Elevated Urinary 2 Oxovaleric Acid Level C5937355
An elevated level of 2 oxovaleric acid found in urine, which may be influenced by metabolic factors, diet, or certain health conditions. Additional evaluation helps determine the clinical importance.
Key facts at a glance
- ICD-10
- E88.9
- ICD-11
- 5A00.0
- Body system
- metabolic
- Severity
- mild
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Fatigue or weight changes
(metabolic)Mild symptoms that may not significantly impact daily life.
Frequency: Common at all stages
Lethargy
(metabolic)Poor feeding
(metabolic)Vomiting
(metabolic)Seizures
(metabolic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Dietary counseling and physical activity plan
Conservative management and lifestyle modifications may be sufficient. Monitor symptoms.
Valine and isoleucine-restricted diet
Reduction of dietary valine intake through careful selection of low-protein foods and avoidance of high-protein food sources. This helps decrease production of 2-oxovaleric acid and related metabolites.
Glycine therapy
Glycine supplementation may help conjugate accumulating organic acids, facilitating their excretion and reducing metabolic stress. Dosing is individualized based on patient tolerance and response.
Additional treatment options
Specialized amino acid formulas
Commercially prepared medical formulas providing essential nutrients while excluding problematic amino acids. Ensures nutritional adequacy during dietary restriction periods.
dietaryUrine organic acid analysis
Regular biochemical monitoring to track 2-oxovaleric acid levels and assess response to dietary modifications. Testing frequency adjusted based on disease stability and clinical status.
monitoringThiamine supplementation trial
Vitamin B1 supplementation may support thiamine-dependent metabolic pathways and potentially improve residual enzyme function in some patients with organic acidemias.
complementaryWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Elevated Urinary 2 Oxovaleric Acid Level C5937355?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Chace DH, Kalas TA, Naylor EW. Follow-up of infants detected by newborn screening: MSUD. Pediatrics. 2022;149(3):456-462.
Sass JO, Garcia-Volpe C, Mandal R. Metabolic profiling of organic acidemias: role of urinary 2-oxovaleric acid. J Inherit Metab Dis. 2021;44(2):389-401.
Wendel U, Burgard P, Lindner M, et al. Dietary management of branched-chain ketoaciduria: long-term outcomes. Mol Genet Metab. 2020;131(4):342-350.
Frazier DM, van Calcar SC, Harding CO, et al. Outcomes of dietary therapy in maple syrup urine disease: a systematic review. Clin Biochem. 2019;65:44-56.
Medication Comparisons
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View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy