Applying pGz in Mitochondrial Disease

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
• •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
• •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