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Pipecolic Acid - Urine

Referred out for testing

Source Urine
Mnemonic

BGLNON

BGL Non-Blood Test Name field: Pipecolic Urine

Specimen Requirements

Prefer 5-10 mL random urine.

Minimum: 1.0 mL urine.

Specimen Handling

If specimen is transferred to secondary container, write "urine" on label. Send a copy of the requisition with the specimen.

PSC:
Send on ice to DSC.

RRL:
Send on ice to ACH.

Extra-regional:
Freeze before transport to DSC.

DSC:
Freeze specimens upon receipt in lab; ensure frozen specimens do not thaw.

Additional Information

For the investigation of peroxisomal biogenesis disorders, this test should be ordered in conjunction with Fatty Acids, Very Long Chain (with Phytanic and Pristanic). For the investigation of pyridoxine-dependent seizures, the sensitivity of this test is known to be reduced by the length of time the patient is receiving pyridoxine prior to sample collection.

This test should not be ordered in conjunction with urine organic acids, as this metabolite is also reported as part of that test, and this test will be reflexively performed on any urine sample with elevated levels detected.

Testing Location ACH Biochemical Genetics Lab
Testing Frequency Monthly; must notify ACH BIochemical Genetics Lab for priority turn-around.
Alternate Name(s)
Reference Interval Provided on test report.