search
Back to results

Applying pGz in Mitochondrial Disease

Primary Purpose

Mitochondrial Myopathies, Mitochondrial Diseases

Status
Recruiting
Phase
Phase 1
Locations
United States
Study Type
Interventional
Intervention
Cardiopulmonary Exercise Testing
pGz Bed
Gentle Jogger
Exercise Pedal
Lumason® contrast agent
Sponsored by
Children's Hospital of Philadelphia
About
Eligibility
Locations
Arms
Outcomes
Full info

About this trial

This is an interventional diagnostic trial for Mitochondrial Myopathies focused on measuring Passive Exercise, periodic acceleration

Eligibility Criteria

10 Years - 60 Years (Child, Adult)All SexesAccepts Healthy Volunteers

Aim 1 Enrollment Criteria Inclusion Criteria for Healthy Controls

  • Males or females, 10 years to 60 years, with a minimum height for participation of 135 cm
  • Ambulatory and able to complete routine clinical exercise testing
  • Willing and able to complete all study procedures
  • For individuals under the age of 18, parental/guardian permission (informed consent) and as appropriate, child assent
  • For individuals over the age of 18 the ability to provide informed consent

Inclusion Criteria for PMD Patients

  • Males or females, 10 years to 60 years, with a minimum height for participation of 135 cm
  • Ambulatory and able to complete routine clinical exercise testing
  • Willing and able to complete all study procedures
  • Genetically confirmed mitochondrial myopathy (MM) as defined by a diagnosis of primary mitochondrial disease (PMD) with predominant symptoms of myopathy as expressed by exercise intolerance and muscle weakness and fatigue
  • Parental/guardian permission (informed consent) and as appropriate, child assent

Exclusion Criteria for All Aim 1 Participants General Exclusion Criteria

  • Tracheostomy
  • Non-ambulatory
  • Unable to complete routine exercise testing
  • Diagnosed with or have symptoms of vertigo
  • Within 1 month of a recent hospital admission for acute illness
  • Severe co-existing cardiac or pulmonary disease
  • Cognitive impairment that may preclude ability to comply with study procedures
  • Pregnant or lactating females
  • Active alcohol and/or substance abuse
  • At the discretion of the principal investigator (PI), any medical condition that will interfere with or prevent the safe completion of the study
  • Parents/guardians or subjects who, in the opinion of the Investigator, may be non-compliant with study schedules or procedures
  • Use of investigational agent(s) within 4 weeks
  • Individuals who are employed by the U.S. Department of Defense, including U.S military personal
  • Patients with biliary atresia with asplenia or polysplenia.
  • Patients with prior liver transplant.
  • Patients with cystic fibrosis.
  • Patients with chronic lung disease.
  • Patients with portal vein thrombosis, cavernous transformation of the portal vein or absent portal vein.
  • Patients with significant heart disease or severe congenital heart disease.
  • Patients with a history of allergic reaction to Lumason®, sulfur hexafluoride, sulfur hexafluoride lipid microsphere components, or other ingredients in Lumason (polyethylene glycol, distearoylphosphatidlycholine (DSPC), dipalmitoylphosphatidylglycerol sodium (DPPG-Na, palmitic acid) or other components of the ultrasound contrast agent
  • Any history of intraocular injury or fragment in or around the orbit that cannot be cleared through radiologic evaluation
  • Any history of bullet, shrapnel, or stabbing wounds that cannot be cleared through radiologic evaluation
  • Past or current employment involving (or exposure to) a metal grinder (e.g., at a construction worksite)
  • Claustrophobia or any known medical conditions which can be exacerbated by stress, anxiety, or panic attacks triggered by enclosed spaces
  • Inability to lie flat in an MRI scanner for up to 45 minutes
  • Unable to perform sub-maximal ankle dorsiflexion leg exercise during the MRI study

Aim 2 Enrollment Criteria Inclusion Criteria for PICU PMD Non-Ambulatory Patients

  • Males or females ages 10 to 23 years (children and adults)
  • Non-ambulatory
  • Genetically confirmed mtDNA-PMD
  • Cooperative and capable of following research procedures
  • Have cognitive ability to enable cooperation with study procedures
  • Admitted to the PICU with an anticipated length of stay for >24 hours
  • Willing and able to complete all study procedures
  • For individuals under the age of 18, parental/guardian permission (informed consent) and as appropriate, child assent
  • For individuals over the age of 18 the ability to provide informed consent

Inclusion Criteria for PICU non-PMD neuromuscular diagnosis

  • Males or females ages 10 to 23 years (children and adults)
  • Non-ambulatory
  • Genetically confirmed non-PMD neuromuscular diagnosis
  • Cooperative and capable of following research procedures
  • Have cognitive ability to enable cooperation with study procedures
  • Admitted to the PICU with an anticipated length of stay for >24 hours
  • For individuals under the age of 18, parental/guardian permission (informed consent) and as appropriate, child assent
  • For individuals over the age of 18 the ability to provide informed consent

Inclusion Criteria for all other PICU Participants

  • Males or females ages 10 to 23 years (children and adults)
  • Non-ambulatory
  • No known genetic diagnosis with healthy pre-morbid status, admitted to PICU
  • Cooperative and capable of following research procedures
  • Have cognitive ability to enable cooperation with study procedures
  • Admitted to the PICU with an anticipated length of stay for >24 hours
  • Willing and able to complete all study procedures
  • For individuals under the age of 18, parental/guardian permission (informed consent) and as appropriate, child assent
  • For individuals over the age of 18 the ability to provide informed consent

Exclusion Criteria for All Aim 2 Participants

  • Have cognitive impairment that may preclude ability to comply with study procedures
  • Have cardiorespiratory instability
  • Patients in whom are so sick that they will not be able to cooperate with the study procedures
  • Have clear contraindications to mobilization
  • Have fixed lower limb deformities/contractures that would prohibit lower extremity exercise
  • Pregnant or lactating females
  • Active alcohol and/or substance abuse
  • At the discretion of the principal investigator (PI), any medical condition that will interfere with or prevent the safe completion of the study
  • Use of investigational agent(s) within 4 weeks
  • Individual who are employed by the U.S. Department of Defense, including U.S military personal
  • Patients with biliary atresia with asplenia or polysplenia.
  • Patients with prior liver transplant.
  • Patients with cystic fibrosis.
  • Patients with chronic lung disease.
  • Patients with portal vein thrombosis, cavernous transformation of the portal vein or absent portal vein.
  • Patients with significant heart disease or severe congenital heart disease.
  • Patients with a history of allergic reaction to Lumason®, sulfur hexafluoride, sulfur hexafluoride lipid microsphere components, or other ingredients in Lumason (polyethylene glycol, distearoylphosphatidlycholine (DSPC), dipalmitoylphosphatidylglycerol sodium (DPPG-Na, palmitic acid) or other components of the ultrasound contrast agent

Exclusion Criteria Specific to study procedure: CrCEST MRI Scan:

  • Any history of intraocular injury or fragment in or around the orbit that cannot be cleared through radiologic evaluation
  • Any history of bullet, shrapnel, or stabbing wounds that cannot be cleared through radiologic evaluation
  • Past or current employment involving (or exposure to) a metal grinder (e.g., at a construction worksite)
  • Claustrophobia or any known medical conditions which can be exacerbated by stress, anxiety, or panic attacks triggered by enclosed spaces
  • Inability to lie flat in an MRI scanner for up to 45 minutes
  • Unable to perform sub-maximal ankle dorsiflexion leg exercise during the MRI study

Sites / Locations

  • Children's Hospital of PhiladelphiaRecruiting

Arms of the Study

Arm 1

Arm 2

Arm 3

Arm Type

Experimental

Experimental

Experimental

Arm Label

Aim 1: Primary Mitochondrial Disease Patients

Aim 1: Healthy Controls

Aim 2: PICU Patients

Arm Description

The participant has the interventions/study visits occur in a random order: CPET pGz administration through pGz Bed pGz administration through Gentle Jogger

The participant has the interventions/study visits occur in a random order: pGz administration through Gentle Jogger CPET pGz administration through pGz Bed

All participants in Aim 2 will have the interventions/study visits occur in the same order: Exercise Pedal and Gentle Jogger

Outcomes

Primary Outcome Measures

Aim 1: Mean Difference in Maximal Oxygen Consumption between primary mitochondrial disease patients and healthy volunteers
Maximal Oxygen Consumption will be measured only during CPET
Aim 2: Arterial-Venous (A-V) O2 difference
This will be measured through blood draws that occur before and after study interventions
Aim 1 and 2: Oxygen Consumption
Measured During the study interventions

Secondary Outcome Measures

Aim 1 and 2: A/B ratio measurement through EKG or Plethsymography
To measure hemodynamic physiologic marker of cardiac output (CO)
Aim 1 and 2: Heart Rate
To measure hemodynamic physiologic marker of cardiac output (CO)
Aim 1 and 2: OXPHOS Capacity
Measured through a CrCEST Leg MRI, which measures creative levels and recovery in the leg
Aim 1 and 2: Plasma Lactate Levels
Measured through venous blood draws
Aim 1 and 2: Vasodilatation
Measured through a vascular ultrasound with contrast
NO release
Measured through Plethsymography
Plasma Nom Levels
Measured through venous blood draws
Post-operative Patient Satisfaction Survey
Participant tolerance to pGz compared to CPET
Borg Scale
Participant tolerance to pGz compared to CPET

Full Information

First Posted
July 7, 2022
Last Updated
May 18, 2023
Sponsor
Children's Hospital of Philadelphia
Collaborators
United States Department of Defense
search

1. Study Identification

Unique Protocol Identification Number
NCT05569122
Brief Title
Applying pGz in Mitochondrial Disease
Official Title
The Utility of pGz in Primary Mitochondrial Disorders
Study Type
Interventional

2. Study Status

Record Verification Date
May 2023
Overall Recruitment Status
Recruiting
Study Start Date
March 22, 2023 (Actual)
Primary Completion Date
October 2024 (Anticipated)
Study Completion Date
October 2024 (Anticipated)

3. Sponsor/Collaborators

Responsible Party, by Official Title
Sponsor
Name of the Sponsor
Children's Hospital of Philadelphia
Collaborators
United States Department of Defense

4. Oversight

Studies a U.S. FDA-regulated Drug Product
Yes
Studies a U.S. FDA-regulated Device Product
No
Data Monitoring Committee
No

5. Study Description

Brief Summary
This is a multi-aim study, studying the effects of conventional exercise (measured through Cardiopulomary Exercises Testing or an in-bed pedal exercise) and passive exercise through periodic acceleration (pGz). Aim 1 will focus on the differences between primary mitochondrial disease (PMD) patients and healthy volunteers. Aim 2 is an exploratory aim, which will be studying the effects in patients admitted to the Children's Hospital of Philadelphia Pediatric Intensive Care Unit (PICU).
Detailed Description
Aim 1: Primary Mitochondrial Disease Patients and Healthy Controls Individuals will be screened for eligibility for study entry, and answer questions relating to their ability to perform study procedures and their physical activity levels. Individuals who meet study criteria will have 3 study visits, and each study visit will involve a different intervention. At each of these study visits, individuals will complete one of the following interventions: Cardiopulmonary Exercise Testing (CPET), pGz administration through a bed or recliner, and pGz through a device called a Gentle Jogger. While participants will complete all three study visits, the order of the study visits will occur in random order. During the study visits, participants will have blood draws before and after the study intervention, a vascular ultrasound with a Lumason contrast agent before and after the study intervention, and a Creatine Chemical Exchange Saturation Transfer (CrCEST) MRI of the lower leg. Aim 2: Patients in the Pediatric Intensive Care Unit (PICU) Individuals will be screened for eligibility for study entry. Individuals who meet study criteria will have 2 study visits during their admission to the PICU. The first study visit will involve a pedal exercise and the second study visit will involve pGz administration through a device called a Gentle Jogger. During the study visits, participants will have blood draws before and after the study intervention, a vascular ultrasound with a Lumason contrast agent before and after the study intervention, and if able to safely complete, an CrCEST MRI of the lower leg.

6. Conditions and Keywords

Primary Disease or Condition Being Studied in the Trial, or the Focus of the Study
Mitochondrial Myopathies, Mitochondrial Diseases
Keywords
Passive Exercise, periodic acceleration

7. Study Design

Primary Purpose
Diagnostic
Study Phase
Phase 1
Interventional Study Model
Parallel Assignment
Model Description
Aim 1 will have the 3 study interventions/study visits occur in random order. The interventions will be performed in primary mitochondrial disease patients and healthy controls Aim 2 will have all participants complete the study interventions/visits in the same order. Patients will be from the PICU
Masking
None (Open Label)
Allocation
Non-Randomized
Enrollment
90 (Anticipated)

8. Arms, Groups, and Interventions

Arm Title
Aim 1: Primary Mitochondrial Disease Patients
Arm Type
Experimental
Arm Description
The participant has the interventions/study visits occur in a random order: CPET pGz administration through pGz Bed pGz administration through Gentle Jogger
Arm Title
Aim 1: Healthy Controls
Arm Type
Experimental
Arm Description
The participant has the interventions/study visits occur in a random order: pGz administration through Gentle Jogger CPET pGz administration through pGz Bed
Arm Title
Aim 2: PICU Patients
Arm Type
Experimental
Arm Description
All participants in Aim 2 will have the interventions/study visits occur in the same order: Exercise Pedal and Gentle Jogger
Intervention Type
Diagnostic Test
Intervention Name(s)
Cardiopulmonary Exercise Testing
Intervention Description
Testing with an exercise bicycle that is considered "standard of care" for determination of exercise capacity. Participants will complete about 20 minutes of pedaling in a stationary exercise bike
Intervention Type
Device
Intervention Name(s)
pGz Bed
Intervention Description
Participants will lay down on a passive exercise (pGz) bed for 45 minutes during which the bed will administer passive exercise through periodic acceleration
Intervention Type
Device
Intervention Name(s)
Gentle Jogger
Intervention Description
Participants will have passive exercise delivered through the gentle jogger device for 45 minutes. This may be sitting down (aim 1 participants) or laying down (aim 2 participants)
Intervention Type
Device
Intervention Name(s)
Exercise Pedal
Intervention Description
Participants will exercise while laying down for 20 minutes with an exercise pedal that attaches to the bed
Intervention Type
Drug
Intervention Name(s)
Lumason® contrast agent
Intervention Description
Contrast agent used during a vascular ultrasound of the upper leg. Will occur at each study visit twice before and after pGz bed, gentle jogger, exercise pedal or CPET. Drug Administration will be through an IV line and take about 5 - 10 minutes.
Primary Outcome Measure Information:
Title
Aim 1: Mean Difference in Maximal Oxygen Consumption between primary mitochondrial disease patients and healthy volunteers
Description
Maximal Oxygen Consumption will be measured only during CPET
Time Frame
During Cardiopulmonary Exercise Testing, which will last 1 hour
Title
Aim 2: Arterial-Venous (A-V) O2 difference
Description
This will be measured through blood draws that occur before and after study interventions
Time Frame
A total of 4 15 minute blood draws
Title
Aim 1 and 2: Oxygen Consumption
Description
Measured During the study interventions
Time Frame
1 hour per study intervention
Secondary Outcome Measure Information:
Title
Aim 1 and 2: A/B ratio measurement through EKG or Plethsymography
Description
To measure hemodynamic physiologic marker of cardiac output (CO)
Time Frame
1 hour per study intervention
Title
Aim 1 and 2: Heart Rate
Description
To measure hemodynamic physiologic marker of cardiac output (CO)
Time Frame
1 hour per study intervention
Title
Aim 1 and 2: OXPHOS Capacity
Description
Measured through a CrCEST Leg MRI, which measures creative levels and recovery in the leg
Time Frame
Aim 1 subjects will complete 2 1 hour MRIs, Aim 2 Subjects will complete 1 1-hour MRI
Title
Aim 1 and 2: Plasma Lactate Levels
Description
Measured through venous blood draws
Time Frame
15 minute blood draws that occur pre and and immediately after each study intervention
Title
Aim 1 and 2: Vasodilatation
Description
Measured through a vascular ultrasound with contrast
Time Frame
30 minute ultrasound that occurs pre and immediately after each study intervention
Title
NO release
Description
Measured through Plethsymography
Time Frame
15 minutes, before and immediately after each study intervention
Title
Plasma Nom Levels
Description
Measured through venous blood draws
Time Frame
15 minute blood draws that occur pre and immediately after each study intervention
Title
Post-operative Patient Satisfaction Survey
Description
Participant tolerance to pGz compared to CPET
Time Frame
15 minutes, taken after each study intervention
Title
Borg Scale
Description
Participant tolerance to pGz compared to CPET
Time Frame
15 minutes, taken after each study intervention

10. Eligibility

Sex
All
Minimum Age & Unit of Time
10 Years
Maximum Age & Unit of Time
60 Years
Accepts Healthy Volunteers
Accepts Healthy Volunteers
Eligibility Criteria
Aim 1 Enrollment Criteria Inclusion Criteria for Healthy Controls Males or females, 10 years to 60 years, with a minimum height for participation of 135 cm Ambulatory and able to complete routine clinical exercise testing Willing and able to complete all study procedures For individuals under the age of 18, parental/guardian permission (informed consent) and as appropriate, child assent For individuals over the age of 18 the ability to provide informed consent Inclusion Criteria for PMD Patients Males or females, 10 years to 60 years, with a minimum height for participation of 135 cm Ambulatory and able to complete routine clinical exercise testing Willing and able to complete all study procedures Genetically confirmed mitochondrial myopathy (MM) as defined by a diagnosis of primary mitochondrial disease (PMD) with predominant symptoms of myopathy as expressed by exercise intolerance and muscle weakness and fatigue Parental/guardian permission (informed consent) and as appropriate, child assent Exclusion Criteria for All Aim 1 Participants General Exclusion Criteria Tracheostomy Non-ambulatory Unable to complete routine exercise testing Diagnosed with or have symptoms of vertigo Within 1 month of a recent hospital admission for acute illness Severe co-existing cardiac or pulmonary disease Cognitive impairment that may preclude ability to comply with study procedures Pregnant or lactating females Active alcohol and/or substance abuse At the discretion of the principal investigator (PI), any medical condition that will interfere with or prevent the safe completion of the study Parents/guardians or subjects who, in the opinion of the Investigator, may be non-compliant with study schedules or procedures Use of investigational agent(s) within 4 weeks Individuals who are employed by the U.S. Department of Defense, including U.S military personal Patients with biliary atresia with asplenia or polysplenia. Patients with prior liver transplant. Patients with cystic fibrosis. Patients with chronic lung disease. Patients with portal vein thrombosis, cavernous transformation of the portal vein or absent portal vein. Patients with significant heart disease or severe congenital heart disease. Patients with a history of allergic reaction to Lumason®, sulfur hexafluoride, sulfur hexafluoride lipid microsphere components, or other ingredients in Lumason (polyethylene glycol, distearoylphosphatidlycholine (DSPC), dipalmitoylphosphatidylglycerol sodium (DPPG-Na, palmitic acid) or other components of the ultrasound contrast agent Any history of intraocular injury or fragment in or around the orbit that cannot be cleared through radiologic evaluation Any history of bullet, shrapnel, or stabbing wounds that cannot be cleared through radiologic evaluation Past or current employment involving (or exposure to) a metal grinder (e.g., at a construction worksite) Claustrophobia or any known medical conditions which can be exacerbated by stress, anxiety, or panic attacks triggered by enclosed spaces Inability to lie flat in an MRI scanner for up to 45 minutes Unable to perform sub-maximal ankle dorsiflexion leg exercise during the MRI study Aim 2 Enrollment Criteria Inclusion Criteria for PICU PMD Non-Ambulatory Patients Males or females ages 10 to 23 years (children and adults) Non-ambulatory Genetically confirmed mtDNA-PMD Cooperative and capable of following research procedures Have cognitive ability to enable cooperation with study procedures Admitted to the PICU with an anticipated length of stay for >24 hours Willing and able to complete all study procedures For individuals under the age of 18, parental/guardian permission (informed consent) and as appropriate, child assent For individuals over the age of 18 the ability to provide informed consent Inclusion Criteria for PICU non-PMD neuromuscular diagnosis Males or females ages 10 to 23 years (children and adults) Non-ambulatory Genetically confirmed non-PMD neuromuscular diagnosis Cooperative and capable of following research procedures Have cognitive ability to enable cooperation with study procedures Admitted to the PICU with an anticipated length of stay for >24 hours For individuals under the age of 18, parental/guardian permission (informed consent) and as appropriate, child assent For individuals over the age of 18 the ability to provide informed consent Inclusion Criteria for all other PICU Participants Males or females ages 10 to 23 years (children and adults) Non-ambulatory No known genetic diagnosis with healthy pre-morbid status, admitted to PICU Cooperative and capable of following research procedures Have cognitive ability to enable cooperation with study procedures Admitted to the PICU with an anticipated length of stay for >24 hours Willing and able to complete all study procedures For individuals under the age of 18, parental/guardian permission (informed consent) and as appropriate, child assent For individuals over the age of 18 the ability to provide informed consent Exclusion Criteria for All Aim 2 Participants Have cognitive impairment that may preclude ability to comply with study procedures Have cardiorespiratory instability Patients in whom are so sick that they will not be able to cooperate with the study procedures Have clear contraindications to mobilization Have fixed lower limb deformities/contractures that would prohibit lower extremity exercise Pregnant or lactating females Active alcohol and/or substance abuse At the discretion of the principal investigator (PI), any medical condition that will interfere with or prevent the safe completion of the study Use of investigational agent(s) within 4 weeks Individual who are employed by the U.S. Department of Defense, including U.S military personal Patients with biliary atresia with asplenia or polysplenia. Patients with prior liver transplant. Patients with cystic fibrosis. Patients with chronic lung disease. Patients with portal vein thrombosis, cavernous transformation of the portal vein or absent portal vein. Patients with significant heart disease or severe congenital heart disease. Patients with a history of allergic reaction to Lumason®, sulfur hexafluoride, sulfur hexafluoride lipid microsphere components, or other ingredients in Lumason (polyethylene glycol, distearoylphosphatidlycholine (DSPC), dipalmitoylphosphatidylglycerol sodium (DPPG-Na, palmitic acid) or other components of the ultrasound contrast agent Exclusion Criteria Specific to study procedure: CrCEST MRI Scan: Any history of intraocular injury or fragment in or around the orbit that cannot be cleared through radiologic evaluation Any history of bullet, shrapnel, or stabbing wounds that cannot be cleared through radiologic evaluation Past or current employment involving (or exposure to) a metal grinder (e.g., at a construction worksite) Claustrophobia or any known medical conditions which can be exacerbated by stress, anxiety, or panic attacks triggered by enclosed spaces Inability to lie flat in an MRI scanner for up to 45 minutes Unable to perform sub-maximal ankle dorsiflexion leg exercise during the MRI study
Central Contact Person:
First Name & Middle Initial & Last Name or Official Title & Degree
Katelynn Stanley, BS
Phone
215-426-0225
Email
stanleyk2@chop.edu
Overall Study Officials:
First Name & Middle Initial & Last Name & Degree
Zuela Zolkipli-Cunningham, MBChB, MRCP
Organizational Affiliation
Attending Physician
Official's Role
Principal Investigator
Facility Information:
Facility Name
Children's Hospital of Philadelphia
City
Philadelphia
State/Province
Pennsylvania
ZIP/Postal Code
19104
Country
United States
Individual Site Status
Recruiting

12. IPD Sharing Statement

Citations:
PubMed Identifier
33006749
Citation
Sackner MA, Lopez JR, Banderas V, Adams JA. Can Physical Activity While Sedentary Produce Health Benefits? A Single-Arm Randomized Trial. Sports Med Open. 2020 Oct 2;6(1):47. doi: 10.1186/s40798-020-00278-3.
Results Reference
background
PubMed Identifier
30350153
Citation
Sackner MA, Patel S, Adams JA. Changes of blood pressure following initiation of physical inactivity and after external addition of pulses to circulation. Eur J Appl Physiol. 2019 Jan;119(1):201-211. doi: 10.1007/s00421-018-4016-7. Epub 2018 Oct 22.
Results Reference
background
PubMed Identifier
32926876
Citation
Burstein DS, McBride MG, Min J, Paridon AA, Perelman S, Huffman EM, O'Malley S, Del Grosso J, Groepenhoff H, Paridon SM, Brothers JA. Normative Values for Cardiopulmonary Exercise Stress Testing Using Ramp Cycle Ergometry in Children and Adolescents. J Pediatr. 2021 Feb;229:61-69.e5. doi: 10.1016/j.jpeds.2020.09.018. Epub 2020 Sep 11.
Results Reference
background
PubMed Identifier
12538407
Citation
Taivassalo T, Jensen TD, Kennaway N, DiMauro S, Vissing J, Haller RG. The spectrum of exercise tolerance in mitochondrial myopathies: a study of 40 patients. Brain. 2003 Feb;126(Pt 2):413-23. doi: 10.1093/brain/awg028.
Results Reference
background
PubMed Identifier
22239882
Citation
Tarnopolsky M. Exercise testing in metabolic myopathies. Phys Med Rehabil Clin N Am. 2012 Feb;23(1):173-86, xii. doi: 10.1016/j.pmr.2011.11.011. Epub 2011 Dec 11.
Results Reference
background
PubMed Identifier
16120411
Citation
Tarnopolsky M. Exercise testing as a diagnostic entity in mitochondrial myopathies. Mitochondrion. 2004 Sep;4(5-6):529-42. doi: 10.1016/j.mito.2004.07.011. Epub 2004 Sep 30.
Results Reference
background
PubMed Identifier
16331135
Citation
Taivassalo T, Haller RG. Exercise and training in mitochondrial myopathies. Med Sci Sports Exerc. 2005 Dec;37(12):2094-101. doi: 10.1249/01.mss.0000177446.97671.2a.
Results Reference
background
PubMed Identifier
24661939
Citation
Adams JA, Uryash A, Bassuk J, Sackner MA, Kurlansky P. Biological basis of neuroprotection and neurotherapeutic effects of Whole Body Periodic Acceleration (pGz). Med Hypotheses. 2014 Jun;82(6):681-7. doi: 10.1016/j.mehy.2014.02.031. Epub 2014 Mar 12.
Results Reference
background
PubMed Identifier
11588467
Citation
Adams JA, Mangino MJ, Bassuk J, Kurlansky P, Sackner MA. Regional blood flow during periodic acceleration. Crit Care Med. 2001 Oct;29(10):1983-8. doi: 10.1097/00003246-200110000-00022.
Results Reference
background
Citation
M. Fujita et al., "Periodic acceleration enhances release of nitric oxide in healthy adults," Int. J. Angiol., vol. 14, no. 1, pp. 11-14, Feb. 2005, doi: 10.1007/s00547-005-2013-2.
Results Reference
background
PubMed Identifier
26133377
Citation
Uryash A, Bassuk J, Kurlansky P, Altamirano F, Lopez JR, Adams JA. Antioxidant Properties of Whole Body Periodic Acceleration (pGz). PLoS One. 2015 Jul 2;10(7):e0131392. doi: 10.1371/journal.pone.0131392. eCollection 2015.
Results Reference
background
PubMed Identifier
25807532
Citation
Uryash A, Bassuk J, Kurlansky P, Altamirano F, Lopez JR, Adams JA. Non-invasive technology that improves cardiac function after experimental myocardial infarction: Whole Body Periodic Acceleration (pGz). PLoS One. 2015 Mar 25;10(3):e0121069. doi: 10.1371/journal.pone.0121069. eCollection 2015.
Results Reference
background
PubMed Identifier
30402499
Citation
Adams JA, Patel S, Lopez JR, Sackner MA. The Effects of Passive Simulated Jogging on Short-Term Heart Rate Variability in a Heterogeneous Group of Human Subjects. J Sports Med (Hindawi Publ Corp). 2018 Oct 1;2018:4340925. doi: 10.1155/2018/4340925. eCollection 2018.
Results Reference
background
PubMed Identifier
15653959
Citation
Sackner MA, Gummels E, Adams JA. Nitric oxide is released into circulation with whole-body, periodic acceleration. Chest. 2005 Jan;127(1):30-9. doi: 10.1378/chest.127.1.30.
Results Reference
background
PubMed Identifier
15501928
Citation
Adams JA, Bassuk J, Wu D, Grana M, Kurlansky P, Sackner MA. Periodic acceleration: effects on vasoactive, fibrinolytic, and coagulation factors. J Appl Physiol (1985). 2005 Mar;98(3):1083-90. doi: 10.1152/japplphysiol.00662.2004. Epub 2004 Oct 22.
Results Reference
background
PubMed Identifier
32826637
Citation
Betik AC, Parker L, Kaur G, Wadley GD, Keske MA. Whole-Body Vibration Stimulates Microvascular Blood Flow in Skeletal Muscle. Med Sci Sports Exerc. 2021 Feb 1;53(2):375-383. doi: 10.1249/MSS.0000000000002463.
Results Reference
background
PubMed Identifier
21622816
Citation
Sjoberg KA, Rattigan S, Hiscock N, Richter EA, Kiens B. A new method to study changes in microvascular blood volume in muscle and adipose tissue: real-time imaging in humans and rat. Am J Physiol Heart Circ Physiol. 2011 Aug;301(2):H450-8. doi: 10.1152/ajpheart.01174.2010. Epub 2011 May 27.
Results Reference
background
PubMed Identifier
24925857
Citation
Kogan F, Haris M, Debrosse C, Singh A, Nanga RP, Cai K, Hariharan H, Reddy R. In vivo chemical exchange saturation transfer imaging of creatine (CrCEST) in skeletal muscle at 3T. J Magn Reson Imaging. 2014 Sep;40(3):596-602. doi: 10.1002/jmri.24412. Epub 2013 Oct 31.
Results Reference
background
PubMed Identifier
27812541
Citation
DeBrosse C, Nanga RPR, Wilson N, D'Aquilla K, Elliott M, Hariharan H, Yan F, Wade K, Nguyen S, Worsley D, Parris-Skeete C, McCormick E, Xiao R, Cunningham ZZ, Fishbein L, Nathanson KL, Lynch DR, Stallings VA, Yudkoff M, Falk MJ, Reddy R, McCormack SE. Muscle oxidative phosphorylation quantitation using creatine chemical exchange saturation transfer (CrCEST) MRI in mitochondrial disorders. JCI Insight. 2016 Nov 3;1(18):e88207. doi: 10.1172/jci.insight.88207.
Results Reference
background
PubMed Identifier
16815877
Citation
Jeppesen TD, Schwartz M, Olsen DB, Wibrand F, Krag T, Duno M, Hauerslev S, Vissing J. Aerobic training is safe and improves exercise capacity in patients with mitochondrial myopathy. Brain. 2006 Dec;129(Pt 12):3402-12. doi: 10.1093/brain/awl149. Epub 2006 Jun 30.
Results Reference
background

Learn more about this trial

Applying pGz in Mitochondrial Disease

We'll reach out to this number within 24 hrs