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Test Code PIPU Pipecolic Acid, Random, Urine

Reporting Name

Pipecolic Acid, U

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

Performing Laboratory

Mayo Clinic Laboratories in Rochester

Specimen Type

Urine


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 Minimum Volume

2 mL

Specimen Stability Information

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

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

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
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

Report Available

3 to 9 days

Reject Due To

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

Method Name

Gas Chromatography Mass Spectrometry (GC-MS)

Highlights

Measurement of pipecolic acid is a useful diagnostic tool for differentiating between peroxisomal biogenesis disorders (Zellweger spectrum disorders) and peroxisomal disorders caused by single enzyme deficiencies, such as X-linked adrenoleukodystrophy.

 

Results must be interpreted together with the results of other biochemical markers for peroxisomal disorders.

 

Both urine and plasma are suitable specimens for the detection of pipecolic acid.

Testing Algorithm

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