Browsing by Subject "Inhalation"
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Item Open Access Effects of respiratory muscle training (RMT) in children with infantile-onset Pompe disease and respiratory muscle weakness.(Journal of pediatric rehabilitation medicine, 2014-01) Jones, Harrison N; Crisp, Kelly D; Moss, Tronda; Strollo, Katherine; Robey, Randy; Sank, Jeffrey; Canfield, Michelle; Case, Laura E; Mahler, Leslie; Kravitz, Richard M; Kishnani, Priya SPurpose
Respiratory muscle weakness is a primary therapeutic challenge for patients with infantile Pompe disease. We previously described the clinical implementation of a respiratory muscle training (RMT) regimen in two adults with late-onset Pompe disease; both demonstrated marked increases in inspiratory and expiratory muscle strength in response to RMT. However, the use of RMT in pediatric survivors of infantile Pompe disease has not been previously reported.Method
We report the effects of an intensive RMT program on maximum inspiratory pressure (MIP) and maximum expiratory pressure (MEP) using A-B-A (baseline-treatment-posttest) single subject experimental design in two pediatric survivors of infantile Pompe disease. Both subjects had persistent respiratory muscle weakness despite long-term treatment with alglucosidase alfa.Results
Subject 1 demonstrated negligible to modest increases in MIP/MEP (6% increase in MIP, d=0.25; 19% increase in MEP, d=0.87), while Subject 2 demonstrated very large increases in MIP/MEP (45% increase in MIP, d=2.38; 81% increase in MEP, d=4.31). Following three-month RMT withdrawal, both subjects maintained these strength increases and demonstrated maximal MIP and MEP values at follow-up.Conclusion
Intensive RMT may be a beneficial treatment for respiratory muscle weakness in pediatric survivors of infantile Pompe disease.Item Open Access Increased inspiratory and expiratory muscle strength following respiratory muscle strength training (RMST) in two patients with late-onset Pompe disease.(Molecular genetics and metabolism, 2011-11) Jones, Harrison N; Moss, Tronda; Edwards, Laurie; Kishnani, Priya SRespiratory muscle strength training (RMST) is an exercise-based intervention which targets respiratory muscle weakness. We implemented RMST in two patients with late-onset Pompe disease (LOPD), both who had received long-term enzyme replacement therapy and had severe respiratory weakness. Over 16-32 weeks, inspiratory muscle strength increased by 73-74%. Expiratory muscle strength increased 31-48% over 12-22 weeks. These findings suggest that RMST may increase respiratory muscle strength, even in the setting of LOPD and severe baseline weakness.Item Open Access Psychiatric disorders in inhalant users: results from The National Epidemiologic Survey on Alcohol and Related Conditions.(Drug and alcohol dependence, 2007-05) Wu, Li-Tzy; Howard, Matthew OwenTo examine the prevalence and correlates of mood, anxiety, and personality disorders among lifetime inhalant users.Statistical analyses were based on data from the 2001-2002 National Epidemiologic Survey on Alcohol and Related Conditions (NESARC), a nationally representative survey of adults in the United States.Inhalant users (N=664) had high lifetime prevalences of DSM-IV mood (48%), anxiety (36%), and personality (45%) disorders. Of all inhalant users, 70% met criteria for at least one lifetime mood, anxiety, or personality disorder and 38% experienced a mood or anxiety disorder in the past year. Prevalences of comorbid psychiatric disorders varied by gender. Compared with male inhalant users, female inhalant users had higher prevalences of lifetime dysthymia (24% versus 16%), any anxiety disorder (53% versus 30%), panic disorder without agoraphobia (25% versus 11%), and specific phobia (28% versus 14%), but a lower prevalence of antisocial personality disorder (22% versus 36%). Female inhalant users also were more likely than male inhalant users to meet criteria for three or more mood or anxiety disorders (15% versus 8%) in the past year. Among inhalant users with comorbid disorders, those who developed social or specific phobia typically experienced onset of these disorders prior to initiation of inhalant use; all other mood and anxiety disorders usually developed following the onset of inhalant use. Inhalant users who were women, poor, less educated, with early onset of inhalant use, family histories of psychopathology, and personal histories of substance abuse treatment had increased odds of psychiatric disorders.Psychiatric disorders are highly prevalent among inhalant users nationally and female inhalant users are more likely than male inhalant users to experience multiple psychiatric disorders. Inhalant use and its consequences among females warrant greater research attention.Item Open Access Respiratory muscle training in late-onset Pompe disease: Results of a sham-controlled clinical trial.(Neuromuscular disorders : NMD, 2020-11) Jones, Harrison N; Kuchibhatla, Maragatha; Crisp, Kelly D; Hobson-Webb, Lisa D; Case, Laura; Batten, Milisa T; Marcus, Jill A; Kravitz, Richard M; Kishnani, Priya STo address progressive respiratory muscle weakness in late-onset Pompe disease (LOPD), we developed a 12-week respiratory muscle training (RMT) program. In this exploratory, double-blind, randomized control trial, 22 adults with LOPD were randomized to RMT or sham-RMT. The primary outcome was maximum inspiratory pressure (MIP). Secondary and exploratory outcomes included maximum expiratory pressure (MEP), peak cough flow, diaphragm ultrasound, polysomnography, patient-reported outcomes, and measures of gross motor function. MIP increased 7.6 cmH2O (15.9) in the treatment group and 2.7 cmH2O (7.6) in the control group (P = 0.4670). MEP increased 14.0 cmH2O (25.9) in the treatment group and 0.0 cmH2O (12.0) in the control group (P = 0.1854). The only statistically significant differences in secondary/exploratory outcomes were improvements in time to climb 4 steps (P = 0.0346) and daytime sleepiness (P = 0.0160). The magnitude of changes in MIP and MEP in the treatment group were consistent with our pilot findings but did not achieve statistical significance in comparison to controls. Explanations for this include inadequate power and baseline differences in subject characteristics between groups. Additionally, control group subjects appeared to exhibit an active response to sham-RMT and therefore sham-RMT may not be an optimal control condition for RMT in LOPD.