Abstract
BACKGROUND: The population with Down syndrome (DS) makes up a significant portion of patients using short- and long-term noninvasive ventilation (NIV). However, limited data are available concerning the use and outcomes for patients with DS compared with other populations. We systematically reviewed articles providing indications, benefits, adherence and limits of long-term NIV use in the paediatric and adult population with DS. METHODS: A systematic review of the literature for articles published between January 2013 and November 2023 was conducted using a protocol registered at PROSPERO (CRD42023488712). RESULTS: In total, 18 studies focusing on the use of noninvasive respiratory support in patients with DS were included in this review. Obstructive sleep apnoea syndrome (OSAS), OSAS post-adenotonsillectomy, hypoventilation and nocturnal hypercapnia were the most common indications for NIV support in patients with DS. However, respiratory anatomical malformation and intellectual disabilities can affect adherence to NIV use. High-flow nasal cannula is an alternative to other noninvasive respiratory support. CONCLUSION: Comprehensive and updated consensus compliant with international standards for clinical guidelines is required to significantly improve clinical practice quality, promote evidence-based recommendations and provide tailored interventions in patients with DS.