Organic Acids, Urine
Ordering Recommendation

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

Gas Chromatography/Mass Spectrometry
3-7 days
New York DOH Approval Status
This test is New York DOH approved.
ARUP Consult®
Disease Topics
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 or by contacting ARUP Client Services. 
Ambient: Unacceptable; Refrigerated: Unacceptable; Frozen: 1 month 
Reference Interval
Reports include age appropriate reference intervals and interpretation.
Interpretive Data

Compliance Statement B: For laboratory developed tests not using a RUO kit, and for FDA approved, cleared or 510(k) exempt assays with alterations. This test was developed and its performance characteristics determined by ARUP Laboratories. The U. S. Food and Drug Administration has not approved or cleared this test; however, FDA clearance or approval is not currently required for clinical use. The results are not intended to be used as the sole means for clinical diagnosis or patient management decisions.

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
Component Test Code*Component Chart NameLOINC
0020533Creatinine, Urine2161-8
0081026Lactic Acid, Urine25112-4
0081027Pyruvic Acid, Urine25132-2
0081028Succinic Acid, Urine25136-3
00810292-Ketoglutaric Acid, Urine29507-1
0081030Fumaric Acid, Urine25101-7
0081031Methylmalonic Acid, Urine25116-5
00810323-OH-Butyric Acid, Urine29509-7
0081033Acetoacetic Acid, Urine29524-6
00810342-Keto-3-methylvaleric Acid, Urine25083-7
00810352-Ketoisocaproic Acid, Urine25084-5
00810362-Ketoisovaleric Acid, Urine25085-2
0081037Ethylmalonic Acid, Urine25099-3
0081038Adipic Acid, Urine29859-6
0081039Suberic Acid, Urine25135-5
0081040Sebacic Acid, Urine25134-8
00810414-OH-phenylacetic Acid, Urine25088-6
00810424-OH-phenyllactic Acid, Urine25089-4
00810434-OH-phenylpyruvic Acid, Urine29519-6
0081169Succinylacetone, Urine25137-1
0095241Organic Acids, Urine Interpretation33477-1
* 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.