Clinics and Departments

Laboratory Services

Lab Dept:

Chemistry

Test Name:

PIPECOLIC ACID, SERUM

General Information

Lab Order Codes:

PIPE

Synonyms:

N/A

CPT Codes:

82543 – Column Chromatography/mass spectrometry, stable isotope dilution, single analyte, quantitative.

Test Includes:

Pipecolic Acid reported in nmol/mL.

Logistics

Test Indications:

Differential diagnosis between disorders of peroxisomal biogenesis and disorders with loss of a single peroxisomal function.

Lab Testing Sections:

Chemistry – Sendouts

Referred to:

Mayo Medical Laboratories (MML Test: 81326/PIPA)

Phone Numbers:

MIN Lab: 612-813-6280

STP Lab: 651-220-6550

Test Availability:

Daily, 24 hours

Turnaround Time:

2 - 31 days

Special Instructions:

Fasting is recommended. See Patient Preparation

Specimen

Specimen Type:

Blood

Container:

Red top tube preferred
Alternate tubes: Any common anticoagulant will be accepted

Draw Volume:

4.5 mL (Minimum: 3 mL) blood

Processed Volume:

1.5 mL (Minimum: 1 mL) serum/plasma

Collection:

Routine venipuncture

Special Processing:

Lab Staff: Centrifuge specimen, remove serum or plasma aliquot into a screw-capped round bottom plastic vial. Store and ship at frozen temperatures. Forward promptly.

Patient Preparation:

Patient should fast 4 hours or more, infants just before next feeding.

Sample Rejection:

Warm; cold refreeze; specimen other than serum or plasma; mislabeled or unlabeled specimens

Interpretive

Reference Range:

Age

Range (nmol/mL)

<6 months:

<or =6.0 nmol/mL

6 months to <1 year:

<or =5.9 nmol/mL

1 – 17 years:

<or =4.3 nmol/mL

>or=18 years:

<or =7.4 nmol/mL

Interpretation: Elevated pipecolic acid levels are seen in disorders of peroxismal 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 plasma C(22)-C(26) very long chain fatty acids, phytanic acid, pristanic acid, RBC plamalogens, and bile acid intermediates.

Critical Values:

N/A

Limitations:

Newborn with disorders of peroxisomal biogenesis often have normal levels of pipecolic acid which increase with age.

Abnormal results may reflect either prematurity or nongenetic liver and/or renal disease.

Methodology:

Gas Chromatography-Mass Spectrometry (GC-MS).

References:

Mayo Medical Laboratories (June 2012)

Updates:

5/8/2012: Reference range updates.



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