Succinylacetone, Quantitative, Urine
Ordering Recommendation

For accurate quantitation of succinylacetone at diagnosis, organic acids recommended as initial or concurrent test. Monitor individuals with tyrosinemia type 1 who are on therapy. To rule out or diagnose tyrosinemia type 1, refer to Organic Acids, Urine (0098389) and Amino Acids Quantitative by LC-MS/MS, Plasma (2009389).

Liquid Chromatography/Tandem Mass Spectrometry
4-12 days
New York DOH Approval Status
This test is New York DOH approved.
ARUP Consult®
Disease Topics
Specimen Required
Patient Preparation
Random urine. (First morning urine is preferred) 
Specimen Preparation
Transfer 1.0 mL urine to an ARUP Standard Transport Tube and freeze immediately. (Min: 0.3 mL) 
Storage/Transport Temperature
CRITICAL FROZEN. Separate specimens must be submitted when multiple tests are ordered. 
Unacceptable Conditions
Ambient specimens. Specimens exposed to more than one freeze/thaw cycle. 
Clinical information is needed for appropriate interpretation. Submit age, gender, diet (e.g., TPN therapy), drug therapy, and family history on a Biochemical Genetics Patient History Form available at or by contacting ARUP Client Services at (800) 522-2787. 
Ambient: Unacceptable; Refrigerated: 24 hours; Frozen: 1 month 
Reference Interval
0.000-0.300 mmol/mol CRT
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.

Hotline History
Component Test Code*Component Chart NameLOINC
2007402Succinylacetone Quant Urine25137-1
2007404Creatinine (Succinylacetone), Urine2161-8
2007405Succinylacetone Quant Urine, Interpret.48767-8
* 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.
  • succinylacetone levels
  • succinylacteone concentration