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Revista chilena de pediatría

versión impresa ISSN 0370-4106

Resumen

PALOMINO, María Angélica  y  CASTIGLIONI, Claudia. Respiratory care in spinal muscular atrophy in the new therapeutic era. Rev. chil. pediatr. [online]. 2018, vol.89, n.6, pp.685-693. ISSN 0370-4106.  http://dx.doi.org/10.4067/S0370-41062018005001208.

Spinal muscular atrophy (SMA) is the first inherited cause of mortality in infants, with four subtypes: SMA0 prenatal onset, SMA1 babies less than 3 months non sitters, SMA2 sitters and SMA3 walkers. Pneumonia and respiratory insufficiency are the most severe complications. Informed parental de cisions are relevant. Respiratory management includes cough assistance, prevention of lung under development due to chest deformity, prompt treatment of respiratory infections, hypoventilation, swallow problems, gastro esophageal reflux and malnutrition. In view of the FDA and EMA approval of the nonsense oligonucleotides nusinersen, the first specific treatment for SMA and the future with gene therapy and others under development, we need to optimize preventive respiratory manage ment with the new standard of care.

Palabras clave : Spinal Muscular Atrophy; Neuromuscular disease; Werdnig Hoffmann’s Disease; Airway clearance; Mechanical cough assistance; Pneumonia; Respiratory failure; Restrictive lung disease; Non-invasive ventilation, Invasive ventilation; Nusinersen.

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