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 (LC-MS/MS)
Random urine. (First morning urine is preferred)
Transfer 1.0 mL urine to an ARUP Standard Transport Tube and freeze immediately. (Min: 0.3 mL)
CRITICAL FROZEN. Separate specimens must be submitted when multiple tests are ordered.
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
0.000-0.300 mmol/mol CRT
This test was developed and its performance characteristics determined by ARUP Laboratories. It has not been cleared or approved by the US Food and Drug Administration. This test was performed in a CLIA certified laboratory and is intended for clinical purposes.
Laboratory Developed Test (LDT)
|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|
- succinylacetone levels
- succinylacteone concentration