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.

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 https://www.aruplab.com/patienthistory 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 days Less than or equal to 19.6 mmol/mol creatinine
1 - 5 months Less than or equal to 12.1 mmol/mol creatinine
6  - 11 months Less than or equal to 7.2 mmol/mol creatinine
Greater than 1 Year Less than or equal to 1.2 mmol/mol creatinine

Interpretive Data

Refer to report.

This test was developed and its performance characteristics determined by ARUP Laboratories. It has not been cleared or approved by the US Food and Drug Administration. This test was performed in a CLIA certified laboratory and is intended for clinical purposes.

Compliance Category

Laboratory Developed Test (LDT)


Hotline History


CPT Codes



Component Test Code* Component Chart Name LOINC
2008132 Creatinine, Urine (Pipecolic) 2161-8
2008134 Pipecolic Acid, Urine 32335-2
2008135 Pipecolic Acid, Urine - Interpretation 48767-8
* 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
Pipecolic Acid, Urine