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Barbara K. Smith D.P.T.

Courtesy Research Assistant Professor

Department: Department of Neurology

Publications

2024
Effects of gene replacement therapy with resamirigene bilparvovec (AT132) on skeletal muscle pathology in X-linked myotubular myopathy: results from a substudy of the ASPIRO open-label clinical trial.
EBioMedicine.99():104894-[DOI] 10.1016/j.ebiom.2023.104894.[PMID] 38086156.
PubMed·Publisher's Site
2024
Longitudinal changes of swallowing safety and efficiency in infants with spinal muscular atrophy who received disease modifying therapies
Pediatric Pulmonology.():-[DOI] 10.1002/ppul.26919.[PMID] .
PubMed·Publisher's Site
2023
Diaphragm pacing and independent breathing in individuals with severe Pompe disease.
Frontiers in rehabilitation sciences.4():1184031-[DOI] 10.3389/fresc.2023.1184031.[PMID] 37583873.
PubMed·Publisher's Site
2023
Intraoperative Hemi-Diaphragm Electrical Stimulation Demonstrates Attenuated Mitochondrial Function without Change in Oxidative Stress in Cardiothoracic Surgery Patients.
Antioxidants (Basel, Switzerland).12(5):-[DOI] 10.3390/antiox12051009.[PMID] 37237876.
PubMed·Publisher's Site
2023
On “Clinical and Research Readiness for Spinal Muscular Atrophy: The Time Is Now for Knowledge Translation.” Krosschell KJ, Young SD, Peterson I, et al. Phys Ther. 2022;102:pzac108. https://doi.org/10.1093/Ptj/pzac108
Physical Therapy.103(3):-[DOI] 10.1093/ptj/pzad008.[PMID] .
PubMed·Publisher's Site
2023
Polysomnography findings in children with spinal muscular atrophy after onasemnogene-abeparvovec.
Sleep medicine.101():234-237[DOI] 10.1016/j.sleep.2022.11.006.[PMID] 36442421.
7 citations·PubMed·Publisher's Site
2023
Predictors of Peak Expiratory Cough Flow in Individuals with Amyotrophic Lateral Sclerosis.
Dysphagia.38(2):719-725[DOI] 10.1007/s00455-022-10503-8.[PMID] 35931882.
1 citations·PubMed·Publisher's Site
2023
Respiratory therapies for Amyotrophic Lateral Sclerosis: A state of the art review
Chronic Respiratory Disease.20():147997312311759-[DOI] 10.1177/14799731231175915.[PMID] 37219417.
2 citations·PubMed·Publisher's Site
2023
Safety and efficacy of gene replacement therapy for X-linked myotubular myopathy (ASPIRO): a multinational, open-label, dose-escalation trial.
The Lancet. Neurology.22(12):1125-1139[DOI] 10.1016/S1474-4422(23)00313-7.[PMID] 37977713.
5 citations·PubMed·Publisher's Site
2022
Acute intermittent hypoxia and respiratory muscle recruitment in people with amyotrophic lateral sclerosis: A preliminary study.
Experimental neurology.347():113890-[DOI] 10.1016/j.expneurol.2021.113890.[PMID] 34624328.
14 citations·PubMed·Publisher's Site
2022
Characterizing Expiratory Respiratory Muscle Degeneration in Duchenne Muscular Dystrophy Using MRI.
Chest.161(3):753-763[DOI] 10.1016/j.chest.2021.08.078.[PMID] 34536384.
4 citations·PubMed·Publisher's Site
2022
Effect of Test Interface on Respiratory Muscle Activity and Pulmonary Function During Respiratory Testing in Healthy Adults: A Pilot Study.
Cardiopulmonary physical therapy journal.33(2):87-95[DOI] 10.1097/cpt.0000000000000183.[PMID] 36148286.
PubMed·Publisher's Site
2022
Exploring inspiratory occlusion metrics to assess respiratory drive in patients under acute intermittent hypoxia.
Respiratory physiology & neurobiology.304():103922-[DOI] 10.1016/j.resp.2022.103922.[PMID] 35680039.
1 citations·PubMed·Publisher's Site
2022
INCEPTUS Natural History, Run-in Study for Gene Replacement Clinical Trial in X-Linked Myotubular Myopathy.
Journal of neuromuscular diseases.9(4):503-516[DOI] 10.3233/JND-210781.[PMID] 35694931.
4 citations·PubMed·Publisher's Site
2021
Case Studies in Neuroscience: Neuropathology and diaphragm dysfunction in ventilatory failure from late-onset Pompe disease
Journal of Neurophysiology.126(2):351-360[DOI] 10.1152/jn.00190.2021.[PMID] 34191636.
7 citations·PubMed·Publisher's Site
2020
Diaphragm remodelling following cervical spinal cord injury: Can intrinsic neural plasticity be harnessed to improve respiratory motor function?
The Journal of physiology.598(11):2049-2050[DOI] 10.1113/JP279707.[PMID] 32266973.
PubMed·Publisher's Site
2020
Ultrafast ultrasound responses to twitch stimulation: bridging the gap between non‐volitional and non‐invasive tests of diaphragm contractility
The Journal of Physiology.598(24):5599-5600[DOI] 10.1113/jp280863.[PMID] .
PubMed·Publisher's Site
2019
Cough Effectiveness and Pulmonary Hygiene Practices in Patients with Pompe Disease
Lung.197(1):1-8[DOI] 10.1007/s00408-018-0171-1.[PMID] 30361764.
9 citations·PubMed·Publisher's Site
2019
Dynamic respiratory muscle function in late-onset Pompe disease
Scientific Reports.9(1):-[DOI] 10.1038/s41598-019-54314-8.[PMID] 31831753.
6 citations·PubMed·Publisher's Site
2019
Imaging respiratory muscle quality and function in Duchenne muscular dystrophy.
Journal of neurology.266(11):2752-2763[DOI] 10.1007/s00415-019-09481-z.[PMID] 31350642.
22 citations·PubMed·Publisher's Site
2019
Inspiratory Muscle Training in Patients with Prolonged Mechanical Ventilation: Narrative Review.
Cardiopulmonary physical therapy journal.30(1):44-50[DOI] 10.1097/CPT.0000000000000092.[PMID] 31105474.
9 citations·PubMed·Publisher's Site
2019
Lower Extremity Functional Outcome Measures in Duchenne Muscular Dystrophy-A Delphi Survey.
Journal of neuromuscular diseases.6(1):75-83[DOI] 10.3233/JND-180337.[PMID] 30562905.
5 citations·PubMed·Publisher's Site
2019
Pompe disease gene therapy: neural manifestations require consideration of CNS directed therapy.
Annals of translational medicine.7(13):290-[DOI] 10.21037/atm.2019.05.56.[PMID] 31392202.
31 citations·PubMed·Publisher's Site
2018
Mechanical Ventilation for Duchenne Muscular Dystrophy: Sinner or Saint?
Muscle & nerve.57(3):353-355[DOI] 10.1002/mus.25986.[PMID] 29023941.
PubMed·Publisher's Site
2018
Strategies for the Integration of Cough and Swallow to Maintain Airway Protection in Humans.
Lung.196(5):601-608[DOI] 10.1007/s00408-018-0133-7.[PMID] 29926179.
13 citations·PubMed·Publisher's Site
2017
Inspiratory muscle conditioning exercise and diaphragm gene therapy in Pompe disease: Clinical evidence of respiratory plasticity.
Experimental neurology.287(Pt 2):216-224[DOI] 10.1016/j.expneurol.2016.07.013.[PMID] 27453480.
33 citations·PubMed·Publisher's Site
2017
Safety of Intradiaphragmatic Delivery of Adeno-Associated Virus-Mediated Alpha-Glucosidase (rAAV1-CMV-hGAA) Gene Therapy in Children Affected by Pompe Disease.
Human gene therapy. Clinical development.28(4):208-218[DOI] 10.1089/humc.2017.146.[PMID] 29160099.
76 citations·PubMed·Publisher's Site
2017
Systemic AAV8-Mediated Gene Therapy Drives Whole-Body Correction of Myotubular Myopathy in Dogs.
Molecular therapy : the journal of the American Society of Gene Therapy.25(4):839-854[DOI] 10.1016/j.ymthe.2017.02.004.[PMID] 28237839.
70 citations·PubMed·Publisher's Site
2016
Altered activation of the diaphragm in late-onset Pompe disease.
Respiratory physiology & neurobiology.222():11-5[DOI] 10.1016/j.resp.2015.11.013.[PMID] 26612101.
16 citations·PubMed·Publisher's Site
2016
Diaphragm Pacing as a Rehabilitative Tool for Patients With Pompe Disease Who Are Ventilator-Dependent: Case Series.
Physical therapy.96(5):696-703[DOI] 10.2522/ptj.20150122.[PMID] 26893511.
16 citations·PubMed·Publisher's Site
2016
Intraoperative hemidiaphragm electrical stimulation reduces oxidative stress and upregulates autophagy in surgery patients undergoing mechanical ventilation: exploratory study.
Journal of translational medicine.14(1):305-[DOI] .[PMID] 27784315.
26 citations·PubMed·Publisher's Site
2016
Reply: Respiratory motor function in centronuclear myopathy.
Muscle & nerve.53(4):660-1[DOI] 10.1002/mus.24961.[PMID] 26561983.
2 citations·PubMed·Publisher's Site
2016
Respiratory motor function in individuals with centronuclear myopathies.
Muscle & nerve.53(2):214-21[DOI] 10.1002/mus.24899.[PMID] 26351754.
6 citations·PubMed·Publisher's Site
2015
Altered activation of the tibialis anterior in individuals with Pompe disease: Implications for motor unit dysfunction.
Muscle & nerve.51(6):877-83[DOI] 10.1002/mus.24444.[PMID] 25186912.
17 citations·PubMed·Publisher's Site
2015
Muscle pathology, limb strength, walking gait, respiratory function and neurological impairment establish disease progression in the p.N155K canine model of X-linked myotubular myopathy.
Annals of translational medicine.3(18):262-[DOI] 10.3978/j.issn.2305-5839.2015.10.31.[PMID] 26605308.
10 citations·PubMed·Publisher's Site
2014
Effect of intermittent phrenic nerve stimulation during cardiothoracic surgery on mitochondrial respiration in the human diaphragm.
Critical care medicine.42(2):e152-6[DOI] 10.1097/CCM.0b013e3182a63fdf.[PMID] 24126442.
54 citations·PubMed·Publisher's Site
2014
Intrinsic transient tracheal occlusion training and myogenic remodeling of rodent parasternal intercostal fibers.
Journal of rehabilitation research and development.51(5):841-54[DOI] .[PMID] 25509059.
1 citations·PubMed·Publisher's Site
2014
Phrenic nerve stimulation increases human diaphragm fiber force after cardiothoracic surgery.
American journal of respiratory and critical care medicine.190(7):837-9[DOI] 10.1164/rccm.201405-0993LE.[PMID] 25271750.
36 citations·PubMed·Publisher's Site
2014
Respiratory assessment in centronuclear myopathies.
Muscle & nerve.50(3):315-26[DOI] 10.1002/mus.24249.[PMID] 24668768.
6 citations·PubMed·Publisher's Site
2014
Transcriptional regulation of myotrophic actions by testosterone and trenbolone on androgen-responsive muscle.
Steroids.87():59-66[DOI] 10.1016/j.steroids.2014.05.024.[PMID] 24928725.
20 citations·PubMed·Publisher's Site
2013
Acid Alpha-Glucosidase Gene Replacement Therapy To the Diaphragm in Ventilator-Dependent Pompe Disease: One-Year Respiratory Motor Outcomes
Molecular Therapy.21():S23-S24[DOI] .[PMID] .
PubMed·Publisher's Site
2013
Adeno-associated virus-mediated gene therapy for metabolic myopathy.
Human gene therapy.24(11):928-36[DOI] 10.1089/hum.2013.2514.[PMID] 24164240.
8 citations·PubMed·Publisher's Site
2013
Clinical Trial Readiness for Gene Replacement To the Diaphragm: Translational Respiratory Endpoints in Ventilator-Dependent Neuromuscular Diseases
Molecular Therapy.21():S235-[DOI] .[PMID] .
PubMed·Publisher's Site
2013
Inspiratory muscle strength training in infants with congenital heart disease and prolonged mechanical ventilation: a case report.
Physical therapy.93(2):229-36[DOI] 10.2522/ptj.20110348.[PMID] 22466028.
5 citations·PubMed·Publisher's Site
2013
Mechanical ventilation, diaphragm weakness and weaning: a rehabilitation perspective.
Respiratory physiology & neurobiology.189(2):377-83[DOI] 10.1016/j.resp.2013.05.012.[PMID] 23692928.
40 citations·PubMed·Publisher's Site
2013
Phase I/II trial of adeno-associated virus-mediated alpha-glucosidase gene therapy to the diaphragm for chronic respiratory failure in Pompe disease: initial safety and ventilatory outcomes.
Human gene therapy.24(6):630-40[DOI] 10.1089/hum.2012.250.[PMID] 23570273.
110 citations·PubMed·Publisher's Site
2013
The respiratory neuromuscular system in Pompe disease.
Respiratory physiology & neurobiology.189(2):241-9[DOI] 10.1016/j.resp.2013.06.007.[PMID] 23797185.
80 citations·PubMed·Publisher's Site
2012
Blunted Ventilatory Response To Central Nervous Stimulation in a Canine Model of X-Linked Myotubular Myopathy
Molecular Therapy.20():S173-[DOI] .[PMID] .
PubMed·Publisher's Site
2012
Chronic intrinsic transient tracheal occlusion elicits diaphragmatic muscle fiber remodeling in conscious rodents.
PloS one.7(11):e49264-[DOI] 10.1371/journal.pone.0049264.[PMID] 23133678.
9 citations·PubMed·Publisher's Site
2012
Clinical Methods of Intramuscular Diaphragm Injections for Gene Therapy
Molecular Therapy.20():S174-S175[DOI] .[PMID] .
PubMed·Publisher's Site
2012
Establishing clinical end points of respiratory function in large animals for clinical translation.
Physical medicine and rehabilitation clinics of North America.23(1):75-94, xi[DOI] 10.1016/j.pmr.2011.11.017.[PMID] 22239876.
12 citations·PubMed·Publisher's Site
2011
Gene expression changes in the human diaphragm after cardiothoracic surgery.
The Journal of thoracic and cardiovascular surgery.142(5):1214-22, 1222.e1[DOI] 10.1016/j.jtcvs.2011.02.025.[PMID] 21463877.
13 citations·PubMed·Publisher's Site
2011
Group-Advantaged Training of Research (Gator): a Metamorphosis of Mentorship
Bioscience.61(4):301-311[DOI] 10.1525/bio.2011.61.4.10.[PMID] .
PubMed·Publisher's Site
2011
Inspiratory muscle strength training improves weaning outcome in failure to wean patients: a randomized trial.
Critical care (London, England).15(2):R84-[DOI] 10.1186/cc10081.[PMID] 21385346.
137 citations·PubMed·Publisher's Site
2011
Inspiratory muscle training in a child with nemaline myopathy and organ transplantation.
Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies.12(2):e94-8[DOI] 10.1097/PCC.0b013e3181dde680.[PMID] 20407395.
12 citations·PubMed·Publisher's Site
2011
Pompe disease gene therapy.
Human molecular genetics.20(R1):R61-8[DOI] 10.1093/hmg/ddr174.[PMID] 21518733.
71 citations·PubMed·Publisher's Site