<?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>1657-4702</journal-id>
<journal-title><![CDATA[Revista Latinoamericana de Bioética]]></journal-title>
<abbrev-journal-title><![CDATA[rev.latinoam.bioet.]]></abbrev-journal-title>
<issn>1657-4702</issn>
<publisher>
<publisher-name><![CDATA[Universidad Militar Nueva Granada]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S1657-47022020000200025</article-id>
<article-id pub-id-type="doi">10.18359/rlbi.4642</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Enfermedad de Fabry clásica en pacientes pediátricos asintomáticos: aspectos bioéticos de la terapia enzimática]]></article-title>
<article-title xml:lang="en"><![CDATA[Classic Fabry disease in asymptomatic pediatric patients: Bioethical aspects of enzyme therapy]]></article-title>
<article-title xml:lang="pt"><![CDATA[Doença de Fabry clássica em pacientes pediátricos assintomáticos: aspectos bioéticos da terapia enzimática]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Villalobos-Azuaje]]></surname>
<given-names><![CDATA[Jacobo José]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Padrón-Nieves]]></surname>
<given-names><![CDATA[Maritza del Carmen]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Universidad Central de Venezuela (UCV)  ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Venezuela</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Universidad Central de Venezuela  ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Venezuela</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>12</month>
<year>2020</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>12</month>
<year>2020</year>
</pub-date>
<volume>20</volume>
<numero>2</numero>
<fpage>25</fpage>
<lpage>40</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.org.co/scielo.php?script=sci_arttext&amp;pid=S1657-47022020000200025&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.co/scielo.php?script=sci_abstract&amp;pid=S1657-47022020000200025&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.co/scielo.php?script=sci_pdf&amp;pid=S1657-47022020000200025&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[Resumen: El propósito de este artículo es proponer un marco bioético para la administración de la terapia enzimática en niños con enfermedad de Fabry asintomáticos. Se realizó un estudio de campo transversal, observacional, descriptivo, con análisis cuantitativo y cualitativo, a la luz de la bioética en médicos que han diagnosticado y tratado pacientes con la enfermedad a nivel nacional e internacional, mediante una encuesta con preguntas cerradas validada por juicio de expertos, se evaluó la aplicación de los principios de no maleficencia, justicia y sacralidad de la vida en la administración del tratamiento enzimático para estos pacientes. La información se agrupó y procesó con estadística descriptiva. El 83,33 % de los encuestados consideró que un niño con enfermedad de Fabry asintomático debe recibir tratamiento enzimático específico, independientemente de su costo (justicia), además, que el tratamiento debe ser financiado por el Estado a fin de prevenir las complicaciones de la enfermedad; esto fue reconocido por el 75 % de los médicos encuestados (no maleficencia). El 66,66 % consideró que la sacralidad de la vida está en función de prevenir las complicaciones que condicionan la muerte. Así, con base en la bioética principialista se justifica iniciar la terapia enzimática específica para el tratamiento de niños con enfermedad de Fabry asintomáticos.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[Abstract: This paper aims to propose a bioethical framework for the administration of enzyme therapy in children with asymptomatic Fabry disease. A cross-sectional, observational, descriptive field study was conducted with quantitative and qualitative analysis, in the light of bioethics with physicians who have diagnosed and treated patients with classic Fabry disease at the local and international level, using a survey with closed questions validated by expert judgment. The application of the principles of non-maleficence, justice, and sacredness of life in the administration of enzymatic treatment for these patients was evaluated. The information was grouped and processed with descriptive statistics. 83,33 % of respondents considered that a child with asymptomatic Fabry disease should receive specific enzymatic treatment, regardless of its cost (justice), and that treatment should be funded by the State in order to prevent complications of the disease. This was recognized by 75% of the physicians surveyed (not maleficence). 66,66 % considered that the sacredness of life must prevent the complications that condition death. Thus, based on the principles of bioethics, initiating specific enzymatic therapy for the treatment of children with asymptomatic Fabry disease is sustained.]]></p></abstract>
<abstract abstract-type="short" xml:lang="pt"><p><![CDATA[Resumo: o propósito deste artigo é propor um referencial bioético para administrar a terapia enzimática em crianças com doença de Fabry assintomáticos. Foi realizado um estudo de campo transversal, observacional, descritivo, com análise quantitativa e qualitativa, à luz da bioética em médicos que diagnosticaram e trataram pacientes com a doença no contexto nacional e internacional, mediante questionário com perguntas fechadas validada porjulga- mento de especialistas; foi avaliada a aplicação dos princípios de não maleficência, justiça e sacralidade da vida na administração do tratamento enzimático para esses pacientes. A informação foi agrupada e processada com estatística descritiva. 83,33 % dos pesquisados consideraram que uma criança com doença de Fabry assintomática deve receber tratamento enzimático específico, independentemente de seu custo (justiça), além disso que o tratamento deve ser financiado pelo Estado para prevenir as complicações da doença. Isso foi reconhecido por 75 % dos médicos investigados (não maleficência). 66,66 % consideraram que a sacralidade da vida está em função de prevenir as complicações que condicionam a morte. Assim, com base na bioética principialista, justifica-se iniciar a terapia enzimática específica para tratar crianças com doença de Fabry assintomáticas.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[enfermedad de Fabry]]></kwd>
<kwd lng="es"><![CDATA[enfermedad de Fabry en niños]]></kwd>
<kwd lng="es"><![CDATA[terapia enzimática]]></kwd>
<kwd lng="es"><![CDATA[bioética]]></kwd>
<kwd lng="en"><![CDATA[Fabry's disease]]></kwd>
<kwd lng="en"><![CDATA[Fabry's disease in children]]></kwd>
<kwd lng="en"><![CDATA[enzyme therapy]]></kwd>
<kwd lng="en"><![CDATA[bioethics]]></kwd>
<kwd lng="pt"><![CDATA[doença de Fabry]]></kwd>
<kwd lng="pt"><![CDATA[doença de Fabry em crianças]]></kwd>
<kwd lng="pt"><![CDATA[terapia enzimática]]></kwd>
<kwd lng="pt"><![CDATA[bioética]]></kwd>
</kwd-group>
</article-meta>
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