Alberta Precision Laboratories

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Organization:

Alberta Precision Laboratories

Message Alert:

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Test Name/Synonym:

Branched Chain Amino Acids Monitoring, Dried Blood Spot

Clinical Indications:

This test should only be ordered for known patients with a diagnosis of an inherited metabolic disease that requires monitoring of branched chain amino acids.
For the investigation of an undiagnosed patient with a suspicion of an amino acid disorder, the Amino Acids Quantitation, Plasma test should be ordered.

Ordering Alert:

Due to the nature of the testing protocol, concentrations of the amino acids leucine, isoleucine, alloisoleucine and hydroxyproline are reported together as one, not as individual concentrations.
Monitoring that requires accurate quantification of any of these amino acids individually should be done using the Amino Acids Quantitation, Plasma test.

Shipping and Handling Alert:

Ensure that blood spot is dry before closing protective flap.

LABID (Connect Care):

LAB5041

Test Code:

OTHER ZONES

NOT SPECIFIED

Specimen Type:

Bloodspot

Primary Container:

Newborn Metabolic Screening Blood Spot Requisition (card)

Minimum Collection Volume:

2 full blood spots

Specimen Collection Requirements:

Ensure that expiry date of Newborn Metabolic Screening card (requisition) has not been exceeded before proceeding with collection.

Use a heel poke if patient is less than one year old or a finger poke if older.

Collect two saturated blood spots on the Newborn Metabolic Screening card.

Alternative:

Collect blood specimen in a Lithium heparin (no gel) or Sodium heparin (no gel) tube.
Using a capillary tube, transfer whole blood to saturate blood spots on the Newborn Metabolic Screening card.
Do not allow the capillary tube to contact the card.
Each blood spot should require less than 100uL of blood.

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:

Room temperature

Protect from moisture and direct sunlight.

Processing:

Dry the Newborn Metabolic Screening card for a minimum of 3 hours with protective card flap folded back.

Ensure "Amino Acid monitoring" is written on the newborn metabolic screening requisiton (card).

Transportation:

Transport at room temperature.

Method:

Flow-injection tandem mass spectrometry (FI-MS/MS)

Method and Interpretation of Results:

Valine and leucine/isoleucine/alloisoleucine/hydroxyproline concentrations are measured using flow-injection electrospray-ionization tandem mass spectrometry (FI-MS/MS) with stable-isotope labelled internal standards.

Branched chain amino acid monitoring allows for the assessment of a patient’s dietary levels following diagnosis, as well as ongoing adjustments based on their prescribed therapy to ensure levels remain within an acceptable range.

Routine Turn Around Time:

4 days

Testing Schedule:

Daily

Reference Interval:

Reference Intervals are age-dependent and will be provided on the report.

Testing Area:

Biochemical Genetics

Performing Site:

University of Alberta Hospital

Contact Comments:

Newborn Screening & Biochemical Genetics North 780-407-8820

Last Updated On:

Monday, September 19, 2022

Date of Last Review:

Aug 1 2022 12:00AM