Mucopolysaccharides (MPS) Screen, Urine

Abbrev Code:MMMISC   
Order Code:LAB4909Order Name:Send Outs Misc Test
Synonyms:Glycosaminoglycans (GAGS); Lysosomal Storage Disease; MPS Test; Urine Glycosaminoglycans
Methodology:Liquid chromatography/tandem mass spectrometry (LC-MS/MS)
CPT Codes: 83864 x1
Turnaround Time:Specimens are sent to the reference laboratory Mon-Fri; results are reported within 8-15 days.

This test was developed and its performance characteristics determined by Laboratory Medicine and Pathology, Mayo Clinic. This test has not been cleared by the U.S. Food and Drug Administration.

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Urine Collection Procedure

Collection Instructions

Specimen:Urine, random collection
Optimal Volume:Submit entire collection; analysis requires 2 mL, 1 mL minimum.
Minimum\Peds Volume:1 mL
Container:Plastic leakproof container, sterile
Collection Instructions:Do not administer low-molecular weight heparin prior to collection. Early morning specimen preferred.

Processing and Shipping

Specimen Processing:Mix collection well before aliquot is taken. Aliquot 2 mL, 1 mL minimum. Store refrigerated. Must include reason for testing.
Shipping Instructions:Ship refrigerated.
Stability:7 days at room temperature; 90 days refrigerated; 365 days frozen.
Test Performed at or Referral Lab Lab Sendouts  (Mayo)
Referral Lab number:MPSQU


Reference Range:
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 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
Use:Supporting the biochemical diagnosis of one of the mucopolysaccharidoses; types I, II, III, IV, VI or VII.
Monitoring patients with mucopolysaccharidosis who have had bone marrow transplants or are receiving enzyme therapy.

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