<?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>S1688-12492021000101401</article-id>
<article-id pub-id-type="doi">10.31134/ap.92.1.9</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Recomendaciones para el manejo respiratorio de los pacientes con atrofia muscular espinal]]></article-title>
<article-title xml:lang="en"><![CDATA[Recommendations for respiratory management of patients with spinal muscular atrophia]]></article-title>
<article-title xml:lang="pt"><![CDATA[Recomendações para a manipulação respiratória de pacientes com atrofia muscular espinhal]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Giménez]]></surname>
<given-names><![CDATA[Gloria]]></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 contrib-type="author">
<name>
<surname><![CDATA[Bersano]]></surname>
<given-names><![CDATA[Celia]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Kakisu]]></surname>
<given-names><![CDATA[Hiromi]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Herrero]]></surname>
<given-names><![CDATA[María Victoria]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Manresa]]></surname>
<given-names><![CDATA[Ana Lucía]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Pronello]]></surname>
<given-names><![CDATA[Damián]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Salinas]]></surname>
<given-names><![CDATA[Pamela]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Morales]]></surname>
<given-names><![CDATA[Juan Carlos]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Gutiérrez]]></surname>
<given-names><![CDATA[Montserrat]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Guillén]]></surname>
<given-names><![CDATA[Silvia]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Aravena]]></surname>
<given-names><![CDATA[Silvia]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Morales]]></surname>
<given-names><![CDATA[Pedro]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Moscoso]]></surname>
<given-names><![CDATA[Gustavo]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Oviedo]]></surname>
<given-names><![CDATA[Valeria]]></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[Núñez]]></surname>
<given-names><![CDATA[Débora]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Galeano]]></surname>
<given-names><![CDATA[Shirley]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Bach]]></surname>
<given-names><![CDATA[John]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Pinchak]]></surname>
<given-names><![CDATA[Catalina]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Universidad Nacional de Asunción Facultad de Ciencias Médicas ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Paraguay</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Hospital Clínico San Borja Arriaraìn Servicio Pediatría ]]></institution>
<addr-line><![CDATA[Santiago de Chile ]]></addr-line>
</aff>
<aff id="Af3">
<institution><![CDATA[,Centro de Rehabilitación Neurológica Infantil Terapia y Rehabilitación Respiratoria Gospa ]]></institution>
<addr-line><![CDATA[Córdoba ]]></addr-line>
<country>Argentina</country>
</aff>
<aff id="Af4">
<institution><![CDATA[,Hospital Privado de Comunidad Unidad Cuidados Intensivos ]]></institution>
<addr-line><![CDATA[Mar del Plata ]]></addr-line>
<country>Argentina</country>
</aff>
<aff id="Af5">
<institution><![CDATA[,Hospital Petrona Villega de Cordero Unidad Internación de Kinesiología y Fisiatría ]]></institution>
<addr-line><![CDATA[San Fernando ]]></addr-line>
<country>Argentina</country>
</aff>
<aff id="Af6">
<institution><![CDATA[,Clínica Zabala Swiss Medical Group Servicio Kinesiología ]]></institution>
<addr-line><![CDATA[Buenos Aires ]]></addr-line>
<country>Argentina</country>
</aff>
<aff id="Af7">
<institution><![CDATA[,Hospital Dr. Humberto Notti  ]]></institution>
<addr-line><![CDATA[Mendoza ]]></addr-line>
<country>Argentina</country>
</aff>
<aff id="Af8">
<institution><![CDATA[,Hospital Alexander Fleming Área de Ventilación no Invasiva Servicio Neumonología Infantil]]></institution>
<addr-line><![CDATA[Mendoza ]]></addr-line>
<country>Argentina</country>
</aff>
<aff id="Af9">
<institution><![CDATA[,Homecare MV Clinical HealthCare ]]></institution>
<addr-line><![CDATA[Santiago de Chile ]]></addr-line>
</aff>
<aff id="A10">
<institution><![CDATA[,Hospital Clínico Universitario  ]]></institution>
<addr-line><![CDATA[Caracas ]]></addr-line>
<country>Venezuela</country>
</aff>
<aff id="A11">
<institution><![CDATA[,Instituto Cardiología Preventiva  ]]></institution>
<addr-line><![CDATA[San Juan del Río ]]></addr-line>
<country>México</country>
</aff>
<aff id="A12">
<institution><![CDATA[,BPS Centro Referencia Nacional de Enfermedades Raras y Congénitas. CRENADECER ]]></institution>
<addr-line><![CDATA[Montevideo ]]></addr-line>
<country>Uruguay</country>
</aff>
<aff id="A13">
<institution><![CDATA[,Universidad Nacional de Asunción Facultad de Ciencias Médicas Hospital de Clínicas de San Lorenzo]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Paraguay</country>
</aff>
<aff id="A14">
<institution><![CDATA[,Universidad Nacional de Asunción Facultad de Ciencias Médicas Cátedra y Servicio de Neumología]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Paraguay</country>
</aff>
<aff id="A15">
<institution><![CDATA[,University Hospital New Jersey Medical School Rutgers]]></institution>
<addr-line><![CDATA[ Newark]]></addr-line>
<country>Estados Unidos</country>
</aff>
<aff id="A16">
<institution><![CDATA[,UDELAR Facultad de Medicina Clínica Pediátrica]]></institution>
<addr-line><![CDATA[Montevideo ]]></addr-line>
<country>Uruguay</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>06</month>
<year>2021</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>06</month>
<year>2021</year>
</pub-date>
<volume>92</volume>
<numero>1</numero>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.edu.uy/scielo.php?script=sci_arttext&amp;pid=S1688-12492021000101401&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.edu.uy/scielo.php?script=sci_abstract&amp;pid=S1688-12492021000101401&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.edu.uy/scielo.php?script=sci_pdf&amp;pid=S1688-12492021000101401&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[Resumen: La atrofia muscular espinal (AME) es una enfermedad del asta anterior de la médula espinal, genéticamente determinada y causada por síntesis insuficiente de la proteína de supervivencia de la motoneurona. La debilidad muscular lleva a una disminución progresiva de la capacidad vital y de flujos medibles durante la tos. La intensidad y precocidad de la expresión motora se vincula con los grados de afectación de los grupos musculares respiratorios, determinando la meseta en la capacidad vital y progresión a la insuficiencia ventilatoria, como también el compromiso de los músculos inervados bulbares. Las formas clínicas más severas de AME, en especial aquellas con presentaciones más tempranas y respiración paradojal, tienen capacidades vitales y flujos pico tosidos menores. La evaluación secuencial de estos parámetros es esencial para el pronóstico funcional y vital de estos pacientes. La subclasificación de AME tipo 1 y 2 se relaciona con momentos deseables para la realización de cuidados respiratorios no invasivos en la infancia temprana y en la edad escolar, que mejoran la sobrevida y calidad de vida. Este documento sintetiza dichas recomendaciones con especial referencia a intervenciones guiadas por etapas que incluyan apilamiento de aire (air stacking), protocolos de tos asistida y soporte ventilatorio no invasivo con alta intensidad de presiones de soporte, incluso en aquellos pacientes con pérdida de la autonomía respiratoria, minimizando el riesgo de traqueotomía. La no consideración de estas recomendaciones en la valoración regular de los pacientes resta la oferta de tratamientos oportunos.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[Summary: Spinal Muscular Atrophy (SMA) is a disease of the anterior horn of the spinal cord, genetically determined, and caused by deficiency of survival motor neuron (SMN) protein. Muscle weakness leads to a progressive decrease in vital capacity and to diminished cough flows. Respiratory morbidity and mortality are a function of respiratory and bulbar-innervated muscle impairment. It can be measured by the sequential evaluation of vital capacity to determine the life time maximum (plateau) and its subsequent rate of decline, progressing to ventilatory failure. Bulbar-innervated muscle impairment can also be monitored and measured by spirometry. The more severe clinical forms of SMA, especially those with earlier onsets and paradoxical breathing, have lower vital capacities and cough peak flows. The sequential assessment of these parameters is key for the vital and functional prognosis of these patients. SMA sub-classification types 1 and 2 of SMA involve appropriate times for non-invasive respiratory interventions in early childhood and school age and improve afterlife and quality of life. This document summarizes these recommendations, as a function of SMA type, with special reference to interventions that include air stacking, manually and mechanically assisted coughing protocols and noninvasive ventilatory support techniques, even for patients who have no ventilator-free breathing ability to minimize or eliminate the need to resort to tracheotomy. Failure to properly evaluate these patients regularly reduces their survival and chances to avoid invasive airway tubes.]]></p></abstract>
<abstract abstract-type="short" xml:lang="pt"><p><![CDATA[Resumo: A Atrofia Muscular Espinhal (SMA) é uma doença do corno anterior da medula espinhal, geneticamente determinada e causada pela síntese insuficiente da proteína de sobrevivência dos neurônios motores. A fraqueza muscular leva a uma diminuição progressiva da capacidade vital e fluxos mensuráveis durante a tosse. A intensidade e a precocidade da expressão motora estão relacionadas aos graus de envolvimento dos grupos musculares respiratórios, determinando o platô da capacidade vital e a progressão para insuficiência ventilatória, bem como o envolvimento dos músculos inervados do bulbar. As formas clínicas mais graves de SMA, especialmente aquelas com apresentações anteriores e respiração paradoxal, têm capacidades vitais mais baixas e fluxos de tosse mais baixos. A avaliação sequencial desses parâmetros é essencial para o prognóstico funcional e vital desses pacientes. A subclassificação de SMA tipo 1 e 2 está relacionada aos momentos desejáveis para cuidados respiratórios não invasivos na primeira infância e idade escolar, que melhoram a sobrevida e a qualidade de vida. Este documento resume essas recomendações com referência especial às intervenções guiadas por etapas que incluem empilhamento de ar, protocolos de tosse assistida e suporte ventilatório não invasivo com suporte pressórico de alta intensidade, mesmo em pacientes com perda de autonomia respiratória, minimizando o risco de traqueostomia. A não consideração dessas recomendações na avaliação regular dos pacientes reduz a oferta de tratamentos oportunos.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[Atrofia muscular espinal]]></kwd>
<kwd lng="es"><![CDATA[Ventilación no invasiva]]></kwd>
<kwd lng="es"><![CDATA[Capacidad vital]]></kwd>
<kwd lng="en"><![CDATA[Spinal muscular atrophy]]></kwd>
<kwd lng="en"><![CDATA[Noninvasive ventilation]]></kwd>
<kwd lng="en"><![CDATA[Vital capacity]]></kwd>
<kwd lng="pt"><![CDATA[Atrofia muscular espinal]]></kwd>
<kwd lng="pt"><![CDATA[Ventilação não invasiva]]></kwd>
<kwd lng="pt"><![CDATA[Capacidade vital]]></kwd>
</kwd-group>
</article-meta>
</front><back>
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