Pipecolic Acid

Last Modified: 12/28/2023 7:22:49 AM


Medical Necessity Documentation:  
Client Notes:
Patient Preparation: 12 hours Fasting - Fasting  (infants and small children just before next feeding)
Specimen Requirements:

1.5 mL Serum in a SST Gold Top Tube
Collection Instructions: Patient age is required on order along with all other required inforamtion.

Spin down within 45 minutes of draw

Required Form if not ordered electronically with Mayo 
Minimum Volume: 1.0 mL Serum
Transport & Storage: Temperature/Stability: 14 days Refrigerated
94 days Frozen (preferred)
Rejection Criteria: Gross hemolysis • gross lipemia • gross icterus
Reference Range:

<6 months: < or =6.0 nmol/mL
6 months-<1 year: < or =5.9 nmol/mL
1-17 years: < or =4.3 nmol/mL
> or =18 years:  < or =7.4 nmol/mL

Elevated pipecolic acid levels are seen in disorders of peroxisomal biogenesis; normal levels are seen in disorders with loss of a single peroxisomal function.

Abnormal levels of pipecolic acid should be interpreted together with the results of other biochemical markers of peroxisomal disorders, such as plasma C22-C26 very long-chain fatty acids, phytanic acid, and pristanic acid (POX / Fatty Acid Profile, Peroxisomal [C22-C26], Serum), RBC plasmalogens, and bile acid intermediates
Critical Ranges:  
Test Comments:  
Methodology: Gas Chromatography-Mass Spectrometry (GC-MS)
Clinical Significance:

Differential diagnosis between disorders of peroxisomal biogenesis (eg, Zellweger syndrome) and disorders with loss of a single peroxisomal function

Abnormal elevations of pipecolic acid can be detected in either serum or urine
Custom Panel: No

PRODUCTION SCHEDULE

Turn Around Time: Batched twice a month -
Sites Performed: Mayo Laboratory
PHL Test Code: MSOT
EPIC Test Code:
Send Out Test Code: PIPA
Alternate Test Names: Pipecolic Acid, S
Included Tests:  
CPT Coding: 82542

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