<?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>1688-1249</journal-id>
<journal-title><![CDATA[Archivos de Pediatría del Uruguay]]></journal-title>
<abbrev-journal-title><![CDATA[Arch. Pediatr. Urug.]]></abbrev-journal-title>
<issn>1688-1249</issn>
<publisher>
<publisher-name><![CDATA[Sociedad Uruguaya de Pediatría]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S1688-12492024000301902</article-id>
<article-id pub-id-type="doi">10.31134/ap.95.2.20</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Extubación de pacientes con atrofia muscular espinal tipo 1. Revisión bibliográfica]]></article-title>
<article-title xml:lang="en"><![CDATA[Extubation of patients with type 1 spinal muscular atrophy. Literature review]]></article-title>
<article-title xml:lang="pt"><![CDATA[Extubação de pacientes com atrofia muscular espinhal tipo 1. Revisão de literatura]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Huerta]]></surname>
<given-names><![CDATA[Antonio]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Valdebenito]]></surname>
<given-names><![CDATA[Carlos]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Manresa]]></surname>
<given-names><![CDATA[Ana]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Herrero]]></surname>
<given-names><![CDATA[Victoria]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Prado]]></surname>
<given-names><![CDATA[Francisco]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Hospital Clínico San Borja Arriarán Unidad Hospitalización Domiciliaria Pediátrica de Niños(as) y Adolescentes con Necesidades Especiales de Atención en Salud ]]></institution>
<addr-line><![CDATA[Santiago ]]></addr-line>
<country>Chile</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Universidad de Chile Facultad de Medicina Depto. Pediatría y Cirugía Infantil]]></institution>
<addr-line><![CDATA[Santiago ]]></addr-line>
<country>Chile</country>
</aff>
<aff id="Af3">
<institution><![CDATA[,NeumoHome Respiratory Care  ]]></institution>
<addr-line><![CDATA[Ciudad de Mendoza ]]></addr-line>
<country>Argentina</country>
</aff>
<aff id="Af4">
<institution><![CDATA[,Swiss Medical Clínica Zabala Servicio Kinesiología]]></institution>
<addr-line><![CDATA[Ciudad Bs.As ]]></addr-line>
<country>Argentina</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>00</month>
<year>2024</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>00</month>
<year>2024</year>
</pub-date>
<volume>95</volume>
<numero>2</numero>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.edu.uy/scielo.php?script=sci_arttext&amp;pid=S1688-12492024000301902&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.edu.uy/scielo.php?script=sci_abstract&amp;pid=S1688-12492024000301902&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.edu.uy/scielo.php?script=sci_pdf&amp;pid=S1688-12492024000301902&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[Resumen: La atrofia muscular espinal 5q, en su modalidad de presentación temprana, se asocia a eventos de insuficiencia ventilatoria dentro del primer año de vida, incluso en aquellos pacientes con intervención farmacológica específica. Esta evolución se debe al compromiso de los músculos inspiratorios, espiratorios y de la deglución con impacto adicional y relevante en la capacidad tusígena. El soporte ventilatorio no invasivo, sumado a estrategias de reclutamiento de volumen pulmonar, y facilitación de la tos manual y mecánica, son herramientas que logran evitarlos. Sin embargo, si estos pacientes requieren ser intubados, son difícilmente extubables con los protocolos habitualmente utilizados en las unidades de cuidado intensivo pediátrico. Este artículo de revisión aborda la información existente para evitar que el fallo ventilatorio y la intubación deriven en una traqueostomía como interfase para la ventilación mecánica invasiva. Detalla aquellos protocolos de destete en atrofia muscular espinal, que al igual que en otros pacientes con enfermedades neuromusculares sin compromiso de la primera motoneurona, permiten la transición al soporte ventilatorio no invasivo.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[Summary: 5q spinal muscular atrophy, at early presentation, is associated with events of ventilatory failure within the first year of life, even in those patients with specific pharmacological intervention. This evolution is due to the involvement of the inspiratory, expiratory and swallowing muscles with an additional and relevant impact on coughing capacity. Non-invasive ventilatory support, added to lung volume recruitment strategies, and facilitation of manual and mechanical cough, are tools used to avoid them. However, if these patients require intubation, they are difficult to extubate with the commonly used protocols in pediatric intensive care units. This article review addresses the existing information to prevent ventilatory failure and intubation leading to a tracheostomy as an interface for invasive prolonged mechanical ventilation. It describes the weaning protocols in spinal muscular atrophy, which, as in other patients with neuromuscular diseases without involvement of the first motor neuron, enable the transition to non-invasive ventilatory support.]]></p></abstract>
<abstract abstract-type="short" xml:lang="pt"><p><![CDATA[Resumo: A atrofia muscular espinhal 5q, em sua apresentação precoce, está associada a eventos de insuficiência ventilatória no primeiro ano de vida, mesmo naqueles pacientes com intervenção farmacológica específica. Esta evolução deve-se ao comprometimento da musculatura inspiratória, expiratória e da deglutição com impacto adicional e relevante na capacidade de tosse. O suporte ventilatório não invasivo, somado às estratégias de recrutamento de volume pulmonar e facilitação da tosse manual e mecânica, são ferramentas que conseguem evitá-los. Porém, se esses pacientes necessitarem de intubação, serão difíceis de extubar com os protocolos habitualmente utilizados em unidades de terapia intensiva pediátrica. Este artigo de revisão aborda as informações existentes para evitar que a falha ventilatória e a intubação levem à traqueostomia como interface para ventilação mecânica invasiva. Detalha os protocolos de desmame na atrofia muscular espinhal, que, como em outros pacientes com doenças neuromusculares sem comprometimento do primeiro neurônio motor, permitem a transição para suporte ventilatório não invasivo.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[Atrofia Muscular Espinal]]></kwd>
<kwd lng="es"><![CDATA[Desconexión del Ventilador]]></kwd>
<kwd lng="es"><![CDATA[Ventilación no Invasiva]]></kwd>
<kwd lng="es"><![CDATA[Respiración Artificial]]></kwd>
<kwd lng="es"><![CDATA[Extubación Traqueal]]></kwd>
<kwd lng="en"><![CDATA[Spinal Muscular Atrophy]]></kwd>
<kwd lng="en"><![CDATA[Ventilator Disconnection]]></kwd>
<kwd lng="en"><![CDATA[Non-invasive Ventilation]]></kwd>
<kwd lng="en"><![CDATA[Artificial Respiration]]></kwd>
<kwd lng="en"><![CDATA[Tracheal Extubation]]></kwd>
<kwd lng="pt"><![CDATA[Atrofia Muscular Espinhal]]></kwd>
<kwd lng="pt"><![CDATA[Desconexão do Ventilador]]></kwd>
<kwd lng="pt"><![CDATA[Ventilação não Invasiva]]></kwd>
<kwd lng="pt"><![CDATA[Respiração Artificial]]></kwd>
<kwd lng="pt"><![CDATA[Extubação Traqueal]]></kwd>
</kwd-group>
</article-meta>
</front><back>
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<article-title xml:lang=""><![CDATA[Cuidados respiratorios de los pacientes con atrofia muscular espinal]]></article-title>
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</article>
