Alberta Precision Laboratories

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Organization:

Alberta Precision Laboratories

Message Alert:

PLEASE NOTE: This Test Directory is in an interim state and due to changes pertaining to the DynaLIFE transition, performing site for routing has not been updated. Please refer to Laboratory Information System for appropriate routing.

Test Name/Synonym:

Beta-Galactosidase, T-cell (Synonym: GM1 gangliosidosis, Morquio B Syndrome, Mucopolysaccharidosis IVB, MPS IVB, Mucopolysaccharidosis Type 4B, Lysosomal Enzymes Panel)

Clinical Indications:

For the investigation of beta-galactosidase deficiency leading to either GM1 Gangliosidosis (a.k.a., Landing disease) or Morquio B Syndrome (a.k.a., mucopolysaccharidosis type IVB) - lysosomal storage disorders.

Test Includes:

Beta-Galactosidase activity

Ordering Alert:

For those sites that routinely refer tests to Edmonton zone labs, refer to Beta-Galactosidase, Leukocyte

This test is considered specialty testing; this test will be cancelled if sufficient clinical justification is not provided. Requests for testing should be supported by a high index of clinical suspicion specific to the disorder in question.

Connect Care Users: Order Lysosomal Enzymes (LAB4043), then select the enzyme(s) to be tested. If ordering through Requisition Entry, order the individual enzyme.

Shipping and Handling Alert:

Specimen must be received by a specific time. See Transportation for details.

LABID (Connect Care):

LAB4722

Test Code:

CALGARY ZONE	BGLBL; IN BGL TEST NAME FIELD ENTER BGAL
OTHER ZONES	NOT SPECIFIED

Specimen Type:

Blood

Primary Container:

Green Top - Sodim Heparin (no gel)

Alternate Containers:

Green Top - Lithium Heparin (no gel)

Minimum Collection Volume:

3.0 mL

Specimen Collection Requirements:

Collect one Sodium Heparin (or alternate) tube.

Note: 3.0 mL of whole blood will be sufficient to test for multiple lysosomal enzymes in T-cells, if requested.

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

Processing:

Do NOT centrifuge.
Do NOT aliquot.
Do NOT refrigerate or freeze.

Transportation:

Transport whole blood at room temperature.

All specimens MUST arrive in lab by:

Monday through Thursday: by 14:00 and within 48 hours of time of collection
Friday: by 12:00 on same day of collection.

Method:

Fluorometric

Method and Interpretation of Results:

Beta-Galactosidase activity is measured fluorometrically using a 4-methylumbelliferyl-linked substrate.

This procedure involves the establishment of an IL-2 stimulated T-lymphocyte culture and storage of cell pellets for the purpose of lysosomal enzyme testing.

Routine Turn Around Time:

45 days

Testing Schedule:

Monthly

Reference Interval:

Enzyme Activity for	Range	Units
beta-Galactosidase	155 - 489	nmol/hour/mg protein

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 South 403-955-7379

Last Updated On:

Monday, August 29, 2022

Date of Last Review:

Aug 1 2022 12:00AM