--- Query Results ---

Program:Genetics and Genomics
Test Name:Uronic Acids, Quantitative (G&G South ACH)
Test Code:UA-U
Performing Site: Alberta Children's Hospital
Performing Dept:Biochemical Genetics
Availability:Test performed on a biweekly basis.
TAT:21 days
Preferred Tube/Container:Random specimen: plain urine container
Alternate:24 hour urine collection container (NO ADDITIVE)
Specimen Requirements:Random Urine Collection
1. Prefer first morning void whenever possible.
2. Collect 5-10 mL random urine sample.

24 hour Urine Collection:
1. Urine specimen MUST be kept FROZEN during entire collection and transport process (no preservative).
2. Instruct patient to ensure to place 24 hour urine collection container into freezer immediately after each void throughout the 24 hour collection period.
Min. Sample Required:4.0 mL urine.
Specimen Processing:Random Urine Collection:
1. If sample is transferred to a secondary aliquot tube or container, write "random urine" on label.
2. Freeze.

24 hour Urine Collection:
1. Thaw 24 hour urine specimen.
2. Measure total volume.
3. Enter TVOL on requisition.
4. Send 10 mL aliquot of urine; write "24hr urine, Tvol #L" on the label.
5. Freeze aliquot.
Specimen Handling:Transport frozen samples on ice. Samples must not thaw during transport.
Additional Information:This test can either be ordered in conjunction with Glycosaminoglycan Electrophoresis, Qualitative (ACH) , or, if the level of uronic acids is found to be elevated in a urine sample, Glycosaminoglycan Electrophoresis, Qualitative will be reflexively tested and reported separately. Minimum volume is for both tests.

Samples will be rejected if:
  • Aliquot tube received thawed
  • Sample type not written on aliquot tube label
    Requisition/Form:Submit request using Biochemical Genetics Laboratory Requisition.
    Please ensure all required information, including patient's clinical history/indication is provided on the requisition.
    Order Restrictions:This test is considered specialty testing; this test will be cancelled if sufficient clinical justification is not provided.
    Indications:This test should be ordered when there is a strong clinical suspicion of a mucopolysaccharidosis in a patient, or when the initial glycosaminoglycan (i.e., mucopolysaccharide) screen was found to be abnormal and there was insufficient sample for reflexive testing.
    Method:Uronic acids are determined spectrophotometrically following precipitation with a cationic detergent, washing and hydrolysis with carbazole reagent.
    Method Details:Elevated uronic acids indicate the presence of abnormal levels of the glycosaminoglycans dermatan and/or heparan sufate in addition to the normal urine constituents chondroitin 4 and 6 sulfates, and is highly suggestive of a biochemical diagnosis of a mucopolysaccharidosis. Such a finding requires further identification of which glycosaminoglycan(s) has contributed to this finding. Further enzyme or molecular-based testing is required to confirm the specific enzyme deficiency in each case.

    NOTE: this method does not detect keratan sulfate, as neither of its constituent subunits (i.e., galactose and N-acetylglucosamine) is a hexuronic acid. Therefore, in patients with a strong clinical suspicion of Morquio syndrome, both quantitative uronic acids AND GAG electrophoresis should be ordered.
    Reference Interval:
    Uronic Acids             (<12 months)  1.32-11.90  ug/mmol creatinine
    Uronic Acids          (12-30 months)    1.16-6.25  ug/mmol creatinine
    Uronic Acids          (30-60 months)    0.72-3.51   ug/mmol creatinine
    Uronic Acids        (60-156 months)    0.48-2.64   ug/mmol creatinine
    Uronic Acids           (>156 months)    0.22-2.22   ug/mmol creatinine
    Comments:Contact ACH Biochemical Genetics Laboratory (403-955-7379) for priority turn-around.
    Last Updated On:Tuesday, May 22, 2018
    Date of Last Review:Jan 2 2018 12:00AM