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Phenylalanine and Tyrosine Monitoring

Referred out for testing

Source Blood
Mnemonic BGLBL

BGL Test Name field: PKU Monitoring 
Specimen Requirements

1 x 4 mL dark green top sodium heparin tube. 1 x 4 mL dark green lithium heparin, NOT PST, is also acceptable.
Minimum: 0.25 mL plasma

Serum will not be accepted.

OR

pre-labelled phenylalanine blood spot card*

Fasting is NOT required for this test.

* Self-collection of sample by patient using the pre-labelled phenylalanine blood spot card requires previous instruction by an ACH metabolic physician or ACH metabolic dietician.
If patient brings pre-labelled phenylalanine blood spot card to collection lab, warm finger or heel before collection and fill a minimum of two circles on card using a single drop of whole blood (blood must soak through to the back of the card; do NOT smear blood onto card or apply to both sides of card).  If a single drop is insufficient, a second drop can be applied.  Do NOT use capillary tube to transfer blood to the card.  

ACH only: If the patient insists on a phenylalanine blood spot card and does not have a pre-labelled phenylalanine blood spot card with them, send the patient to the ACH Inherited Metabolic Disease Clinic.  Do not use newborn metabolic screen cards

Specimen Handling

Send a copy of the requisition with the specimen

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

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

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

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

If phenylalanine blood spot card is used, either suspend by clipping or lean card against an object on a clean flat non-absorbent surface to dry. Keep card away from sunlight and heat. Allow card to dry for at least 4 hours until the card is COMPLETELY dry before bagging for transport.

Test may be collected any day, however if results are required by Friday, specimen MUST reach Biochemical Genetics Laboratory (located at ACH) by 16:00 on Wednesday.

Additional Information This test is ONLY for the monitoring of patients with an established diagnosis of phenylketonuria or hyperphenylalaninemia. For the investigation of a patient with a suspected diagnosis, the Amino Acids - Blood test should be ordered. 

Patients must bring pre-labeled phenylalanine blood spot card and requisition to the lab for CLS collection staff to perform sample collection.  Do not use newborn metabolic screen cards
Testing Location ACH Biochemical Genetics Lab
Testing Frequency Daily (plasma); weekly (blood spot)
Alternate Name(s)
Reference Interval Provided on test report.