Pipecolic Acid, Serum or Plasma 
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.
ARUP Consult®
Disease Topics
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 one 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 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
AgeReference Interval
0-12 monthsLess than or equal to 5.2 umol/L
Greater than 1 yearLess than or equal to 6.3 umol/L

Interpretive Data

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
2007407Pipecolic Acid, Serum/Plasma32334-5
2007408Pipecolic Acid Ser/Plas 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, serum
  • Delta1-piperideine-6-carboxylate, Serum
  • Pipecolic Acid, Serum/Plasma