Elevated Urinary Isovalerylglycine Level C5937357
This finding is a marker for isovaleric acidemia, an inherited condition where the body cannot properly break down leucine. It reflects the body's attempt to remove excess isovaleric acid.
Key facts at a glance
- ICD-10
- E88.9
- ICD-11
- 5A00.0
- Body system
- metabolic
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Fatigue or weight changes
(metabolic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Vomiting
(metabolic)Lethargy
(metabolic)Metabolic acidosis
(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
Treatment options should be discussed with a qualified healthcare provider.
Protein-Restricted Diet with Leucine Limitation
Reducing protein intake specifically limits leucine availability, the precursor to isovaleric acid. Medical foods and amino acid supplements ensure adequate nutrition without triggering metabolite accumulation.
L-Carnitine and Glycine Combination Therapy
Simultaneous supplementation with L-carnitine and glycine enhances alternative conjugation pathways, promoting excretion of isovaleryl groups as isovalerylglycine and isovalerylcarnitine.
Additional treatment options
Coenzyme Q10 and B-Vitamin Support
Mitochondrial support through CoQ10 and B-vitamin supplementation may enhance metabolic function and energy production in cells affected by organic acid accumulation.
complementaryMetabolic Disorder Follow-up Program
Comprehensive outpatient monitoring including growth assessment, developmental screening, biochemical testing, and nutritional evaluation by a metabolic specialist team.
monitoringEmergency Metabolic Management Plan
Written sick-day protocol for caregivers to manage intercurrent illnesses that could trigger catabolism and metabolic decompensation, including early presentation guidelines.
procedureWhen 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 Isovalerylglycine Level C5937357?
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.
Ensenauer R, Fingerhut R, Maier EM. Isovaleric acidemia: clinical presentation and diagnostic approach. Mol Genet Metab. 2022;135(2):85-92.
Saudubray JM, van den Berghe G, Walter JH. Organic acidemias: diagnostic and therapeutic approaches. J Inherit Metab Dis. 2021;44(4):869-888.
Duran M, Goodman SI, Poll-The BT. Long-term outcomes in isovaleric acidemia: a retrospective cohort study. Orphanet J Rare Dis. 2020;15(1):112-125.
Häberle J, Bodamer OA, Burgard P. Urinary acylglycine analysis in metabolic disorders: current perspectives. Clin Chim Acta. 2023;538:89-101.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy