Factors associated with lower forced vital capacity in children and adults with Duchenne muscular dystrophy using non-invasive ventilation: a multicenter analysis

• DOI: 10.1007/s11325-024-03183-1
• Publication Date: 2025-01-07T07:26:59Z
• Authors: Kanokkarn Sunkonkit; Manju Hurvitz; Andrew Defante; Jeremy Orr; Abhishek Chakraborty; Reshma Amin; Rakesh Bhattacharjee
• Keywords: Vital capacity

Abstract Background Reduced forced vital capacity (FVC) is associated with morbidity and mortality in individuals Duchenne muscular dystrophy (DMD). Non-invasive ventilation (NIV) often prescribed for the treatment of sleep-disordered breathing (SDB), chronic respiratory insufficiency. Despite common practice initiating NIV later progression DMD, factors influencing FVC subsequent to commencement remain unclear. Objective To evaluate demographic, clinical socioeconomic determinants FVC% predicted across several cohorts DMD children adults NIV. Methods A multicenter retrospective review was performed between February 2016 October 2020. Patients were identified from three sites: The Hospital Sick Children, Canada; Rady Children’s San Diego, USA; University California Diego Health, USA. Multivariate regression analysis determine that influence FVC. Results Fifty-nine male patients (mean ± SD age BMI 20.1 6.7 years 23.8 8.8 kg/m 2 ) included. Following multivariate analysis, a lower older (β = -1.44, p 0.001 ), presence scoliosis -16.94, 0.002 absent deflazacort prescription 14.43, 0.009 use in-ex sufflator -39.73, < respectively. Conclusion In our study, reduced population using Future, prospective, longitudinal studies are imperative comprehend trajectory over time