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HelpTest Code LAB1965 Succinylacetone Screen, Urine
Clinical System Name
Succinylacetone Screen, Urine
Synonyms
SUAC
Sample Requirements
Specimen: Urine
Container(s): Leak-Proof Container, Sterile Screw-Capped Container
Preferred Vol: 10.0 mL
Minimum Vol: 5.0 (See note)
Note: Minimum volume not defined; the department will determine if quantity is sufficient depending on creatinine concentration.
Processing Instructions
Reject due to:
Spin:N
Aliquot:Y
Temp:-20 C
Storage location: -20 C BCG Box
Off-site collection: Aliquots should be taked from a thoroughly thawed & mixed refrigerated sample. Ship frozen.
Stability
| Temperature | Time |
|---|---|
| Room temp | ≤24 hrs |
| Refrigerated | <24 hrs |
| Frozen | 3 month |
Availability
| STAT | Performed | TAT |
|---|---|---|
| N | M,W,F | 3-7 days |
Contact the Biochemical Genetics Lab for requests outside of stated availability (206)987-2216.
Performing Laboratory
Seattle Children's Laboratory
Department
Department: Biochemical Genetics
Phone Number: 206-987-2216
CPT Codes
83918
Methodology
Method: Gas chromatography/mass spectrometry (GC/MS)
Analytical Volume: Dependent on creatinine concentration.
Limitations:
Description
This is a semi-quantitative assay for succinylacetone as part of urine organic acids. This test can be used to rule out tyrosinemia type I.
Requisition
Biochemical Genetics Requisition
On the requisition include clinical inforamtion needed for appropriate interpretation. (Age, gender, diet (e.g. TPN therapy), drug therapy and family history)
Special Instructions
This test is the same as Organic Acids but "Succinylacetone" must be specified on the requisition.
Clinical Utility
Succinylacetone and succinylacetoacetate are responsible for the liver and kidney toxicity in patients with tyrosinemia type 1, a rare disease causing progressive liver failure and liver cancer in young children. Presence of succinylacetone supports a diagnosis of tyrosinemia type 1. The absence of succinylacetone rules out tyrosinemia type 1.