Status | Study |
Recruiting |
Study Name: Fat and Sugar Metabolism During Exercise in Patients With Metabolic Myopathy Condition: Metabolism, Inborn Errors Lipid Metabolism, Inborn Errors Date: 2015-12-09 Interventions: Other: Sugar 10 minutes before exercising, the subjects are given a bolus of glucose solution 0.2 g/kg i |
Active, not recruiting |
Study Name: A Study of the Safety, Efficacy and Pharmacokinetics of Glycerol Phenylbutyrate in Pediatric Subjects Under 2 Years of Age With Urea Cycle Disorders (UCDs) Condition: Urea Cycle Disorder Date: 2014-09-17 Interventions: Drug: RAVICTI Other Names: HPN-100 Glycerol Phenylbutyrate |
Terminated |
Study Name: Ammonia Levels and Cognitive Impairment Condition: Dementia Cognitive Decline Cognitive Impairment Date: 2014-01-06 |
Completed |
Study Name: Phase 2B Efficacy/Safety of Ornithine Phenylacetate in Hospitalized Cirrhotic Patients With Hepatic Encephalopathy (STOP-HE) Condition: Acute Episode of Overt Hepatic Encephalopathy Hepatic Encephalopathy Date: 2013-10-11 Interventions: Drug: ornithine phenylacetate |
Completed |
Study Name: Fat and Sugar Metabolism During Exercise, With and Without L-carnitine in Patients With Carnitine Transporter Deficiency Condition: Carnitine Transporter, Plasma-membrane, Deficiency of Date: 2013-08-05 Interventions: Dietary Supplement: Break in L-carnitine treatment Levocarnitine oral tablet supplement at individual do |
Completed |
Study Name: Pilot Study For Hypothermia Treatment In Hyperammonemic Encephalopathy In Neonates And Very Young Infants Condition: Urea Cycle Disorders Organic Acidemias Date: 2012-06-13 Interventions: Other: Therapeutic Hypothermia |
Recruiting |
Study Name: Short-term Outcome of N-Carbamylglutamate in the Treatment of Acute Hyperammonemia Condition: Propionic Acidemia, Type I and/or Type II Methylmalonic Acidemia Date: 2012-05-11 Interventions: Drug: Carbaglu |
Completed |
Study Name: Investigation of Brain Nitrogen in Partial Ornithine Transcarbamylase Deficiency (OTCD) Using 1 H MRS, DTI, and fMRI Condition: Ornithine Transcarbamylase Deficiency Date: 2012-03-30 Interventions: Other: MRI scanning 1H MRS, DT |
Completed |
Study Name: Study of the Safety, Pharmacokinetics and Efficacy of HPN-100, in Pediatric Subjects With Urea Cycle Disorders (UCDs) Condition: Urea Cycle Disorders Date: 2011-04-29 Interventions: Drug: HPN-100 HPN-100 is a pro-drug of PAA that combines with glutamine to provide an alternative vehicl |
Completed |
Study Name: To Evaluate the Safety of Long-term Use of HPN-100 in the Management of Urea Cycle Disorders (UCDs) Condition: Urea Cycle Disorders Date: 2010-12-02 Interventions: Drug: HPN-100 HPN-100 will be administered orally three times daily (TID) with meals. The maximum recomm |