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HelpTest Code PIPASO Pipecolic Acid, Serum
Additional Codes
Mayo Test Code: PIPA
Reporting Name
Pipecolic Acid, SUseful For
Differentiating between disorders of peroxisomal biogenesis (eg, Zellweger syndrome) and disorders with loss of a single peroxisomal function
Detecting abnormal elevations of pipecolic acid in serum
Performing Laboratory
Mayo Clinic Laboratories in Rochester
Specimen Type
SerumNecessary Information
Patient's age is required.
Specimen Required
Patient Preparation:
Fasting 12 hours, required; Infants and small children should have specimen collected before next feeding/meal
Supplies: Sarstedt Aliquot Tube, 5 mL (T914)
Collection Container/Tube:
Preferred: Serum gel
Acceptable: Red top
Submission Container/Tube: Plastic vial
Specimen Volume: 1.5 mL serum
Collection Instructions: Centrifuge and aliquot serum into plastic vial.
Specimen Minimum Volume
Serum: 1 mL
Specimen Stability Information
| Specimen Type | Temperature | Time |
|---|---|---|
| Serum | Frozen (preferred) | 94 days |
| Refrigerated | 14 days |
Special Instructions
Reference Values
<6 months: ≤6.0 nmol/mL
6 months-<1 year: ≤5.9 nmol/mL
1-17 years: ≤4.3 nmol/mL
≥18 years: ≤7.4 nmol/mL
Day(s) Performed
Tuesday
Test Classification
This test was developed and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. It has not been cleared or approved by the US Food and Drug Administration.CPT Code Information
82542
LOINC Code Information
| Test ID | Test Order Name | Order LOINC Value |
|---|---|---|
| PIPA | Pipecolic Acid, S | 32334-5 |
| Result ID | Test Result Name | Result LOINC Value |
|---|---|---|
| 81326 | Pipecolic Acid, S | 32334-5 |
| 29962 | Interpretation | 59462-2 |
| 29964 | Reviewed By | 18771-6 |
Cautions
Newborns with disorders of peroxisomal biogenesis often have normal levels of pipecolic acid that increase with age.
Abnormal results may reflect either prematurity or nongenetic liver or kidney disease.
Vigabatrin interferes with pipecolic acid determination.
Methylmalonic acid interferes with pipecolic acid determination.
Report Available
3 to 9 daysSpecimen Retention Time
1 monthReject Due To
| Gross hemolysis | OK |
| Gross lipemia | OK |
| Gross icterus | OK |
Method Name
Gas Chromatography Mass Spectrometry (GC-MS)
Testing Algorithm
For more information see Epilepsy: Unexplained Refractory and/or Familial Testing Algorithm
Forms
If not ordering electronically, complete, print, and send a Biochemical Genetics Test Request (T798) with the specimen.