Respiratory management of spinal muscular atrophy type 1 patients treated with Nusinersen.

Joris MenardAndreea M SeferianEmmanuelle FleurenceAudrey BarzicAlexandra BinocheGéraldine LabouretLaurianne Coutier Carole VuillerotBlaise M BieleuMarta Gomez Garcia de la BandaHarriet Corvol Laurent Servais Jessica Taytard

Published in: Pediatric pulmonology (2022)

This study shows that patients do not present with nocturnal hypoventilation before respiratory decompensations and NIV initiation, and suggests that a delay in NIV initiation might result in respiratory complications. There is a need for disease-centered guidelines for the respiratory management of these patients, including NIV indications.

Keyphrases

end stage renal disease
ejection fraction
newly diagnosed
chronic kidney disease
peritoneal dialysis
prognostic factors
obstructive sleep apnea
depressive symptoms
respiratory tract
clinical practice