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Fatty Acids, Very Long Chain (with Phytanic and Pristanic)

Referred out for testing

Source Blood
Mnemonic

BGLBL

BGL Test Name field: Fatty Acids, VLC

Specimen Requirements

1 x 4 mL dark green top sodium heparin tube.  1 x 4 mL dark green lithium heparin, NOT PST, and red top non-additive tube are also acceptable.

Minimum: 0.25 mL plasma or serum.

Requires a minimum four-hour fast, or just prior to next feed for newborns.  Patients should avoid eating peanut-rich goods (e.g., peanut butter) 24 hours prior to collection.

Specimen Handling

Send a copy of the requisition with the specimen.

PSC:
Centrifuge, transfer plasma or serum to aliquot tube, write "NaHep", "LiHep" or "Red Top" on the aliquot tube, and transport on ice to DSC.

RRL:
Centrifuge, transfer plasma or serum to aliquot tube, write "NaHep", "LiHep" or "Red Top" on the aliquot tube, and transport on ice to ACH.

Extra-regional:
Centrifuge, transfer plasma or serum to aliquot tube, write "NaHep", "LiHep" or "Red Top" on the aliquot tube, and freeze prior to transport to DSC.

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

Additional Information

This test is for the investigation of peroxisomal biogenesis disorders and specific peroxisomal fatty-acid oxidation disorders.  For the investigation of very long chain acyl-CoA dehydrogenase deficiency, order test Acylcarnitine - Blood

Metabolites reported: behenic (docosanoic) acid (C22:0), lignoceric (tetracosanoic) acid (C24:0), ceratinic (hexacosanoic) acid (C26:0), pristanic (2,6,10,14-tetramethylpentadecanoic) acid, phytanic (3,7,11,15-tetramethylhexadecanoic) acid.

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.