Factors associated with lower forced vital capacity in children and adults with Duchenne muscular dystrophy using non-invasive ventilation: a multicenter analysis.
Kanokkarn Sunkonkit, Manju Hurvitz, Andrew Defante, Jeremy Orr, Abhishek Chakraborty, Reshma Amin, Rakesh Bhattacharjee
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Abstract
Background: Reduced forced vital capacity (FVC) is associated with morbidity and mortality in individuals with Duchenne muscular dystrophy (DMD). Non-invasive ventilation (NIV) is often prescribed for the treatment of sleep-disordered breathing (SDB), and chronic respiratory insufficiency. Despite the common practice of initiating NIV later in the progression of DMD, the factors influencing FVC subsequent to the commencement of NIV remain unclear.
Objective: To evaluate the demographic, clinical and socioeconomic determinants of FVC% predicted across several cohorts of DMD children and adults prescribed NIV.
Methods: A multicenter retrospective review of individuals with DMD prescribed NIV was performed between February 2016 to October 2020. Patients were identified from three sites: The Hospital for Sick Children, Canada; Rady Children's Hospital San Diego, USA; and University of California San Diego Health, USA. Multivariate regression analysis was performed to determine factors that influence FVC.
Results: Fifty-nine male patients with DMD prescribed NIV (mean ± SD for age and BMI was 20.1 ± 6.7 years and 23.8 ± 8.8 kg/m2) were included. Following multivariate analysis, a lower FVC% predicted was associated with older age (β = -1.44, p = 0.001), presence of scoliosis (β = -16.94, p = 0.002), absent deflazacort prescription (β = 14.43, p = 0.009), and use of in-ex sufflator (β = -39.73, p < 0.001), respectively.
Conclusion: In our study, several factors were associated with reduced FVC% predicted in a DMD population using NIV. Future, prospective, longitudinal studies are imperative to comprehend the trajectory of FVC% predicted over time in individuals with DMD using NIV.
期刊介绍：
The journal Sleep and Breathing aims to reflect the state of the art in the international science and practice of sleep medicine. The journal is based on the recognition that management of sleep disorders requires a multi-disciplinary approach and diverse perspectives. The initial focus of Sleep and Breathing is on timely and original studies that collect, intervene, or otherwise inform all clinicians and scientists in medicine, dentistry and oral surgery, otolaryngology, and epidemiology on the management of the upper airway during sleep.
Furthermore, Sleep and Breathing endeavors to bring readers cutting edge information about all evolving aspects of common sleep disorders or disruptions, such as insomnia and shift work. The journal includes not only patient studies, but also studies that emphasize the principles of physiology and pathophysiology or illustrate potentially novel approaches to diagnosis and treatment. In addition, the journal features articles that describe patient-oriented and cost-benefit health outcomes research. Thus, with peer review by an international Editorial Board and prompt English-language publication, Sleep and Breathing provides rapid dissemination of clinical and clinically related scientific information. But it also does more: it is dedicated to making the most important developments in sleep disordered breathing easily accessible to clinicians who are treating sleep apnea by presenting well-chosen, well-written, and highly organized information that is useful for patient care.
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