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Test Directory

Test Directory Very Long Chain Fatty Acids

Very Long Chain Fatty Acids

Test Summary

Peroxisomes play a vital role in several metabolic pathways, including the synthesis of plasminogen's and bile acids. They are also involved in the catabolism of Very Long Chain Fatty Acids (VLCFAs), phytanic acid, and pristanic acid. Defects in these pathways usually result in the accumulation in tissues and body fluids of one or more metabolites derived from the blocked metabolic steps. Specific accumulations are used for the differential biochemical diagnosis of numerous peroxisomal disorders. These disorders include (A) Zellweger spectrum disorders of peroxisomal biogenesis, (B) X-linked adrenoleukodystrophy (X-ALD) and its adult form X-linked adrenomyeloneuropathy (X-AMN), (C) Refsum disease (Phytanoyl-CoA hydroxylase Deficiency), and (D) 2-methylacyl-CoA racemase deficiency.

Aliases

Specimen Collection

Special Instructions

N/A

Preferred Specimen

1 mL serum collected in red-top tube (no gel)

Minimum Volume

0.2 mL

Instructions

Patient age and gender are required for reporting.

Patient Preparation

Fasting Required (overnight fast). No alcohol consumption for 24 hours prior to draw. For pediatric patients, fasting is not required, but sample collection should occur prior to the next meal or scheduled feeding.

Storage

N/A

Transport Temperature

Frozen

Specimen Stability

Room temperature: Unacceptable
Refrigerated: 7 days
Frozen: 28 days

Limitations

N/A

Other Acceptable Specimens

Plasma collected in: EDTA (lavender-top) tube

Unacceptable Specimens

Non-fasting samples • Lipemic samples • Serum separator tube • Hemolysis

Order Code

VLNGFA

EPIC (Premier) Code

LAB5740

Includes

Phytanic Acid
Pristanic Acid
Docosanoic, C22:0
Tetracosanoic, C24:0
Hexacosanoic, C26:0
Ratio C24/C22
Ratio C26/C22
Ratio Pristanic/Phytanic
Interpretation

CPT Code

82726

Billing Code

670959

CPT Statement

Methodology

Gas Chromatography/Tandem Mass Spectrometer

FDA Status

This test was developed and its analytical performance characteristics have been determined by Quest Diagnostics. It has not been cleared or approved by the U.S. Food and Drug Administration. The FDA has determined that such clearance or approval is not necessary. This assay has been validated pursuant to the CLIA regulations and is used for clinical purposes.

Physician Attestation of Informed Consent

N/A

Testing Laboratory

Quest Diagnostics Nichols Institute
33608 Ortega Highway
San Juan Capistrano CA, 92675

Department

Reference Testing

Reference Range

See Laboratory Report

Setup Schedule / Expected Turnaround Time

Tuesday, Thursday, Saturday; Report available: 6 - 8 days