Alberta Precision Laboratories

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Organization:

Alberta Precision Laboratories

Message Alert:

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Test Name/Synonym:

Phenylalanine and Tyrosine Monitoring, Plasma (Synonyms: Phenylketonuria monitoring, Hyperphenylalaninemia monitoring, Tyrosinemia monitoring, PKU)

Clinical Indications:

This test is ONLY for monitoring phenylalanine and tyrosine concentrations for known patients with a diagnosis of phenylketonuria/ hyperphenylalaninemia or tyrosinemia.

For the investigation of an undiagnosed patient with a suspicion of an amino acid disorder, the Amino Acids Quantitation, Plasma test should be ordered.

Test Includes:

Amino Acids reported: Phenylalanine and Tyrosine

Ordering Alert:

This test is considered specialty testing; this test will be cancelled if sufficient clinical justification is not provided.

Phenylalanine and Tyrosine Monitoring test may be collected any day, however if results are required by Friday, specimen MUST reach Biochemical Genetics Laboratory by 1600hr on Wednesday.

LABID (Connect Care):

LAB4529

Test Code:

CALGARY ZONE	BGLNON; IN BGL TEST NAME FIELD ENTER PKU MONITORING
CENTRAL ZONE NORTH ZONE (ASPEN) SOUTH ZONE (MEDICINE HAT)	PHTY
EDMONTON ZONE	BPHTY
SOUTH ZONE (LETHBRIDGE)	PHENY
OTHER ZONES	NOT SPECIFIED

Specimen Type:

Blood

Primary Container:

Green top – Sodium heparin (no gel)

Alternate Containers:

Green top – Lithium heparin (no gel)

Minimum Collection Volume:

0.25 mL plasma

Specimen Collection Requirements:

Collect one Sodium Heparin (or alternate) tube.

Test Resources:

Submit request using the applicable requisition or Connect Care online order entry:

Biochemical Genetics Laboratory Requisition

Please ensure all required information, including patient's clinical history/indication, is provided on the requisition.

Stability and Storage:

Frozen: plasma aliquot

Processing:

For ALL collection locations except for Alberta Children’s Hospital (ACH):
1. Centrifuge.
2. Transfer plasma to aliquot tube.
3. Write "NaHep" or "LiHep" on the aliquot tube label.
4. Freeze aliquot tube.

If collected at ACH outpatient Lab:

The primary tube can be put on ice (ensure not to freeze sample) or refrigerated for routine pick-ups between 08:30 to 14:30, Monday through Friday (excluding holidays).
If specimen is collected after 14:30, process sample as described above.

Transportation:

Transport frozen specimens on dry ice.
Samples must not thaw during transport.

Method:

Liquid chromatography tandem mass spectrometry (LC-MS/MS)

Method and Interpretation of Results:

Plasma phenylalanine and tyrosine are measured using electrospray ionization tandem mass spectrometry (ESI-MS/MS) with heavy labelled isotopes as internal standards.

Phenylalanine and tyrosine monitoring allows for the assessment of a patient’s dietary phenylalanine tolerance following diagnosis, as well as ongoing adjustments based on their prescribed therapy to ensure the phenylalanine level remains within an acceptable range. It is recommended that all patients on dietary restriction therapy have a full plasma amino acid profile performed at least once a year, in addition to other micronutrient testing, for evaluation of potential nutritional deficiencies.

Routine Turn Around Time:

7 days

Testing Schedule:

Weekly

Testing Area:

Biochemical Genetics

Performing Site:

Alberta Children’s Hospital

Additional Test Information:

For priority turn-around contact Biochemical Genetics South Laboratory.

Contact Comments:

Biochemical Genetics 403-955-7379

Last Updated On:

Monday, September 19, 2022

Date of Last Review:

Aug 1 2022 12:00AM