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Acylcarnitine Profile

Referred out for testing

Source Blood
Mnemonic BGLBL

BGL Test Name field: Acylcarnitine
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.

Minimum Whole Blood Volumes for Microcollections

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 mitochondrial fatty acid oxidation disorders and certain organic acidurias.

Metabolites reported: acetylcarnitine (C2), propionylcarnitine (C3), butyryl-/isobutyrylcarnitine (C4), tiglylcarnitine (C5:1), isovaleryl-/2-methylbutyryl-/pivalylcarnitine (C5), hexanoylcarnitine (C6), 3-hydroxyisovaleryl-/2-methyl-3-hydroxybutyrylcarnitine (C5-OH), octenoylcarnitine (C8:1), octanoylcarnitine (C8), malonylcarnitine (C3-DC), decadienoylcarnitine (C10:2), decenoylcarnitine (C10:1), decanoylcarnitine (C10), methylmalonyl-/succinylcarnitine (C4-DC), glutarylcarnitine (C5-DC), dodecenoylcarnitine (C12:1), dodecanoylcarnitine (C12), adipyl-/methylglutarylcarnitine (C6-DC), tetradecadienoylcarnitine (C14:2), tetradecenoylcarnitine (C14:1), myristoylcarnitine (C14), suberylcarnitine (C8-DC), 3-hydroxymyristoylcarnitine (C14-OH), palmitoleylcarnitine (C16:1), palmitoylcarnitine (C16), sebacylcarnitine (C10-DC), 3-hydroxypalmitoleylcarnitine (C16:1-OH), 3-hydroxypalmitoylcarnitine (C16), linoleylcarnitine (C18:2), oleylcarnitine (C18:1), stearoylcarnitine (C18), 3-hydroxylinoleylcarnitine (C18:2-OH), 3-hydroxyoleylcarnitine (C18:1-OH), 3-hydroxystearoylcarnitine (C18-OH), hexadecanedioylcarnitine (C16-DC).
 

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