<?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>0120-9957</journal-id>
<journal-title><![CDATA[Revista colombiana de Gastroenterología]]></journal-title>
<abbrev-journal-title><![CDATA[Rev. colomb. Gastroenterol.]]></abbrev-journal-title>
<issn>0120-9957</issn>
<publisher>
<publisher-name><![CDATA[Asociación Colombiana de Gastroenterología  ]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S0120-99572024000200219</article-id>
<article-id pub-id-type="doi">10.22516/25007440.1082</article-id>
<title-group>
<article-title xml:lang="en"><![CDATA[Primary Congenital Intestinal Lymphangiectasia: A Case Report]]></article-title>
<article-title xml:lang="es"><![CDATA[Linfangiectasia intestinal primaria congénita: a propósito de un caso]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Díaz-Gómez]]></surname>
<given-names><![CDATA[Tatiana]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Sempértegui-Cárdenas]]></surname>
<given-names><![CDATA[Pablo]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Tinoco-Ochoa]]></surname>
<given-names><![CDATA[Ana]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Universidad de Cuenca  ]]></institution>
<addr-line><![CDATA[Cuenca ]]></addr-line>
<country>Ecuador</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Department of Pediatrics  ]]></institution>
<addr-line><![CDATA[Cuenca ]]></addr-line>
<country>Ecuador</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>06</month>
<year>2024</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>06</month>
<year>2024</year>
</pub-date>
<volume>39</volume>
<numero>2</numero>
<fpage>219</fpage>
<lpage>223</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.org.co/scielo.php?script=sci_arttext&amp;pid=S0120-99572024000200219&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.co/scielo.php?script=sci_abstract&amp;pid=S0120-99572024000200219&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.co/scielo.php?script=sci_pdf&amp;pid=S0120-99572024000200219&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="en"><p><![CDATA[Abstract Primary intestinal lymphangiectasia (PIL) is a rare disease that was discovered in 1961 by Waldmann et al., who first described the concept of intestinal lymphangiectasia. PIL is manifested by large and dilated intestinal lymphatic vessels that lead to the loss of lymphatic fluid to the intestinal lumen, including severe hypoalbuminemia and, consequently, generalized edema. The etiology of PIL is unknown; however, several genes are involved in the development of the lymphatic system. The case of a two-month-old patient with a prenatal diagnosis of ascites on third-trimester ultrasound was presented. The patient was born with rapidly progressive ascites and showed an endoscopic study with evidence of snowflake images and histology compatible with intestinal lymphangiectasia. The patient remained on octreotide infusion at baseline and subsequently with refractory ascites the dose was increased. Several evacuatory paracentesis were performed and he remained on enteral nutrition based on an extensively hydrolyzed formula with a 54% contribution of medium chain triglycerides (MCTs) and parenteral nutrition (PN). Subsequently, the patient was switched to a subcutaneous infusion of octreotide, which allowed hospital discharge and adequate outpatient follow-up with a favorable evolution.]]></p></abstract>
<abstract abstract-type="short" xml:lang="es"><p><![CDATA[Resumen La linfangiectasia intestinal primaria (LIP) es una enfermedad rara que se descubrió en 1961 por Waldmann y colaboradores, quienes describieron por primera vez el concepto de linfangiectasia intestinal. La LIP se manifiesta por vasos linfáticos intestinales grandes y dilatados que conllevan la pérdida de líquido linfático hacia la luz intestinal, siendo responsable de una hipoalbuminemia grave y, en consecuencia; de un edema generalizado. La etiología de la LIP es desconocida, sin embargo, varios genes están implicados en el desarrollo del sistema linfático. Se presenta el caso de una paciente de dos meses de edad con diagnóstico prenatal de ascitis en ecografía de tercer trimestre. Nació con ascitis rápidamente progresiva y presentó un estudio endoscópico con evidencia de imágenes en copos de nieve e histología compatible con linfangiectasia intestinal. Se mantuvo en tratamiento con infusión de octreotida al inicio y posteriormente con ascitis refractaria se aumentó la dosis. Se realizaron varias paracentesis evacuatorias y permaneció con nutrición enteral a base de una fórmula extensamente hidrolizada con aporte del 54% de triglicéridos de cadena media (TCM) y nutrición parenteral (NPT). Más tarde, se cambió a infusión de octreotida por vía subcutánea, lo que permitió el alta hospitalaria y un seguimiento adecuado por consulta externa con una evolución favorable.]]></p></abstract>
<kwd-group>
<kwd lng="en"><![CDATA[Ascites]]></kwd>
<kwd lng="en"><![CDATA[intestinal lymphangiectasia]]></kwd>
<kwd lng="en"><![CDATA[octreotide]]></kwd>
<kwd lng="en"><![CDATA[children]]></kwd>
<kwd lng="es"><![CDATA[Ascitis]]></kwd>
<kwd lng="es"><![CDATA[linfangiectasia intestinal]]></kwd>
<kwd lng="es"><![CDATA[octreótido]]></kwd>
<kwd lng="es"><![CDATA[niños]]></kwd>
</kwd-group>
</article-meta>
</front><back>
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