Systemic Biomarkers of Brain Injury From Hyperammonemia

Inclusion Criteria:

1. Inherited Hyperammonemias:

   1. A clinical diagnosis of 1 of 7 diagnosed urea cycle disorders:

      * N-acetylglutamate Synthetase Deficiency (NAGS)
      * Carbamyl Phosphate Synthetase Deficiency (CPSD)
      * Ornithine Transcarbamylase Deficiency (OTCD)
      * Argininosuccinate Synthetase Deficiency (ASD)
      * Argininosuccinate Lyase Deficiency (ALD)
      * Arginase Deficiency (AD)
      * Hyperammonemia-Hyperornithinemia-Homocitrullinuria (HHH)
   2. A clinical diagnosis of 1 of 2 organic acidemias:

      * Propionic Acidemia (PA)
      * Methylmalonic Acidemia (MMA)
2. Acute metabolic disorder without hyperammonemia, with neurological sequelae

   1. Maple Syrup Urine Disease (MSUD)
   2. Glutaric Acidemia (GA1)
3. Acute metabolic disorder without hyperammonemia and without neurological sequelae

   * Fatty Acid Oxidation Disorders:
   * Medium Chain-Acyl CoA Dehydrogenase Deficiency
   * Very Long Chain-Acyl CoA Dehydrogenase Deficiency
   * Trifunctional Protein Deficiency
   * Long Chain Hydroxyacyl-CoA Dehydrogenase Deficiency
   * Carnitine Palmitoyltransferase I or II Deficiency
   * Carnitine/Acylcarnitine Translocase Deficiency
   * Primary Carnitine Transport Deficiency
4. Hypoxic-Ischemic Encephalopathy

Exclusion Criteria:

* Prior Solid-Organ Transplant
* Use of any other investigational drug, biologic, or therapy or any clinical or laboratory abnormality or medical condition that, as determined by the investigator, may interfere with or obscure the biomarker measurements