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Hexanoylglycine in Urine (Mass Concentration)

Published: August 9, 2026Updated: August 16, 2026

This test measures the presence of hexanoylglycine, a breakdown product of certain amino acids, in your urine. It may be used to assess metabolic or genetic conditions affecting amino acid metabolism.

Quick summary

["This test helps evaluate how your body processes certain amino acids, potentially linked to inherited metabolic disorders."]

Medically reviewed by WellAlly Editorial TeamLast reviewed August 16, 2026Editorial policy

Key facts at a glance

LOINC
110431-4
Short code
Hexanoylgly/Creat Msmt Ur
Synonyms
Hexanoylgly/Creat Msmt Ur
Category
glucose
Sample
urine
Container
Sterile urine container
Units
N/A
Fasting
false
Population
adult
Sex
all
Age range
18–65 years
Specimen volume
10 mL
Stability
Room temperature: 2h, Refrigerated: 24h, Frozen: 7d
Hemolysis max
0
Lipemia max
0
Icterus max
0
TAT
4–24 hours
Order
routine

Why This Test Might Be Ordered

Your healthcare provider may recommend this test if you have symptoms like fatigue, muscle weakness, or unexplained health issues that could be related to amino acid metabolism. It may also be part of a broader evaluation for conditions like maple syrup urine disease or other inborn errors of metabolism.

What to Expect Before the Test

No special preparation is typically required. You may be asked to collect a urine sample in the morning or over a specified time period, depending on the test protocol. Always follow your healthcare provider’s instructions precisely.

What the Test Involves

You will provide a urine sample, which is then analyzed to measure hexanoylglycine levels. The sample may be tested using techniques like chromatography or mass spectrometry to detect the compound accurately.

Possible Results and Interpretation

Results are compared to reference ranges specific to the test method used. Elevated levels may suggest metabolic issues, while normal levels do not definitively rule out underlying conditions. Your healthcare provider will discuss results in the context of your symptoms and medical history.

Clinical interpretation

How abnormal results are interpreted and what conditions they suggest.

Clinical Significance

Short name

Hexanoylglycine Urine Test

Frequently asked questions

What does elevated or low hexanoylglycine mean?
Elevated levels of hexanoylglycine in urine can indicate certain metabolic disorders, such as maple syrup urine disease or other inborn errors of metabolism. These conditions may affect how your body breaks down amino acids, leading to symptoms like vomiting, poor growth, or neurological issues. Your doctor may recommend further testing or dietary adjustments if abnormalities are found.
How often should I get this test?
The frequency of this test depends on your medical condition and treatment plan. If you have a known metabolic disorder, your doctor may order this test regularly to monitor your progress. If it’s part of a routine checkup or screening, follow your healthcare provider’s guidance on timing and frequency.
Can diet or lifestyle changes affect this test?
Yes, diet can influence amino acid metabolism. If you have a suspected metabolic disorder, your doctor may recommend a specific diet to manage your condition. Avoiding certain foods or following a prescribed diet can help stabilize your test results and overall health. Always discuss dietary changes with your healthcare provider before making adjustments.
  1. 1
    MedlinePlus — Laboratory TestsSource
  2. 2
    Lab Tests OnlineSource
  3. 3
    Mayo Clinic — Tests & ProceduresSource

Click any link to access the full source. PubMed Central (PMC) links provide free full-text access.

This information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.

Medical Disclaimer

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.

If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.

Medically reviewed by WellAlly Editorial Team Last reviewed August 16, 2026. · Read our editorial policy