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Test Code PIPA Pipecolic Acid, Serum

Performing Laboratory

Mayo Clinic Laboratories in Rochester

Reporting Name

Pipecolic Acid, S

Specimen Type

Serum


Necessary 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.


Reject Due To

Gross hemolysis OK
Gross lipemia OK
Gross icterus OK

Specimen Stability Information

Specimen Type Temperature Time
Serum Frozen (preferred) 94 days
  Refrigerated  14 days

Specimen Minimum Volume

Serum: 1 mL

Day(s) Performed

Tuesday

Specimen Retention Time

1 month

Report Available

3 to 9 days

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

Useful 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

Testing Algorithm

For more information see Epilepsy: Unexplained Refractory and/or Familial Testing Algorithm

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

Genetics Test Information

In the newborn period, pipecolic acid levels are more likely to be abnormal in urine than in plasma or serum. 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, pristanic acid, red blood cell plasmalogens, and bile acid intermediates.