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Ethylmalonic Acid, Urine

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

Measures ethylmalonic acid in urine, sometimes interpreted relative to creatinine. It can help evaluate suspected inborn errors of metabolism with other findings.

Quick summary

["This urine test measures ethylmalonic acid, a marker that can be elevated in certain rare metabolic disorders."]

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

Key facts at a glance

LOINC
13741-4
Short code
Ethylmalonate/Creat Ur
Synonyms
Ethylmalonate/Creat Ur
Category
kidney
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

What is this test?

This test measures ethylmalonic acid, a substance that can be produced during normal metabolism. Depending on the laboratory, the result may be reported as a urine concentration or interpreted relative to urine creatinine.

Why might I need this test?

This test may be ordered to investigate a suspected inborn error of metabolism, especially when a child has unexplained developmental, neurologic, or metabolic symptoms. It is usually interpreted with other laboratory and clinical findings.

How is the test performed?

A urine sample is collected and analyzed in the laboratory. The laboratory may also measure urine creatinine at the same time so the ethylmalonic acid result can account for variations in urine concentration.

How do I prepare for the test?

Follow the collection instructions provided by your healthcare professional or laboratory. A clean-catch sample may be requested, or all urine may need to be collected for 24 hours. Do not change your diet, fasting pattern, or medications unless specifically instructed.

Clinical interpretation

How abnormal results are interpreted and what conditions they suggest.

◆Associated findings1

Short name

Urine ethylmalonic acid

Frequently asked questions

What does an elevated ethylmalonic acid level mean?
It may suggest certain inherited metabolic disorders, particularly ethylmalonic encephalopathy, but the finding is not specific by itself. Your clinician will interpret it with your symptoms, medical history, and additional test results.
Is this test the same as a 24-hour urine test?
Not necessarily. It may be performed on a single urine sample or measured during a 24-hour collection, depending on the laboratory and the reason for testing.
Why is urine creatinine sometimes included?
Urine creatinine helps account for how concentrated or dilute a urine sample is. Expressing ethylmalonic acid relative to creatinine can make results from different samples easier to compare.
  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