Elevated Urinary Glutarylglycine Level C5936984
An organic acidemia where the body cannot properly break down certain amino acids, leading to acid buildup. It is usually detected in infancy. Treatment focuses on diet changes and supplements to prevent neurological problems.
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.
Warning signs - seek immediate care
Metabolic acidosis
Fatigue or weight changes
(metabolic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Developmental delay
(metabolic)Failure to thrive
(metabolic)Fatigue
(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
Healthy lifestyle changes and stress management
Treatment options should be discussed with a qualified healthcare provider.
Lysine-Restricted Diet
Specialized diet limiting lysine and tryptophan intake, which are precursors to glutaric acid accumulation. Involves medical foods and careful meal planning to reduce toxic metabolite production.
L-Carnitine Supplementation
Oral or intravenous carnitine to help conjugate glutaric acid and facilitate its excretion, reducing toxic accumulation in tissues and fluids.
Additional treatment options
Metabolic Monitoring
Regular laboratory assessment of urinary glutaric acid levels, plasma carnitine, and liver function to guide treatment adjustments and prevent metabolic decompensation.
monitoringEmergency Metabolic Protocol
Structured plan for illness or stress periods including increased caloric intake, IV glucose, and intensified carnitine to prevent catabolism-induced crises.
lifestyleDialysis for Acute Crises
Temporary dialysis or hemofiltration to rapidly remove accumulated glutaric acid during severe metabolic decompensation when medical management fails.
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 Glutarylglycine Level C5936984?
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.
Hoffmann GF, Kölker S. Guideline for diagnosis and management of organic acidemias. J Inherit Metab Dis. 2021.
Schulze-Bergkamen A, Okun JG. Metabolic disorders with elevated glutaric acid and glutarylglycine. Mol Genet Metab. 2018.
Nji AM, Mamas S. Long-term outcome of early-treated glutaric acidemia type 1 patients. Orphanet J Rare Dis. 2019.
Margoni M, Dionisi-Vici C. Newborn screening for organic acidemias. Curr Opin Pediatr. 2022.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy