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Very Long Chain Fatty Acids
MessageFasting overnight required (exception is pediatric patients).
No alcohol consumption for 24 hours prior to draw.
CENTRIFUGE AND FREEZE SERUM/PLASMA
No alcohol consumption for 24 hours prior to draw.
CENTRIFUGE AND FREEZE SERUM/PLASMA
Test Code
90559
Alias/See Also
LAB987
FATTY ACIDS, LONG CHAIN
FATTY ACIDS, LONG CHAIN
CPT Codes
82726
Includes
Phytanic Acid
Pristanic Acid
Docosanoic, C22:0
Tetracosanoic, C24:0
Hexacosanoic, C26:0
Ratio C24/C22
Ratio C26/C22
Ratio Pristanic/Phytanic
Interpretation
Pristanic Acid
Docosanoic, C22:0
Tetracosanoic, C24:0
Hexacosanoic, C26:0
Ratio C24/C22
Ratio C26/C22
Ratio Pristanic/Phytanic
Interpretation
Preferred Specimen
1 mL serum collected in red-top tube (no gel)
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.
Minimum Volume
0.2 mL
Other Acceptable Specimens
Plasma collected in: EDTA (lavender-top) tube
Instructions
Patient age and gender are required for reporting
Transport Container
Transport tube
Transport Temperature
Frozen
Specimen Stability
Room temperature: Unacceptable
Refrigerated: 7 days
Frozen: 28 days
Refrigerated: 7 days
Frozen: 28 days
Reject Criteria (Eg, hemolysis? Lipemia? Thaw/Other?)
Hemolysis • Lipemia • Serum separator tube (SST) • Non-fasting samples
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 FDA. This assay has been validated pursuant to the CLIA regulations and is used for clinical purposes.
Setup Schedule
Mon, thurs
Report Available
4-8 days
Reference Range
See Laboratory Report
Clinical Significance
Peroxisomes play a vital role in several metabolic pathways, including the synthesis of plasmalogens 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.
Performing Laboratory
Quest Diagnostics Nichols Institute-San Juan Capistrano, CA |
33608 Ortega Highway |
San Juan Capistrano, CA 92675-2042 |