Elevated Urine 3 Hydroxyglutaric Level C5826565
An elevated biochemical marker associated with certain metabolic disorders affecting how the body processes amino acids. Requires specialist evaluation.
Key facts at a glance
- ICD-10
- E88.9
- ICD-11
- 5A00.0
- Body system
- metabolic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Seizures
Fatigue or weight changes
(metabolic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Macrocephaly
(neurological)Dystonia
(neurological)Spasticity
(neurological)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
Lysine-Restricted Diet
Limit dietary lysine intake, which converts to the neurotoxic metabolite 3-hydroxyglutaric acid, through low-protein foods and specialized metabolic formulas.
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
Riboflavin Supplementation
High-dose riboflavin (vitamin B2) supplementation to support improved function of the deficient glutaryl-CoA dehydrogenase enzyme.
Additional treatment options
Urine Organic Acid Analysis
Regular monitoring of 3-hydroxyglutaric acid levels in urine to evaluate treatment efficacy and guide dietary modifications.
monitoringNeurodevelopmental Support Therapy
Early intervention physical and occupational therapy to address movement disorders, hypotonia, and motor skill delays associated with the condition.
physical_therapyEmergency Management Protocol
Establish clear protocols for metabolic crisis prevention during illness, fasting, or stress, including early hospital contact and IV glucose administration.
lifestyleWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Elevated Urine 3 Hydroxyglutaric Level C5826565?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
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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