Resumo

HUERTA, Antonio et al. Extubation of patients with type 1 spinal muscular atrophy. Literature review. Arch. Pediatr. Urug. [online]. 2024, vol.95, n.2, e902.  Epub 01-Dez-2024. ISSN 0004-0584.  https://doi.org/10.31134/ap.95.2.20.

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.

Palavras-chave : Spinal Muscular Atrophy; Ventilator Disconnection; Non-invasive Ventilation; Artificial Respiration; Tracheal Extubation.

        · resumo em Português | Espanhol     · texto em Espanhol     · Espanhol ( pdf )