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:

Quantitative Total GAGs (Monitoring) (Synonyms: Glycosaminoglycans, Mucopolysaccharides, DMB, DMMB, Carbazole, Uronic Acids)

Clinical Indications:

This test is used to monitor known mucopolysaccharidosis patients on treatment.

Test Includes:

Total sulfated GAGs (by dimethylmethylene blue)
Total uronic acids (by carbazole reagent)

Ordering Alert:

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

LABID (Connect Care):

LAB1234021

Test Code:

CALGARY ZONE	BGLNON; IN BGL NON-BLOOD TEST NAME FIELD ENTER GAG MONITOR
OTHER ZONES	NOT SPECIFIED

Specimen Type:

Urine

Primary Container:

Sterile container

Alternate Containers:

Non sterile container

Minimum Collection Volume:

4.0 mL

Specimen Collection Requirements:

First morning void is preferred.
Collect a 5.0 to 10.0 mL random urine specimen.

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

Processing:

This test requires creatinine determination for result reporting. For sites using EPIC, a separate aliquot should be removed at the time of initial specimen receipt and sent to the EPIC-designated lab for creatinine testing.

One aliquot with primary container, or two separate aliquots, can be submitted. Write “urine” on all secondary aliquot tubes. Freeze container and/or tubes.

Transportation:

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

Method:

Spectrophotometric

Method and Interpretation of Results:

Total sulfated GAGs are determined spectrophotometrically following formation of a complex with dimethylmethylene blue (DMMB) dye.

Total uronic acids are determined spectrophotometrically following precipitation with a cationic detergent, washing, hydrolysis and detection using the carbazole reagent.

NOTE: The uronic acid method does not detect keratan sulfate, as neither of its constituent subunits (i.e., galactose and N-acetylglucosamine) is a hexuronic acid.

Very dilute urine samples may give false negative results and therefore may be cancelled.

Routine Turn Around Time:

21 days

Testing Schedule:

Biweekly

Reference Interval:

Total Sulfated GAGs (DMMB) Normal Reference Interval is Age-Dependent

Age	Normal Range	Units
Less than 5 months	< 53	mg/mmol creatinine
5 to 19 months	< 31	mg/mmol creatinine
19 to 36 months	< 24	mg/mmol creatinine
36 - 72 months	< 16	mg/mmol creatinine
72 - 132 months	< 12	mg/mmol creatinine
132 - 168 months	< 10	mg/mmol creatinine
Greater than 168 months	<6.5	mg/mmol creatinine

Total Uronic Acids (carbazole) Normal Reference Interval is Age-Dependent

Age	Nommal Range	Units
Less than 12 months	1.32 - 11.90	ug/mmol creatinine
12 to 30 months	1.16 - 6.25	ug/mmol creatinine
30 to 60 months	0.72 - 3.51	ug/mmol creatinine
60 - 156 months	0.48 - 2.64	ug/mmol creatinine
Greater than 156 months	0.22 - 2.22	ug/mmol creatinine

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 26, 2022

Date of Last Review:

Aug 1 2022 12:00AM