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24810 Mucopolysaccharides, Quantitative, Random, Urine (MPSQU)

Mucopolysaccharides, Quantitative, Random, Urine (MPSQU)
Test Code: MPSQUSO
Synonyms/Keywords
​Arylsulfatase B Deficiency, Beta-Galactosidase Deficiency, Beta-Glucoronidase Deficiency, Chondroitin-6-Sulfate, Dermatan Sulfate,
GAGS (Glycosaminoglycans), Galactose-6-Sulfatase Deficiency, Glycosaminoglycans (GAGS), Heparan Sulfate, Hunter Syndrome, Hurler Syndrome,
Hurler/Scheie Syndrome, Iduronate Sulfatase Deficiency, Iduronidase Deficiency, Keratan Sulfate, Maroteaux Lamy Syndrome, Morquio A, Morquio B, MPS, MPS I, MPS II, MPS III, MPS IVA, MPS IVB, MPS VI, MPS VII, Mucopolysaccharides, Multiple Sulfatase Deficiency, Sanfilippo Syndrome, Scheie Syndrome, Sly Syndrome
Useful For

Supporting the biochemical diagnosis of one of the mucopolysaccharidoses:  types I, II, III, IV, VI or VII.

ADVISORY INFORMATION:  This test alone is not appropriate for the diagnosis of a specific mucopolysaccharidosis (MPS).  Follow-up enzymatic testing must be performed to confirm a diagnosis of an MPS. 

Specimen Requirements
Specimen Type Preferred Container/Tube Acceptable Container/Tube Specimen Volume Specimen Minimum Volume
(allows for 1 repeat)
Pediatric Minimum Volume
(no repeat)
Urine​ ​Plastic, 5 mL urine tube 2 mL ​3 mL
Collection Processing Instructions
1. Patient's age is required.
2. Reason for referral is required.
3. DO NOT administer low-molecular weight heparin prior to collection.
4. Collect a random urine specimen (early morning preferred). 
Specimen Stability Information
Specimen Type Temperature Time
​Urine ​ ​ Refrigerated (preferred) ​21 days
​Frozen​365 days
​Ambient​7 days
Performing Laboratory Information
Performing Location Day(s) Test Performed Analytical Time Methodology/Instrumentation
Mayo Medical Laboratories​ Varies ​8 days
Liquid Chromatography-Tandem Mass Spectrometry (LC-MS/MS)
Reference Lab
Test Information

This test is used to aid in the diagnosis and monitoring of patients with mucopolysacchariodoses (MPS) types I, II, III, IV, VI, and VII.

Accumulation of undegraded glycosaminoglycans (GAGs) leads to progressive cellular dysfunction and results in the typical clinical features seen with this group of disorders.

Dermatan sulfate (DS), heparan sulfate (HS), keratan sulfate (KS) and chondroitin-6-sulfate (C6S) are markers for a subset of MPS.

DS and HS in urine are markers for MPS types I, II, III, VI and VII.

KS in urine is a marker for MPS IVA and MPS IVB. 

C6S in urine is a marker for MPS IVA and MPS VII.

Reference Range Information

DERMATAN SULFATE

< or = 1.00 mg/mmol creatinine

 

HEPARAN SULFATE

< or =4 years: < or = 0.50 mg/mmol creatinine

> or =5 years: < or = 0.25 mg/mmol creatinine

 

CHONDROITIN-6 SULFATE

< or =24 months: < or = 10.00 mg/mmol creatinine

25 months-10 years: < or = 2.50 mg/mmol creatinine

> or =11 years: < or = 1.50 mg/mmol creatinine

 

KERATAN SULFATE

< or =12 months: < or = 2.00 mg/mmol creatinine

13-24 months: < or = 1.50 mg/mmol creatinine

25 months-4 years: < or = 1.00 mg/mmol creatinine

5-18 years: < or = 0.50 mg/mmol creatinine

> or =19 years: < or = 0.30 mg/mmol creatinine

 

Interpretation

Elevations of dermatan sulfate and/or heparan sulfate and/or keratan sulfate and/or chondroitin-6-sulfate may be indicative of one of the mucopolysaccharidoses: types I, II, III, IV, VI, or VII.

Elevations of any or all sulfate species may be indicative of multiple sulfatase deficiency or mucolipidosisII/III.

Rarely, an elevation of keratan sulfate may be indicative of alpha-fucosidosis.

Outreach CPTs
CPT Modifier
(if needed)
Quantity Description Comments
​83864 ​1
​82570​1
Synonyms/Keywords
​Arylsulfatase B Deficiency, Beta-Galactosidase Deficiency, Beta-Glucoronidase Deficiency, Chondroitin-6-Sulfate, Dermatan Sulfate,
GAGS (Glycosaminoglycans), Galactose-6-Sulfatase Deficiency, Glycosaminoglycans (GAGS), Heparan Sulfate, Hunter Syndrome, Hurler Syndrome,
Hurler/Scheie Syndrome, Iduronate Sulfatase Deficiency, Iduronidase Deficiency, Keratan Sulfate, Maroteaux Lamy Syndrome, Morquio A, Morquio B, MPS, MPS I, MPS II, MPS III, MPS IVA, MPS IVB, MPS VI, MPS VII, Mucopolysaccharides, Multiple Sulfatase Deficiency, Sanfilippo Syndrome, Scheie Syndrome, Sly Syndrome
Ordering Applications
Ordering Application Description
​Centricity ​Mucopolysac, Qnt
​Cerner ​Mucopolysac, Qnt
​COM​Mucopolysaccharides, Qnt, Rand
If the ordering application you are looking for is not listed, contact your local laboratory for assistance.
Specimen Requirements
Specimen Type Preferred Container/Tube Acceptable Container/Tube Specimen Volume Specimen Minimum Volume
(allows for 1 repeat)
Pediatric Minimum Volume
(no repeat)
Urine​ ​Plastic, 5 mL urine tube 2 mL ​3 mL
Collection Processing
1. Patient's age is required.
2. Reason for referral is required.
3. DO NOT administer low-molecular weight heparin prior to collection.
4. Collect a random urine specimen (early morning preferred). 
Specimen Stability Information
Specimen Type Temperature Time
​Urine ​ ​ Refrigerated (preferred) ​21 days
​Frozen​365 days
​Ambient​7 days
Useful For

Supporting the biochemical diagnosis of one of the mucopolysaccharidoses:  types I, II, III, IV, VI or VII.

ADVISORY INFORMATION:  This test alone is not appropriate for the diagnosis of a specific mucopolysaccharidosis (MPS).  Follow-up enzymatic testing must be performed to confirm a diagnosis of an MPS. 

Reference Range Information

DERMATAN SULFATE

< or = 1.00 mg/mmol creatinine

 

HEPARAN SULFATE

< or =4 years: < or = 0.50 mg/mmol creatinine

> or =5 years: < or = 0.25 mg/mmol creatinine

 

CHONDROITIN-6 SULFATE

< or =24 months: < or = 10.00 mg/mmol creatinine

25 months-10 years: < or = 2.50 mg/mmol creatinine

> or =11 years: < or = 1.50 mg/mmol creatinine

 

KERATAN SULFATE

< or =12 months: < or = 2.00 mg/mmol creatinine

13-24 months: < or = 1.50 mg/mmol creatinine

25 months-4 years: < or = 1.00 mg/mmol creatinine

5-18 years: < or = 0.50 mg/mmol creatinine

> or =19 years: < or = 0.30 mg/mmol creatinine

 

Interpretation

Elevations of dermatan sulfate and/or heparan sulfate and/or keratan sulfate and/or chondroitin-6-sulfate may be indicative of one of the mucopolysaccharidoses: types I, II, III, IV, VI, or VII.

Elevations of any or all sulfate species may be indicative of multiple sulfatase deficiency or mucolipidosisII/III.

Rarely, an elevation of keratan sulfate may be indicative of alpha-fucosidosis.

For more information visit:
Performing Laboratory Information
Performing Location Day(s) Test Performed Analytical Time Methodology/Instrumentation
Mayo Medical Laboratories​ Varies ​8 days
Liquid Chromatography-Tandem Mass Spectrometry (LC-MS/MS)
Reference Lab
For billing questions, see Contacts
Outreach CPTs
CPT Modifier
(if needed)
Quantity Description Comments
​83864 ​1
​82570​1
For most current information refer to the Marshfield Laboratory online reference manual.