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HelpTest ID MPSQU Mucopolysaccharides Quantitative, Random, Urine
Ordering Guidance
This test alone is not appropriate for the diagnosis of a specific mucopolysaccharidosis (MPS). Follow-up enzymatic or molecular genetic testing must be performed to confirm a diagnosis of an MPS.
Necessary Information
1. Patient's age is required.
2. Reason for testing is required.
3. Biochemical Genetics Patient Information (T602) is recommended. This information aids in providing a more thorough interpretation of results. Send information with specimen.
Specimen Required
Patient Preparation: Do not administer low-molecular weight heparin prior to collection
Supplies: Sarstedt Aliquot Tube, 5 mL (T914)
Container/Tube: Plastic, 5-mL urine tube
Specimen Volume: 2 mL
Pediatric Volume: 1 mL
Collection Instructions: Collect a random urine specimen (early morning preferred).
Forms
1. Biochemical Genetics Patient Information (T602)
2. If not ordering electronically, complete, print, and send a Biochemical Genetics Test Request (T798) with the specimen.
Useful For
Supporting the biochemical diagnosis of one of the mucopolysaccharidoses: types I, II, III, IV, VI, or VII
Testing Algorithm
For more information see Lysosomal Storage Disorders Diagnostic Algorithm, Part 1
Special Instructions
Method Name
Liquid Chromatography Tandem Mass Spectrometry (LC-MS/MS)
Reporting Name
Mucopolysaccharides Quant, USpecimen Type
UrineSpecimen Minimum Volume
1 mL
Specimen Stability Information
| Specimen Type | Temperature | Time |
|---|---|---|
| Urine | Refrigerated (preferred) | 90 days |
| Frozen | 365 days | |
| Ambient | 7 days |
Reference Values
DERMATAN SULFATE
≤1.00 mg/mmol creatinine
HEPARAN SULFATE
≤4 years: ≤0.50 mg/mmol creatinine
≥5 years: ≤0.25 mg/mmol creatinine
CHONDROITIN-6 SULFATE
≤24 months: ≤10.00 mg/mmol creatinine
25 months-10 years: ≤2.50 mg/mmol creatinine
≥11 years: ≤1.50 mg/mmol creatinine
KERATAN SULFATE
≤12 months: ≤2.00 mg/mmol creatinine
13-24 months: ≤1.50 mg/mmol creatinine
25 months-4 years: ≤1.00 mg/mmol creatinine
5-18 years: ≤0.50 mg/mmol creatinine
≥19 years: ≤0.30 mg/mmol creatinine
Day(s) Performed
Monday
Report Available
8 to 15 daysPerforming Laboratory
Mayo Clinic Laboratories in Rochester
Test Classification
This test was developed and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. It has not been cleared or approved by the US Food and Drug Administration.CPT Code Information
83864
82570
LOINC Code Information
| Test ID | Test Order Name | Order LOINC Value |
|---|---|---|
| MPSQU | Mucopolysaccharides Quant, U | 94691-3 |
| Result ID | Test Result Name | Result LOINC Value |
|---|---|---|
| BG716 | Reason for Referral | 42349-1 |
| 605986 | Dermatan Sulfate | 94692-1 |
| 605987 | Heparan Sulfate | 94693-9 |
| 605988 | Chondroitin-6 Sulfate | 94690-5 |
| 605989 | Keratan Sulfate | 92806-9 |
| 605990 | Interpretation | 59462-2 |
| 605985 | Reviewed By | 18771-6 |