<?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>1608-8921</journal-id>
<journal-title><![CDATA[Gaceta Médica Espirituana]]></journal-title>
<abbrev-journal-title><![CDATA[Gac Méd Espirit]]></abbrev-journal-title>
<issn>1608-8921</issn>
<publisher>
<publisher-name><![CDATA[Universidad de Ciencias Médicas de Sancti Spíritus]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S1608-89212021000200099</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Linfedema congénito primario bilateral. Reporte de caso]]></article-title>
<article-title xml:lang="en"><![CDATA[Bilateral primary congenital lymphedema. Case report]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[García Román]]></surname>
<given-names><![CDATA[Dayelis]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Ibargollín Ulloa]]></surname>
<given-names><![CDATA[Rafael]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Tavio Reyes]]></surname>
<given-names><![CDATA[Ana Ledia]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[León Bernal]]></surname>
<given-names><![CDATA[Danysleidi]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Pérez Concepción]]></surname>
<given-names><![CDATA[Irma]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Hospital General Provincial Camilo Cienfuegos  ]]></institution>
<addr-line><![CDATA[Sancti Spíritus ]]></addr-line>
<country>Cuba</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Centro de Ingeniería Genética y Biotecnología  ]]></institution>
<addr-line><![CDATA[Sancti Spíritus ]]></addr-line>
<country>Cuba</country>
</aff>
<aff id="Af3">
<institution><![CDATA[,Policlínico Dr. Rudersindo García del Rijo  ]]></institution>
<addr-line><![CDATA[Sancti Spíritus ]]></addr-line>
<country>Cuba</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>08</month>
<year>2021</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>08</month>
<year>2021</year>
</pub-date>
<volume>23</volume>
<numero>2</numero>
<fpage>99</fpage>
<lpage>106</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_arttext&amp;pid=S1608-89212021000200099&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_abstract&amp;pid=S1608-89212021000200099&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_pdf&amp;pid=S1608-89212021000200099&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[RESUMEN  Fundamento: El linfedema congénito primario es una condición rara con un componente genético importante que se caracteriza por edema crónico de la zona afectada.  Objetivo:  Presentar un linfedema congénito primario bilateral y discutir su origen.  Presentación de caso: Se presentó un caso de linfedema congénito primario bilateral en un niño de 2 años de edad, sin antecedentes patológicos familiares de la enfermedad. Se discutieron sus posibles causas genéticas ya que existen varias mutaciones que explican su origen. Aunque no se pudieron realizar estudios genéticos para conocer la etiología exacta, existen evidencias clínicas de que no se trata de una enfermedad de Milroy, a menos que se presente como una mutación de novo. Se le realizó al paciente un seguimiento desde su diagnóstico hasta la actualidad.  Conclusiones: Existen múltiples mutaciones genéticas que explican el origen de un linfedema congénito primario, por lo que no necesariamente debe tratarse de enfermedad de Milroy cuando este se presente. Se destacó como elemento importante que en este caso no se evidenciaron antecedentes familiares. Se empleó el tratamiento conservador como conducta fundamental a seguir, se evidenció en el paciente una notable mejoría clínica.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[ABSTRACT  Background:  Primary congenital lymphedema is a rare condition with an important genetic component characterized by chronic edema of the affected area.  Objective:  To present a bilateral primary congenital lymphedema and discuss its origin.  Case report:  A case of bilateral primary congenital lymphedema was presented in a 2-year-old boy with no any family background of the disease. Its possible genetic causes were discussed since there are several mutations that explain its origin. Although genetic studies could not be performed to know the exact etiology, there is clinical evidence that it is not a Milroy's disease, unless it presents as a de novo mutation. The patient was followed up from diagnosis to the present.  Conclusions:  There are multiple genetic mutations that explain the origin of a primary congenital lymphedema, so it should not necessarily be Milroy's disease when present. A highlighted and important element was that in this case no any family background was evidenced. Conservative treatment was used as the essential conduct to follow up, a remarkable clinical progress was evidenced in the patient.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[Linfedema congénito primario]]></kwd>
<kwd lng="es"><![CDATA[genética]]></kwd>
<kwd lng="es"><![CDATA[etiología]]></kwd>
<kwd lng="es"><![CDATA[enfermedad de Milroy]]></kwd>
<kwd lng="en"><![CDATA[Primary congenital lymphedema]]></kwd>
<kwd lng="en"><![CDATA[genetics]]></kwd>
<kwd lng="en"><![CDATA[etiology]]></kwd>
<kwd lng="en"><![CDATA[Milroy's disease]]></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[Grada]]></surname>
<given-names><![CDATA[AA]]></given-names>
</name>
<name>
<surname><![CDATA[Phllps]]></surname>
<given-names><![CDATA[TJ]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Lymphedema: pathophysiology and clinical manifestations]]></article-title>
<source><![CDATA[J Am Acad Dermatol]]></source>
<year>2017</year>
<volume>77</volume>
<numero>6</numero>
<issue>6</issue>
<page-range>1009-20</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[Pereira]]></surname>
<given-names><![CDATA[CN]]></given-names>
</name>
<name>
<surname><![CDATA[Koshima]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Linfedema: actualización en el diagnóstico y tratamiento quirúrgico]]></article-title>
<source><![CDATA[Rev Chil Cir]]></source>
<year>2018</year>
<volume>70</volume>
<numero>6</numero>
<issue>6</issue>
<page-range>589-97</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[Urbaneja Rodríguez]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Garrote Molpeceres]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<name>
<surname><![CDATA[Pino Vázquez]]></surname>
<given-names><![CDATA[MA]]></given-names>
</name>
<name>
<surname><![CDATA[González García]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Linfedema congénito secundario a enfermedad de Milroy]]></article-title>
<source><![CDATA[An Pediatr (Barc)]]></source>
<year>2017</year>
<volume>86</volume>
<numero>3</numero>
<issue>3</issue>
<page-range>169-70</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[Mortime]]></surname>
<given-names><![CDATA[PS]]></given-names>
</name>
<name>
<surname><![CDATA[Gordon]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Brice]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Mansour]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Hereditary and Familial Lymphedemas]]></article-title>
<source><![CDATA[Lymphedema]]></source>
<year>2018</year>
<page-range>29-43</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[Ouattara]]></surname>
<given-names><![CDATA[CZ]]></given-names>
</name>
<name>
<surname><![CDATA[Kalmogho]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Yonaba]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Bouda]]></surname>
<given-names><![CDATA[CG]]></given-names>
</name>
<name>
<surname><![CDATA[Yaméogo]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Kam]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Le lymph&#339;dème congénital primaire: la maladie de Milroy: à propos du premier cas observé dans le Département de Pédiatrie du Centre Hospitalier Universitaire Yalgado Ouédraogo, Ouagadougou&#8221; [Linfedema congénito primario: enfermedad de Milroy: el primer caso observado en el Departamento de Pediatría en el Hospital Universitario Yalgado Ouedraogo, Uagadugú]]]></article-title>
<source><![CDATA[Pan Afr Med J]]></source>
<year>2017</year>
<volume>27</volume>
<numero>1</numero>
<issue>1</issue>
</nlm-citation>
</ref>
<ref id="B6">
<label>6</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Neves]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Brito]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Mota]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Linfedema congénito]]></article-title>
<source><![CDATA[Nascer e Crecer]]></source>
<year>2017</year>
<volume>26</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>68-70</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[Mrha]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Bukovansky]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Kafkova]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Kraft]]></surname>
<given-names><![CDATA[O]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Primary Congenital Lymphedema]]></article-title>
<source><![CDATA[Clin Nucl Med]]></source>
<year>2018</year>
<volume>43</volume>
<numero>7</numero>
<issue>7</issue>
<page-range>515-7</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[Quiñones Castro]]></surname>
<given-names><![CDATA[JM]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Linfedema congénito hereditario: (síndrome de Milroy). Presentación de un caso]]></article-title>
<source><![CDATA[Rev cubana pediatr.]]></source>
<year>1972</year>
<volume>11</volume>
<page-range>115-22</page-range></nlm-citation>
</ref>
<ref id="B9">
<label>9</label><nlm-citation citation-type="book">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Liu]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Primary Lymphedema and Lymphatic Malformation]]></article-title>
<person-group person-group-type="editor">
<name>
<surname><![CDATA[Lee]]></surname>
<given-names><![CDATA[B-B]]></given-names>
</name>
<name>
<surname><![CDATA[Rockson]]></surname>
<given-names><![CDATA[SG]]></given-names>
</name>
<name>
<surname><![CDATA[Bergan]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
</person-group>
<source><![CDATA[Lymphedema: A Concise Compendium of Theory and Practice]]></source>
<year>2018</year>
<page-range>743-52</page-range><publisher-loc><![CDATA[Cham ]]></publisher-loc>
<publisher-name><![CDATA[Springer International Publishing]]></publisher-name>
</nlm-citation>
</ref>
<ref id="B10">
<label>10</label><nlm-citation citation-type="book">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Schlögel]]></surname>
<given-names><![CDATA[MJ]]></given-names>
</name>
<name>
<surname><![CDATA[Brouillard]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Boon]]></surname>
<given-names><![CDATA[LM]]></given-names>
</name>
<name>
<surname><![CDATA[Vikkula]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Molecular Genetics of Lymphatic and Complex Vascular Malformations]]></article-title>
<person-group person-group-type="editor">
<name>
<surname><![CDATA[Lee]]></surname>
<given-names><![CDATA[B-B]]></given-names>
</name>
<name>
<surname><![CDATA[Rockson]]></surname>
<given-names><![CDATA[SG]]></given-names>
</name>
<name>
<surname><![CDATA[Bergan]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
</person-group>
<source><![CDATA[Lymphedema: A Concise Compendium of Theory and Practice]]></source>
<year>2018</year>
<page-range>753-63</page-range><publisher-loc><![CDATA[Cham ]]></publisher-loc>
<publisher-name><![CDATA[Springer International Publishing]]></publisher-name>
</nlm-citation>
</ref>
</ref-list>
</back>
</article>
