<?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>1028-9933</journal-id>
<journal-title><![CDATA[Revista Información Científica]]></journal-title>
<abbrev-journal-title><![CDATA[Rev. inf. cient.]]></abbrev-journal-title>
<issn>1028-9933</issn>
<publisher>
<publisher-name><![CDATA[Universidad de Ciencias Médicas Guantánamo]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S1028-99332020000100089</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Síndrome Sturge-Weber. Revisión de la literatura a propósito de un caso]]></article-title>
<article-title xml:lang="en"><![CDATA[Sturge-Weber syndrome. Literature review about a case]]></article-title>
<article-title xml:lang="pt"><![CDATA[Síndrome de Sturge-Weber. Revisão de literatura sobre um caso]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Cremé-Lambert]]></surname>
<given-names><![CDATA[Limnet]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Díaz-Estévez]]></surname>
<given-names><![CDATA[Harasay]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Lamas-Ávila]]></surname>
<given-names><![CDATA[Mireya]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Hospital Pediátrico Docente &#8220;General Pedro Agustín Pérez&#8221;  ]]></institution>
<addr-line><![CDATA[ Guantánamo]]></addr-line>
<country>Cuba</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>02</month>
<year>2020</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>02</month>
<year>2020</year>
</pub-date>
<volume>99</volume>
<numero>1</numero>
<fpage>89</fpage>
<lpage>101</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_arttext&amp;pid=S1028-99332020000100089&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_abstract&amp;pid=S1028-99332020000100089&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_pdf&amp;pid=S1028-99332020000100089&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[RESUMEN  Introducción:  Entre las hamartoblastosis se reconoce el síndrome de Sturge-Weber (SSW), cuyo diagnóstico es inusual y complejo su tratamiento.  Objetivo:  Socializar la experiencia en la atención a un paciente pediátrico con síndrome de Sturge-Weber en el Hospital Pediátrico Docente &#8220;General Pedro Agustín Pérez&#8221; de Guantánamo.  Método:  Se presentó un paciente preescolar, sin antecedentes familiares de interés. Al mes de nacido fue operado de glaucoma congénito. Durante el primer año de vida presentó retardo del desarrollo psicomotor y movimientos involuntarios considerados convulsiones tónico-clónicas. Se revisó la literatura para transmitir a la comunidad médica, de modo particular, a estudiantes de medicina y médicos generales, información para lograr un diagnóstico y un seguimiento adecuado de esta afección.  Resultados:  Según las manifestaciones clínicas, examen físico mediante el método clínico y los resultados de los exámenes complementarios, se planteó el diagnóstico de síndrome neurocutáneo, de modo particular un SSW. Se aplicó tratamiento con diazepam y al persistir la crisis, fenitoína. La primera clasificación de los síndromes neurocutáneos la realizó Jan Van der Hoeve, que acuñó el término facomatosis. Es un trastorno neurológico congénito, no hereditario aunque hay descrito casos familiares, poco común pero frecuente respecto a otros síndromes neurocutáneos. Se estima una incidencia de 5 x 100,000 nacidos vivos. Afecta a todos los grupos étnicos y ambos sexos.  Conclusiones:  No siempre hay relación entre la gravedad de las manifestaciones cutáneas, neurológicas y oculares del SSW con las alteraciones cerebrales. Es importante el examen físico para establecer el diagnóstico oportuno y evitar futuras secuelas y complicaciones.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[ABSTRACT  Introduction:  Sturge-Weber syndrome (SSW) is recognized among hamartoblastosis, whose diagnosis is unusual and complex treatment.  Objective:  To socialize the experience in the care of a pediatric patient with Sturge-Weber syndrome at the "General Pedro Agustín Pérez" Pediatric Teaching Hospital in Guantanamo.  Method:  A preschool patient was presented, with no family history of interest. One month after birth, he underwent congenital glaucoma surgery. During the first year of life he presented retardation of psychomotor development and involuntary movements considered tonic-clonic seizures. Literature was reviewed to transmit to the medical community, in particular, to medical students and general practitioners, information to achieve a diagnosis and adequate follow-up of this condition.  Results:  According to the clinical manifestations, physical examination by means of the clinical method and the results of the complementary examinations, the diagnosis of neurocutaneous syndrome was proposed, in particular a SSW. Treatment with diazepam was applied and when the crisis persisted, phenytoin. The first classification of neurocutaneous syndromes was carried out by Jan Van der Hoeve, who coined the term phacomatosis. It is a congenital neurological disorder, not inherited although there have been family cases described, uncommon but frequent compared to other neurocutaneous syndromes. An incidence of 5 x 100,000 live births is estimated. It affects all ethnic groups and both sexes.  Conclusions:  There is not always a relationship between the severity of cutaneous, neurological and ocular manifestations of SSW with brain disorders. The physical examination is important to establish the timely diagnosis and avoid future sequelae and complications.]]></p></abstract>
<abstract abstract-type="short" xml:lang="pt"><p><![CDATA[RESUMO  Introdução:  A síndrome de Sturge-Weber (SSW) é reconhecida entre as hamartoblastose, cujo diagnóstico é tratamento incomum e complexo.  Objetivo:  Socializar a experiência no atendimento de um paciente pediátrico com síndrome de Sturge-Weber no Hospital Pediátrico "General Pedro Agustín Pérez" em Guantánamo.  Método:  Foi apresentado um paciente pré-escolar, sem histórico familiar de interesse. Um mês após o nascimento, ele foi submetido a uma cirurgia de glaucoma congênito. Durante o primeiro ano de vida, apresentou retardo no desenvolvimento psicomotor e movimentos involuntários considerados convulsões tônico-clônicas. A literatura foi revisada para transmitir à comunidade médica, em particular aos estudantes de medicina e clínicos gerais, informações para obter um diagnóstico e acompanhamento adequado dessa condição.  Resultados:  De acordo com as manifestações clínicas, o exame físico por meio do método clínico e os resultados dos exames complementares, foi proposto o diagnóstico de síndrome neurocutânea, em particular um SSW. O tratamento com diazepam foi aplicado e, quando a crise persistiu, fenitoína. A primeira classificação das síndromes neurocutâneas foi realizada por Jan Van der Hoeve, que cunhou o termo facomatose. É um distúrbio neurológico congênito, não herdado, embora haja casos familiares descritos, incomum, mas frequente em comparação com outras síndromes neurocutâneas. É estimada uma incidência de 5 x 100.000 nascidos vivos. Afeta todos os grupos étnicos e ambos os sexos.  Conclusões:  Nem sempre existe relação entre a gravidade das manifestações cutâneas, neurológicas e oculares da SSW com distúrbios cerebrais. O exame físico é importante para estabelecer o diagnóstico oportuno e evitar futuras sequelas e complicações.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[síndrome Sturge-Weber]]></kwd>
<kwd lng="es"><![CDATA[angioma]]></kwd>
<kwd lng="es"><![CDATA[hamartoblastosis]]></kwd>
<kwd lng="en"><![CDATA[Sturge-Weber syndrome]]></kwd>
<kwd lng="en"><![CDATA[angioma]]></kwd>
<kwd lng="en"><![CDATA[hamartoblastosis]]></kwd>
<kwd lng="pt"><![CDATA[síndrome de Sturge-Weber]]></kwd>
<kwd lng="pt"><![CDATA[angioma]]></kwd>
<kwd lng="pt"><![CDATA[hamartoblastose]]></kwd>
</kwd-group>
</article-meta>
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