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Test Code PIPU Pipecolic Acid, Random, Urine

Reporting Name

Pipecolic Acid, U

Performing Laboratory

Mayo Clinic Laboratories in Rochester

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 urine

Method Name

Gas Chromatography Mass Spectrometry (GC-MS)


Necessary Information


Patient's age is required.



Specimen Required


Supplies: Urine tubes, 10 mL (T068)

Container/Tube: Plastic, 10-mL urine tube

Specimen Volume: 5 mL

Collection Instructions:

1. Collect a random urine specimen.

2. No preservative.


Specimen Type

Urine

Specimen Minimum Volume

2 mL

Specimen Stability Information

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

Reject Due To

  All specimens will be evaluated at Mayo Clinic Laboratories for test suitability.

Reference Values

≤31 days: ≤223.8 nmol/mg creatinine

32 days-5 months: ≤123.1 nmol/mg creatinine

6 months-11 months: ≤45.0 nmol/mg creatinine

≥1 year: ≤5.7 nmol/mg creatinine

Interpretation

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 serum C22-C26 very long-chain fatty acids, phytanic acid, pristanic acid (POX / Fatty Acid Profile, Peroxisomal [C22-C26], Serum); red blood cell plasmalogens (PGRBC / Plasmalogens, Blood); and bile acid intermediates (BAIPD / Bile Acids for Peroxisomal Disorders, Serum).

Day(s) Performed

Tuesday

Report Available

3 to 9 days

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
PIPU Pipecolic Acid, U 33659-4

 

Result ID Test Result Name Result LOINC Value
81248 Pipecolic Acid, U 33659-4
29952 Interpretation 59462-2
29954 Reviewed By 18771-6

Testing Algorithm

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