Succinylacetone, Quantitative, Urine
Also known as: SUAC URINE
Use
The quantitative measurement of succinylacetone in urine is critical for diagnosing and monitoring individuals with tyrosinemia type 1, a genetic disorder affecting tyrosine metabolism. Accurate quantitation at diagnosis is essential, and organic acids testing is recommended as an initial or concurrent test. Monitoring succinylacetone is vital for patients undergoing therapy for tyrosinemia type 1 to assess treatment effectiveness and adjust as needed. Elevations in succinylacetone are consistent with hepatorenal tyrosinemia, and further evaluation with plasma amino acids and potential genetic analysis is suggested for confirmation.
Special Instructions
Not provided.
Limitations
Ambient specimens are unacceptable, and exposure to more than one freeze/thaw cycle can impact the integrity of the specimen. Interpretation may be compromised in specimens with creatinine content below 10 mg/dL, and a more concentrated specimen may be necessary.
New York Approved
Methodology
Mass Spectrometry
Biomarkers
Creatinine
AnalyteSuccinylacetone
Analyte
LOINC Codes
- 25137-1 - Succinylacetone/Creat Ur-sRto
- 2161-8 - Creat Ur-mCnc
- 48767-8 - Annotation comment Imp
Result Turnaround Time
4-12 days
Related Documents
For more information, please review the documents below
Specimen
Urine
Volume
1.0 mL
Minimum Volume
0.3 mL
Container
ARUP standard transport tube
Collection Instructions
Random urine collection with preference for first morning specimen.
Storage Instructions
Freeze immediately after transferring to the transport tube.
Causes for Rejection
Ambient specimens and those exposed to more than one freeze/thaw cycle.
Stability Requirements
| Temperature | Period |
|---|---|
| Room Temperature | Unacceptable |
| Refrigerated | 24 hours |
| Frozen | 1 month |
Other tests from different labs that may be relevant