Pipecolic Acid, Urine
Also known as: PIPECOL U
Use
This test is used for the diagnostic evaluation of pyridoxine responsive seizures. It is particularly relevant in assessing patients for potential peroxisomal disorders, which are associated with elevated levels of urinary pipecolic acid. Mild elevation can also indicate immature or impaired hepatocellular function, underscoring the importance of clinical correlation for accurate diagnosis. The assessment of pipecolic acid levels thus serves as a supplementary tool in understanding metabolic conditions that affect pyridoxine metabolism and liver function.
Special Instructions
Specimens should ideally follow Critical Frozen guidelines to ensure the accuracy of results, though samples not treated as such may still be valid for testing. Clinical information such as age, gender, diet, drug therapy, and family history is necessary for interpretation and should be submitted using the Biochemical Genetic Testing Patient History Form available on the ARUP website. Ensure specimens are handled according to stability characteristics to avoid compromised results.
Limitations
The test's accuracy may be affected if the urine sample is too dilute, marked by creatinine levels under 10 mg/dL. In such cases, a more concentrated specimen is recommended. Test results should be interpreted with caution in the context of other clinical findings, particularly when an isolated increase in pipecolic acid is observed. Repeated freeze/thaw cycles can also affect the integrity of the sample, thus should be avoided.
New York Approved
Methodology
Mass Spectrometry
Biomarkers
LOINC Codes
- 2161-8
- 32335-2
- 48767-8
Result Turnaround Time
4-12 days
Related Documents
For more information, please review the documents below
Specimen
Urine
Volume
1 mL
Minimum Volume
0.3 mL
Container
ARUP standard transport tube
Collection Instructions
Collect random urine; first morning urine is preferred. Transfer to a transport tube and freeze immediately.
Storage Instructions
Specimens must be frozen for transportation.
Causes for Rejection
Inappropriate storage conditions, such as ambient temperature.
Stability Requirements
| Temperature | Period |
|---|---|
| Room Temperature | Unacceptable |
| Refrigerated | 24 hours |
| Frozen | 1 month |