Ordering Recommendation

Diagnostic evaluation of patients with possible organic acidemias, fatty acid oxidation disorders, and other conditions.

New York DOH Approval Status

This test is New York state approved.

Specimen Required

Patient Preparation

Random urine. Avoid dilute urine when possible.

Specimen Preparation

Transfer 9 mL urine to ARUP standard tubes and freeze immediately. (Min: 3mL. For volumes less than 3mL, contact the Biochemical Genetics Lab before sending the specimen.) Avoid dilute urine when possible.

Storage/Transport Temperature

CRITICAL FROZEN. Separate specimens must be submitted when multiple tests are ordered.

Unacceptable Conditions

Clinical information is needed for appropriate interpretation. Additional required information includes age, gender, diet (e.g., TPN therapy), drug therapy, and family history. Biochemical Genetics Patient History Form is available on the ARUP Web site at https://www.aruplab.com/patienthistory or by contacting ARUP Client Services.


Ambient: Unacceptable; Refrigerated: Unacceptable; Frozen: 1 month


Gas Chromatography-Mass Spectrometry (GC-MS)




3-7 days

Reference Interval

Reports include age appropriate reference intervals and interpretation.

Interpretive Data

Compliance Category

Laboratory Developed Test (LDT)


Certain analytes will be reported only if present at clinically significant concentrations (elevated). Client may request special reporting of an analyte of interest by one of the following methods: transmitting an ORDER COMMENT; faxing the request on the form, Patient History for Biochemical Genetics; or by contacting an ARUP Genetic Counselor at 800- 242-2787 extension 2141.

Hotline History


CPT Codes



Component Test Code* Component Chart Name LOINC
0081026 Lactic Acid, Urine 25112-4
0081027 Pyruvic Acid, Urine 25132-2
0081028 Succinic Acid, Urine 25136-3
0081029 2-Ketoglutaric Acid, Urine 29507-1
0081030 Fumaric Acid, Urine 25101-7
0081031 Methylmalonic Acid, Urine 25116-5
0081032 3-OH-Butyric Acid, Urine 29509-7
0081033 Acetoacetic Acid, Urine 29524-6
0081034 2-Keto-3-methylvaleric Acid, Urine 25083-7
0081035 2-Ketoisocaproic Acid, Urine 25084-5
0081036 2-Ketoisovaleric Acid, Urine 25085-2
0081037 Ethylmalonic Acid, Urine 25099-3
0081038 Adipic Acid, Urine 29859-6
0081039 Suberic Acid, Urine 25135-5
0081040 Sebacic Acid, Urine 25134-8
0081041 4-OH-phenylacetic Acid, Urine 25088-6
0081042 4-OH-phenyllactic Acid, Urine 25089-4
0081043 4-OH-phenylpyruvic Acid, Urine 29519-6
0081169 Succinylacetone, Urine 25137-1
0095241 Organic Acids, Urine Interpretation 33477-1
3002336 Creatinine, Urine 2161-8
* Component test codes cannot be used to order tests. The information provided here is not sufficient for interface builds; for a complete test mix, please click the sidebar link to access the Interface Map.


Organic Acids, Urine