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Long-Term Non-invasive Ventilation in Children With Down Syndrome: A Systematic Review
Summer Hudson1, Tamer Abusido2,3, Meghan Sebastianski4
1MD Program, Department of Pediatrics, Faculty of Medicine & Dentistry, University of Alberta, Edmonton, AB, Canada.
Insights
Children with Down syndrome using long-term non-invasive ventilation (NIV) show comparable efficacy and adherence to other children. However, they may face challenges initiating NIV, necessitating further research into optimal support strategies.
Area of Science:
- Pediatric Respiratory Medicine
- Genetics and Genetic Diseases
- Sleep Medicine
Background:
- Children with Down syndrome (DS) have a high risk of obstructive sleep apnea and other respiratory issues.
- Long-term non-invasive ventilation (NIV), including CPAP and BiPAP, is an alternative treatment for these conditions.
Approach:
- A scoping review systematically searched multiple databases (MEDLINE, Embase, CINAHL, Cochrane, PubMed) from 1990-2021.
- Identified 28 articles detailing the use and outcomes of NIV in 543 children with DS.
- Extracted data on study and subject characteristics, technology, and outcomes.
Key Points:
- Children with DS represent 18% of pediatric NIV users.
- Efficacy, feasibility, and adherence in children with DS are comparable to other pediatric populations.
- Children with DS may experience greater difficulty initiating NIV and require longer to establish use.
Conclusions:
- Long-term NIV is an effective and well-tolerated therapy for children with DS.
- Limited outcome data suggest potential benefits for cardiac function and attention.
- Further research is needed to address challenges in establishing NIV use for children with DS.
Context:
Children with Down syndrome are at risk for obstructive sleep apnea, which may not be resolved by adenotonsillecotmy, as well as other respiratory disorders that may impact breathing during sleep. Long-term non-invasive ventilation, including continuous and bilevel positive airway pressure delivery, is an alternate treatment strategy.
Objective:
To assess the use and outcomes of long-term non-invasive ventilation in children with Down syndrome including comparison to other children using long-term non-invasive ventilation.
Data Sources:
The search strategy for the scoping review used Medical Subject Headings (MeSH) and free-text terms for "child" and "non-invasive ventilation." MEDLINE (Ovid), Embase (Ovid), CINAHL (Ebsco), Cochrane Library (Wiley), and PubMed databases were searched (1990-2021).
Study Selection:
The scoping review results were searched to identify studies including data on at least three children with Down Syndrome using long-term non-invasive ventilation.
Data Extraction:
Study characteristics, subject characteristics, technology type, and outcome measurements were extracted.
Results:
A total of 28 articles included 543 children with Down syndrome using long-term non-invasive ventilation. Children with Down syndrome accounted for 18% of children using long-term non-invasive ventilation. Data on efficacy, feasibility, and adherence in children with Down syndrome are comparable to other children. Children with Down syndrome may have greater difficulty initiating long-term non-invasive ventilation, longer time to establish use, and a higher rate of inability to establish use. Outcome data is limited but suggest favorable impact on cardiac function and attention.
Limitations:
Articles related to long-term non-invasive ventilation use in adolescents and young adults may have been excluded.
Conclusions:
Children with Down syndrome make up a significant portion of the population of children using long-term non-invasive ventilation. While there is more limited data available with respect to the use and outcomes for children with Down syndrome compared to the other children, long-term non-invasive ventilation is an effective and well-tolerated therapy with no clear differences in the use or outcomes for children with Down syndrome. Additional work is needed to understand potential challenges around establishing long-term non-invasive ventilation use in children with Down syndrome.
Systematic Review Registration:
https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=206533, identifier: CRD206533.
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