Resumo
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.
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