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).

New York DOH Approval Status

This test is New York state approved.

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 www.aruplab.com/patienthistory or by contacting ARUP Client Services at 800-522-2787.


Ambient: Unacceptable; Refrigerated: 24 hours; Frozen: 1 month


Liquid Chromatography-Tandem Mass Spectrometry




4-12 days

Reference Interval

0.000-0.300 mmol/mol CRT

Interpretive Data

Compliance Category

Laboratory Developed Test (LDT)


Hotline History


CPT Codes



Component Test Code* Component Chart Name LOINC
2007402 Succinylacetone Quant Urine 25137-1
2007404 Creatinine (Succinylacetone), Urine 2161-8
2007405 Succinylacetone 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
Succinylacetone, Quantitative, Urine