Ordering Recommendation

Diagnostic evaluation for peroxisomal disorders (in conjunction with very long chain fatty acids testing) and/or 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

Adults: Fasting specimen preferred.
Infants and children: Draw specimen prior to feeding or 2-3 hours after a meal.


Green (sodium or lithium heparin), lavender (EDTA), plain red, or serum separator tube.

Specimen Preparation

Separate serum/plasma from cells immediately. Transfer 0.5 mL serum/plasma to an ARUP Standard Transport Tube and freeze immediately. (Min: 0.2 mL)

Storage/Transport Temperature

CRITICAL FROZEN. Separate specimens must be submitted when multiple tests are ordered.

Unacceptable Conditions

Samples exposed to more than two freeze/thaw cycle.


Clinical information is needed for appropriate interpretation. Submit age, gender, diet (e.g.TPN therapy), drug therapy, and family history on a Biochemical Genetics Patient History Form available at www.aruplab.com/patienthistory or by contacting ARUP Client Services at (800) 522-2787.


After separation from cells: Ambient: Unacceptable; Refrigerated: 24 hours; Frozen: 1 month

Reference Interval

Effective August 19, 2013

Age Reference Interval
0-12 months Less than or equal to 5.2 umol/L
Greater than 1 year Less than or equal to 6.3 umol/L

Interpretive Data

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
2007407 Pipecolic Acid, Serum/Plasma 32334-5
2007408 Pipecolic Acid Ser/Plas 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, serum
  • Delta1-piperideine-6-carboxylate, Serum
  • Pipecolic Acid, Serum/Plasma
Pipecolic Acid, Serum or Plasma