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
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