Clinical trials: Fatty Acid Oxidation Disorder
Long-chain Fatty Acid Oxidation Disorders (LC-FAOD)
Clinical Outcome of Triheptanoin Treatment in Patients With Long-chain Fatty Acid Oxidation Disorders (LC-FAOD) Treated Under Expanded Access Program
The primary objective of the study is to evaluate the clinical outcomes for the trigger event of patients who receive triheptanoin in the emergency Investigational New Drug (eIND) program.
- Trial name
- NCT03768817
- Testing
- No Intervention
- Where
- 15 sites in United States
- Volunteers
- 20 people
- Sponsor
- Ultragenyx Pharmaceutical Inc
Diabetes Mellitus, Type 2, Overweight
Mitochondrial Dysfunction in Type 2 Diabetes Mellitus and Capacity for Fat Oxidation During Exercise
The purpose of this study is to compare the effects of an 8-week aerobic training program upon fat oxidation in vivo and markers of skeletal muscle mitochondrial content and oxidative capacity in sedentary obese subjects with and…
- Trial name
- NCT00382473
- Testing
- Aerobic exercise program - 8 weeks
- Where
- United States
- Volunteers
- 29 people, healthy volunteers welcome
- Sponsor
- University of Pittsburgh
Very Long-chain Acyl-CoA Dehydrogenase Deficiency, Trifunctional Protein Deficiency
High Protein Diet in Patients With Long-chain Fatty Acid Oxidation Disorders
The study also determines if eating a diet higher in protein alters body composition, energy balance and metabolic control among patients with a long-chain fatty acid oxidation disorder.
- Conditions
- Very Long-chain Acyl-CoA Dehydrogenase Deficiency, Trifunctional Protein Deficiency, Carnitine Palmitoyltransferase 2 Deficiency, Long-chain 3-hydroxyacyl-CoA Dehydrogenase Deficiency
- Trial name
- NCT01494051
- Testing
- Diet counseling
- Volunteers
- 13 people
- Sponsor
- Oregon Health and Science University
Metabolic Syndrome, Obesity
Genistein Stimulates Insulin Sensitivity Through Gut Microbiota
There is evidence that genistein present in soy can improve insulin resistance in rodents and humans with metabolic syndrome (MetS). However, it is not known if this improvement is associated with changes in the gut microbiota.
- Trial name
- GENISTEIN (NCT04105023)
- Testing
- genistein
- Volunteers
- 45 people, healthy volunteers welcome
- Sponsor
- Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran
Carnitine Palmitoyltransferase II Deficiency, Very Long Chain Acyl Coa Dehydrogenase Deficiency
Effect of Bezafibrate on Muscle Metabolism in Patients With Fatty Acid Oxidation Defects
The investigators propose to evaluate the effect of bezafibrate on metabolism during exercise in 22 adult patients affected with carnitine palmitoyltransferase II (CPTII) or very-long chain acyl-CoA-dehydrogenase (VLCAD)…
- Trial name
- Bezafibrate (NCT00983788)
- Testing
- Bezafibrate
- Where
- Denmark
- Volunteers
- 12 people
- Sponsor
- Copenhagen University Hospital, Rigshospitalet
Very Long-chain Acyl-CoA Dehydrogenase Deficiency, Trifunctional Protein Deficiency
Fatty Acid Oxidation Defects and Insulin Sensitivity
The purpose of this study is to learn more about what causes insulin resistance. It has been suggested that proper breakdown of fat into energy (oxidation) in the body is important to allow insulin to keep blood sugar in the…
- Conditions
- Very Long-chain Acyl-CoA Dehydrogenase Deficiency, Trifunctional Protein Deficiency, Long-chain 3-hydroxyacyl-CoA Dehydrogenase Deficiency, Medium-chain Acyl-CoA Dehydrogenase Deficiency
- Trial name
- NCT02517307
- Testing
- Intralipid/Heparin, Glycerol/Saline, Hyperinsulinemic euglycemic clamp
- Where
- United States
- Volunteers
- 41 people, healthy volunteers welcome
- Sponsor
- Oregon Health and Science University
Long-chain 3-hydroxyacyl-CoA Dehydrogenase Deficiency, Carnitine Palmitoyltransferase Deficiency 2
Oral Ketones and Exercise Among Patients With Long-chain Fatty Acid Oxidation Disorders
The purpose of the study is to determine if an oral ketone beverage is safe and well-tolerated during moderate intensity exercise in participants with long-chain fatty acid oxidation disorders and if it will raise blood ketones…
- Conditions
- Long-chain 3-hydroxyacyl-CoA Dehydrogenase Deficiency, Carnitine Palmitoyltransferase Deficiency 2, Very Long Chain Acyl Coa Dehydrogenase Deficiency, Trifunctional Protein Deficiency
- Trial name
- NCT05411835
- Testing
- Nutritional Ketone Supplement
- Where
- United States
- Volunteers
- 5 people
- Sponsor
- Oregon Health and Science University
Urea Cycle Disorder, Organic Acidemia
Systemic Biomarkers of Brain Injury From Hyperammonemia
Ammonia is a waste product of protein and amino acid catabolism and is also a potent neurotoxin. High blood ammonia levels on the brain can manifest as cytotoxic brain edema and vascular compromise leading to intellectual and…
- Conditions
- Urea Cycle Disorder, Organic Acidemia, Maple Syrup Urine Disease, Glutaric Acidemia I
- Trial name
- NCT04602325
- Where
- United States
- Volunteers
- 24 people
- Sponsor
- Children's National Research Institute
Acute Liver Failure, Mitochondrial Diseases
Longitudinal Study of Mitochondrial Hepatopathies
The specific aims of this study are (1) to determine the clinical phenotypes and natural history of hepatic RC and FAO disorders, (2) to determine the correlation between genotype and phenotype, (3) to determine if circulating…
- Conditions
- Acute Liver Failure, Mitochondrial Diseases, End Stage Liver Disease, Respiratory Chain Deficiencies, Mitochondrial
- Trial name
- MITOHEP (NCT01148550)
- Where
- 16 sites in United States, Canada
- Volunteers
- 90 people
- Sponsor
- Arbor Research Collaborative for Health
Inborn Errors of Metabolism
Nutritional Therapy of the Deficits of Oxidation Mitochondrial of the Fatty Acids
Usual dietary therapies of mitochondrial fatty acid oxidation disorders (FAO) are based on 3 strategies: limitation of lipid intake in the diet; supplementation of the diet with medium-chain triglycerides (MCT) for patients…
- Trial name
- NCT00328159
- Testing
- Oil special 107 and MYGLIOL 810
- Where
- France
- Volunteers
- 20 people
- Sponsor
- Assistance Publique - Hôpitaux de Paris
Urea Cycle Disorders, Organic Acidemias
At Home Ammonia Monitoring of Inborn Errors of Ammonia Metabolism
The goal of this observational study is to learn if people with certain ammonia metabolism disorders will measure their ammonia levels at home.
- Conditions
- Urea Cycle Disorders, Organic Acidemias, Fatty Acid Oxidation Disorder, Ammonia; Metabolic Disorder
- Trial name
- NCT06953505
- Testing
- Ammonia Study Device
- Where
- United States
- Volunteers
- 30 people
- Sponsor
- Sequitur Health Corp.
Obesity
A Study to Test Whether Survodutide Improves How the Body Uses Energy and Breaks Down Fat in People With Obesity
This study is open to adults between 18 and 65 years of age who have obesity. People can join the study if they have a body mass index (BMI) between 30 and 45 km/m².
- Trial name
- NCT06745284
- Testing
- Survodutide, Semaglutide
- Where
- 2 sites in United States
- Volunteers
- 64 people
- Sponsor
- Boehringer Ingelheim
Metabolic Syndrome X, Insulin Resistance
Physical Inactivity and Insulin Resistance in Skeletal Muscle.
The purpose of this study is to determine how a decline in physical activity acutely leads to a decrease in insulin sensitivity in skeletal muscle.
- Conditions
- Metabolic Syndrome X, Insulin Resistance, Hypertension, Hypercholesterolemia
- Trial name
- NCT00536211
- Testing
- Exercise, Metformin
- Where
- United States
- Volunteers
- healthy volunteers welcome
- Sponsor
- University of Kansas Medical Center
Tarui Disease, Debrancher Deficiency
The Effect of Triheptanoin on Fatty Acid Oxidation and Exercise Tolerance in Patients With Glycogenoses
The aim of this study is to investigate the effect of 14 days of treatment with the dietary oil-supplement Triheptanoin on fat metabolism and exercise tolerance in patients with Phosphofructokinase deficiency, Debrancher…
- Conditions
- Tarui Disease, Debrancher Deficiency, GYG1 DEFICIENCY
- Trial name
- NCT03642860
- Testing
- Triheptanoin
- Where
- Denmark
- Volunteers
- 3 people
- Sponsor
- Copenhagen University Hospital, Rigshospitalet
Metabolic Syndrome X, Prediabetic State
Effects of PPAR Ligands on Ectopic Fat Accumulation and Inflammation
The relationship between obesity and insulin resistance is known, however the mechanism(s) associating obesity with insulin resistance is not well understood.
- Trial name
- NCT00470262
- Testing
- Fenofibrate 145mg PO QD, Pioglitazone 45 mg PO QD
- Where
- United States
- Volunteers
- 27 people, healthy volunteers welcome
- Sponsor
- VA Office of Research and Development
Long-chain Fatty Acid Oxidation Disorders (LC-FAOD)
Long-Chain Fatty Acid Oxidation Disorders In-Clinic Disease Monitoring Program
The primary objective of this study is to assess the long-term safety, including pregnancy, infant, and lactation outcomes, of patients with LC-FAOD who are enrolled in the DMP.
- Trial name
- NCT04632953
- Testing
- No Intervention
- Where
- 16 sites in United States, Canada
- Volunteers
- 150 people
- Sponsor
- Ultragenyx Pharmaceutical Inc
Trifunctional Protein Deficiency
Fatty Acid Oxidation Disorders & Body Weight Regulation Grant
Several hormones involved in body weight regulation increase the subject's ability to burn fat for energy. The purpose of this study is to investigate how burning fat for energy may affect those hormones and body weight in…
- Trial name
- NCT00654004
- Where
- United States
- Volunteers
- 26 people, healthy volunteers welcome
- Sponsor
- Oregon Health and Science University
Obesity, Overweight
Effect of Caloric Restriction on Fat Oxidation in Obese Men and Women (Magellan II)
The purpose of this study is to better understand the different ways our bodies burn fat which may be important for obesity, diabetes, and cardiovascular disease.
- Conditions
- Obesity, Overweight, Metabolic Diseases
- Trial name
- MagellanII (NCT01616082)
- Testing
- Low Calorie Diet (LCD), Phentermine
- Where
- United States
- Volunteers
- 39 people
- Sponsor
- AdventHealth Translational Research Institute
Other Fatty Acid Oxidation Disorders, Diabetes Mellitus
Fat Induced Insulin Resistance and Atherosclerosis
The overall objective of the current proposal is to strengthen the putative link between FFA induced insulin resistance and atherosclerotic vascular disease (ASVD).
- Conditions
- Other Fatty Acid Oxidation Disorders, Diabetes Mellitus, Insulin Resistance
- Trial name
- NCT02348190
- Testing
- Lipid/heparin
- Where
- United States
- Volunteers
- 86 people, healthy volunteers welcome
- Sponsor
- Temple University
Pulmonary Artery Hypertension
Trimetazidine in Pulmonary Artery Hypertension
Pulmonary artery hypertension (PAH) is a chronic and progressive disease that affects 15 persons per million. Although current therapy has improve disease prognosis, PAH still has a poor survival, with a median survival of 2.8…
- Trial name
- NCT02102672
- Testing
- Trimetazidine
- Where
- Chile
- Volunteers
- 25 people
- Sponsor
- Pontificia Universidad Catolica de Chile