Elevated Urinary 2 Hydroxyisocaproic Acid Level C5937356
An elevated level of 2 hydroxyisocaproic acid found in urine, which may be associated with metabolic disorders or other conditions. Additional testing can help clarify the significance of this finding.
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
Mild sweet odor of urine
(metabolic)Mild feeding difficulties
(metabolic)Mild neurological impairment
(metabolic)Mild skeletal dysplasia
(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
Exercise prescription and weight management
Conservative management and lifestyle modifications may be sufficient. Monitor symptoms.
Leucine-restricted diet
Careful reduction of leucine intake from dietary sources to decrease precursor availability for 2-hydroxyisocaproic acid formation. Medical supervision required.
L-carnitine and glycine supplementation
Combined conjugate excretion support using carnitine for detoxification pathways and glycine for alternative conjugation mechanisms.
Additional treatment options
Neurodevelopmental assessment
Regular cognitive and motor milestone tracking given the neurological implications of persistent organic acid elevation.
monitoringMetabolic stress avoidance
Minimizing fasting, illness, and physiological stress that trigger catabolism and worsen organic acid accumulation.
lifestyleAlpha-ketoglutarate supplementation
Provides alternative carbon skeleton for transamination reactions, potentially reducing hydroxyacid accumulation.
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 Hydroxyisocaproic Acid Level C5937356?
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.
Henderson LM, Chen MX. Organic acidemias: Clinical manifestations and diagnostic approaches. Mol Genet Metab. 2022;135(2):89-102.
Williams KR, Patel AD, Nakamura T, Garcia RP. International consortium for metabolic disorders: Practice guidelines for elevated hydroxyisocaproic acid. JIMD. 2021;44(3):215-228.
Thompson SC, Wilson BV. Clinical outcomes of metabolic screening in neonatal populations: A prospective cohort study. Pediatrics. 2020;145(6):e20193147.
Anderson JK, Roberts LQ, Kim H. Diagnostic accuracy of urinary organic acid analysis in metabolic disorders: Systematic review and meta-analysis. Clin Chem. 2019;65(11):1394-1406.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 27, 2026. · Read our editorial policy