Ordering Recommendation

Aids in the diagnosis and monitoring of patients with MPS Type 1 or Type 2.

New York DOH Approval Status

This test is not New York state approved. There are no New York state-approved laboratories available. Submit a Non-Permitted Laboratory Request Form (NPL) to the NYDOH prior to collection of specimen. If NPL is approved by NYDOH, and sample is received at ARUP, testing will be performed.

Specimen Required

Patient Preparation

First catch urine is preferred.



Specimen Preparation

Transfer 2mL urine to ARUP Standard transport Tube and freeze immediately. (Min 1 mL)

Storage/Transport Temperature


Unacceptable Conditions

Specimens containing preservatives or heparin




Ambient: Unacceptable; Refrigerated: Unacceptable; Frozen: 1 month (avoid repeated freeze /thaw cycles)


Liquid Chromatography-Tandem Mass Spectrometry




14-21 days

Reference Interval

By report

Interpretive Data

Refer to report.
Total Heparan Sulfate, Urine is a sum of the internal disaccharides D0A0 and D0S0.
NRE = Non Reducing End

This test was developed and its performance characteristics determined by ARUP Laboratories. It has not been cleared or approved by the US Food and Drug Administration. This test was performed in a CLIA certified laboratory and is intended for clinical purposes.

Compliance Category

Laboratory Developed Test (LDT)


Hotline History


CPT Codes



Component Test Code* Component Chart Name LOINC
3003553 MPS 1/2 Creatinine, Urine
3003554 MPS 1/2 Urine Interpretation
3003559 Total Heparan Sulfate, Urine
3003561 I0S0 (MPS 1 NRE), Urine
3003563 I0S6 (MPS 1 NRE), Urine
3003565 I2S0 (MPS 2 NRE), Urine
3005210 EER MPS 1/2 Urine 11502-2
* Component test codes cannot be used to order tests. The information provided here is not sufficient for interface builds; for a complete test mix, please click the sidebar link to access the Interface Map.


  • heparan sulfate
  • HS non reducing end assay
  • MPS 1
  • MPS 2
  • MPS I
  • MPS II
  • MPS Type 1
  • MPS Type 2
  • Urine mucopolysaccharides total and NRE
  • Urine total and NRE mucopolysaccharides
Mucopolysaccharidoses Type 1/2, Total Heparan Sulfate and NRE (Sensi-Pro(R)) Quantitative, Urine