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Very long chain acyl-CoA dehydrogenase deficiency

VCLAD deficiencyACADVL
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Clinical Trials

This information is provided by ClinicalTrials.gov

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Displaying 1-10 of 11 results.
Effect of Bezafibrate on Muscle Metabolism in Patients With Fatty Acid Oxidation Defects
Status: Completed
Last Changed: May 30, 2012
First Received: Sep 24, 2009
Disease(s): Carnitine Palmitoyltransferase II Deficiency, Very Long Chain Acyl Coa Dehydrogenase Deficiency
Intervention(s): Bezafibrate, Placebo
Locations: Neuromusculare Research Unit, Rigshospitalet, Copenhagen, Denmark
Acute Nutritional Ketosis in VLCAD Deficiency
Status: Completed
Last Changed: May 21, 2018
First Received: May 21, 2018
Disease(s): VLCAD Deficiency, Fatty Acid Oxidation Defects
Intervention(s): ketone ester drink, exercise, muscle biopsy, Magnetic Resonance Imaging
Locations: Academic Medical Center, Amsterdam, Noord-Holland, Netherlands
Dept of Neuroscience/ Neuroimaging Center, Groningen, Netherlands
High Protein Diet in Patients With Long-chain Fatty Acid Oxidation Disorders
Status: Completed
Last Changed: Dec 16, 2011
First Received: Dec 16, 2011
Disease(s): Very Long-chain Acyl-CoA Dehydrogenase Deficiency, Trifunctional Protein Deficiency, Carnitine Palmitoyltransferase 2 Deficiency, Long-chain 3-hydroxyacyl-CoA Dehydrogenase Deficiency
Intervention(s): Diet counseling
Fatty Acid Oxidation Defects and Insulin Sensitivity
Status: Recruiting
Last Changed: Jul 17, 2019
First Received: Aug 07, 2015
Disease(s): Very Long-chain Acyl-CoA Dehydrogenase Deficiency, Trifunctional Protein Deficiency, Long-chain 3-hydroxyacyl-CoA Dehydrogenase Deficiency, Medium-chain Acyl-CoA Dehydrogenase Deficiency, Normal Volunteers, Carnitine Palmitoyltransferase II Deficiency, Myopathic
Intervention(s): Intralipid/Heparin, Glycerol/Saline, Hyperinsulinemic euglycemic clamp
Locations: Oregon Health & Science University, Portland, Oregon, United States
Assessing Immune Function in Young Patients With Cytopenia That Did Not Respond to Treatment
Status: Completed
Last Changed: Jan 16, 2015
First Received: Jul 11, 2007
Disease(s): Dyskeratosis Congenita, Fanconi Anemia, Myelodysplastic Syndromes, Pearson Marrow-pancreas Syndrome, Shwachman-diamond Syndrome
Intervention(s): polymerase chain reaction, flow cytometry, immunologic technique, biopsy
Locations: St. Anna Children's Hospital, Vienna, Austria
Ghent University, Ghent, Belgium
University Hospital Motol, Prague, Czech Republic
Arhus Universitetshospital - Skejby, Aarhus, Denmark
Universitaetskinderklinik - Universitaetsklinikum Freiburg, Freiburg, Germany
... and 6 other locations.
Fat and Sugar Metabolism During Exercise in Patients With Metabolic Myopathy
Status: Active, not recruiting
Last Changed: Oct 16, 2019
First Received: Dec 18, 2015
Disease(s): Metabolism, Inborn Errors, Lipid Metabolism, Inborn Errors, Carbohydrate Metabolism, Inborn Errors, Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency, Glycogenin-1 Deficiency (Glycogen Storage Disease Type XV), Carnitine Palmitoyl Transferase 2 Deficiency, VLCAD Deficiency, Medium-chain Acyl-CoA Dehydrogenase Deficiency, Multiple Acyl-CoA Dehydrogenase Deficiency, Carnitine Transporter Deficiency, Neutral Lipid Storage Disease, Glycogen Storage Disease Type II, Glycogen Storage Disease Type III, Glycogen Storage Disease Type IV, Glycogen Storage Disease Type V, Muscle Phosphofructokinase Deficiency, Phosphoglucomutase 1 Deficiency, Phosphoglycerate Mutase Deficiency, Phosphoglycerate Kinase Deficiency, Phosphorylase Kinase Deficiency, Beta Enolase Deficiency, Lactate Dehydrogenase Deficiency, Glycogen Synthase Deficiency
Intervention(s): Sugar
Locations: Neuromuscular Research Unit, 3342, Copenhagen, Denmark
Study of Triheptanoin for Treatment of Long-Chain Fatty Acid Oxidation Disorder
Status: Completed
Last Changed: Mar 23, 2017
First Received: Jun 23, 2011
Disease(s): Very Long-chain acylCoA Dehydrogenase (VLCAD) Deficiency, Carnitine Palmitoyltransferase 2 (CPT2) Deficiency, Mitochondrial Trifunctional Protein (TFP) Deficiency, Long-chain 3 hydroxyacylCoA Dehydrogenase (LCHAD) Deficiency
Intervention(s): Triheptanoin
Locations: Oregon Health & Science University, Portland, Oregon, United States
University of Pittsburgh, Pittsburgh, Pennsylvania, United States
Expanded Access to Triheptanoin
Status: Available
Last Changed: Feb 13, 2020
First Received: Dec 12, 2018
Disease(s): Long-chain Fatty Acid Oxidation Disorders (LC-FAOD)
Intervention(s): Triheptanoin
Compassionate Use of Triheptanoin (C7) for Inherited Disorders of Energy Metabolism
Status: Available
Last Changed: Dec 16, 2019
First Received: Oct 28, 2011
Disease(s): Very Long-chain acylCoA Dehydrogenase (VLCAD) Deficiency, Carnitine Palmitoyltransferase Deficiencies (CPT1, CPT2), Mitochondrial Trifunctional Protein Deficiency, Long-chain Hydroxyacyl-CoA Dehydrogenase Deficiency, Glycogen Storage Disorders, Pyruvate Carboxylase Deficiency Disease, ACYL-CoA DEHYDROGENASE FAMILY, MEMBER 9, DEFICIENCY of, Barth Syndrome
Intervention(s): triheptanoin
Locations: University of Pittsburgh Division of Medical Genetics, Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania, United States
Long-Chain Fatty Acid Oxidation Disorders (LC-FAOD) Extension Study for Subjects Previously Enrolled in Triheptanoin Studies.
Status: Enrolling by invitation
Last Changed: Mar 04, 2020
First Received: Aug 12, 2014
Disease(s): Carnitine Palmitoyltransferase (CPT I or CPT II) Deficiency, Very Long Chain Acyl-CoA Dehydrogenase (VLCAD) Deficiency, Long-chain 3-hydroxy-acyl-CoA Dehydrogenase (LCHAD) Deficiency, Trifunctional Protein (TFP) Deficiency, Carnitine-acylcarnitine Translocase (CACT) Deficiency
Intervention(s): UX007
Locations: University of California San Francisco, San Francisco, California, United States
Children's National Health System, Washington, District of Columbia, United States
University of South Florida, Tampa, Florida, United States
Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois, United States
Boston Children's Hospital, Boston, Massachusetts, United States
... and 7 other locations.