases in Cuba: An Overview of the First 30 Years.]]></article-title>
<source><![CDATA[Journal of Inborn Errors of Metabolism &amp; Screening]]></source>
<year>2016</year>
<volume>4</volume>
<page-range>1-9</page-range></nlm-citation>
</ref>
<ref id="B3">
<label>3</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Rodríguez]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Espinosa]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Uso de glucocorticoides sistémicos en Pediatría: generalidades.]]></article-title>
<source><![CDATA[Acta Pediatr Mex]]></source>
<year>2016</year>
<volume>37</volume>
<numero>6</numero>
<issue>6</issue>
<page-range>349-354.</page-range></nlm-citation>
</ref>
<ref id="B4">
<label>4</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Feldman]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Congenital Adrenal Hyperplasia]]></article-title>
<source><![CDATA[Pediatr Adolesc Gynecol]]></source>
<year>2017</year>
<volume>30</volume>
<numero>5</numero>
<issue>5</issue>
<page-range>520-34</page-range></nlm-citation>
</ref>
<ref id="B5">
<label>5</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Phyllis]]></surname>
<given-names><![CDATA[W.]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Congenital Adrenal Hyperplasia Due to Steroid 21-Hydroxylase Deficiency: An Endocrine Society* Clinical Practice Guideline.]]></article-title>
<source><![CDATA[J Clin Endocrinol Metab]]></source>
<year>2018</year>
<volume>103</volume>
<numero>11</numero>
<issue>11</issue>
<page-range>4043-88</page-range></nlm-citation>
</ref>
<ref id="B6">
<label>6</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Roblejo]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
<name>
<surname><![CDATA[Marcheco]]></surname>
<given-names><![CDATA[B]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Genetics and genomic medicine in Cuba]]></article-title>
<source><![CDATA[Molecular Genetics &amp; Genomic Medicine]]></source>
<year>2017</year>
<volume>5</volume>
<numero>3</numero>
<issue>3</issue>
<page-range>196-201</page-range></nlm-citation>
</ref>
<ref id="B7">
<label>7</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Ponce]]></surname>
<given-names><![CDATA[CF]]></given-names>
</name>
<name>
<surname><![CDATA[Campos]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Milán]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Hiperplasia Suprarrenal Congénita. Reporte de Caso]]></article-title>
<source><![CDATA[Acta Pediátrica Hondureña]]></source>
<year>2014</year>
<volume>4</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>313-7</page-range></nlm-citation>
</ref>
<ref id="B8">
<label>8</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Donoso Sanz]]></surname>
<given-names><![CDATA[MA.]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Aspectos éticos en el abordaje médico de menores con diferencias en el desarrollo sexual. Hechos, valores y deberes en las DDS]]></article-title>
<source><![CDATA[Rev Esp Endocrinol Pediatr]]></source>
<year>2016</year>
<volume>7</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>27-31</page-range></nlm-citation>
</ref>
<ref id="B9">
<label>9</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Vicente]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Casas]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Ardanaz]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Origen de los programas de cribado neonatal y sus inicios en España]]></article-title>
<source><![CDATA[An Sist Sanit Navar]]></source>
<year>2017</year>
<volume>40</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>131-40</page-range></nlm-citation>
</ref>
<ref id="B10">
<label>10</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Bañuelos]]></surname>
<given-names><![CDATA[B]]></given-names>
</name>
<name>
<surname><![CDATA[González]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<name>
<surname><![CDATA[Ludwikowski]]></surname>
<given-names><![CDATA[B]]></given-names>
</name>
<name>
<surname><![CDATA[Lingnau]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Effectiveness of Prenatal Intervention on the Outcome of Diseases That Have a Postnatal Urological Impact]]></article-title>
<source><![CDATA[Front Pediatr]]></source>
<year>2019</year>
<volume>7</volume>
<numero>118</numero>
<issue>118</issue>
<page-range>1-6.</page-range></nlm-citation>
</ref>
<ref id="B11">
<label>11</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Clemente]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Puga]]></surname>
<given-names><![CDATA[B]]></given-names>
</name>
<name>
<surname><![CDATA[Campos]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Yeste]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Tratamiento prenatal de la HSC.]]></article-title>
<source><![CDATA[Rev Esp Endocrinol Pediatr]]></source>
<year>2018</year>
<volume>9</volume>
<numero>^sSuppl1</numero>
<issue>^sSuppl1</issue>
<supplement>Suppl1</supplement>
<page-range>26-9</page-range></nlm-citation>
</ref>
<ref id="B12">
<label>12</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Fidalgo]]></surname>
<given-names><![CDATA[SG]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Hiperplasia suprarrenal congénita: diagnóstico y tratamiento prenatal. Actualización]]></article-title>
<source><![CDATA[Revista de la Sociedad Argentina de Endocrinología Ginecológica y Reproductiva]]></source>
<year>2016</year>
<volume>23</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>18-28</page-range></nlm-citation>
</ref>
<ref id="B13">
<label>13</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Riaño]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[Chueca]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Consideraciones sobre el tratamiento prenatal en Hiperplasia Suprarrenal Congénita]]></article-title>
<source><![CDATA[Rev Esp Endocrinol Pediatr]]></source>
<year>2016</year>
<volume>7</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>32-6</page-range></nlm-citation>
</ref>
</ref-list>
</back>
</article>
