Pipecolic Acid, Urine
Ordering Recommendation

Diagnostic evaluation of pyridoxine responsive seizures.

Liquid Chromatography-Tandem Mass Spectrometry
4-12 days
New York DOH Approval Status
This test is New York DOH approved.
ARUP Consult®
Disease Topics
Specimen Required
Patient Preparation
It is preferable that collection and handling of specimens for testing follow Critical Frozen guidelines listed under Specimen Preparation and Storage/Transport Temperature fields. However, specimens that have not been treated as a critical frozen may still be acceptable for testing. Please refer to the information in the Stability field. If urine has been refrigerated for up to 24 hours; freeze the specimen immediately and submit for testing 
Random urine. (first morning urine is preferred) 
Specimen Preparation
Transfer 1 mL urine to an ARUP Standard Transport Tube and freeze immediately. (Min: 0.3 mL) 
Storage/Transport Temperature
Unacceptable Conditions
Clinical information is needed for appropriate interpretation. Additional required information includes age, gender, diet (e.g.TPN therapy), drug therapy, and family history. Biochemical Genetics Patient History Form is available on the ARUP Web site at or by contacting ARUP Client Services. 
Ambient: Unacceptable; Refrigerated: 24 hours; Frozen: 1 month (Avoid repeated freeze/thaw cycles) 
Reference Interval
Reference Interval
0 - 30 daysLess than or equal to 19.6 mmol/mol creatinine
1 - 5 monthsLess than or equal to 12.1 mmol/mol creatinine
6  - 11 monthsLess than or equal to 7.2 mmol/mol creatinine
Greater than 1 YearLess than or equal to 1.2 mmol/mol creatinine

Interpretive Data
Refer to report.

Compliance Statement B: For laboratory developed tests not using a RUO kit, and for FDA approved, cleared or 510(k) exempt assays with alterations. This test was developed and its performance characteristics determined by ARUP Laboratories. The U. S. Food and Drug Administration has not approved or cleared this test; however, FDA clearance or approval is not currently required for clinical use. The results are not intended to be used as the sole means for clinical diagnosis or patient management decisions.

Hotline History
View Hotline History
Component Test Code*Component Chart NameLOINC
2008132Creatinine, Urine (Pipecolic)2161-8
2008134Pipecolic Acid, Urine32335-2
2008135Pipecolic Acid, Urine - Interpretation48767-8
* Component test codes cannot be used to order tests. The information provided here is not sufficient for interface builds; for a complete test mix, please click the sidebar link to access the Interface Map.
  • Alpha-aminoadipic semialdehyde, Urine
  • Delta1-piperideine-6-carboxylate 1, Urine
  • Delta1-piperideine-6-carboxylate 2, Urine
  • Pipecolic Acid, Urine
  • Urine pipecolic acid