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

Lavender (K2EDTA, K3EDTA), Plain Red, or Serum Separator Tube (SST).

Specimen Preparation

Transfer 500 µL serum or plasma to ARUP Standard transport Tube and freeze immediately. (Min 250 µL)

Storage/Transport Temperature

Critical Frozen

Unacceptable Conditions

Plasma collected in green (heparin) tube. Ambient or Refrigerated Specimens


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

Total Heparan Sulfate, Serum/Plasma 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
3003567 MPS 1/2 Serum/Plasma Interpretation 59462-2
3003570 Total Heparan Sulfate, Serum/Plasma 93725-0
3003571 I0S0 (MPS 1 NRE), Serum/Plasma
3003572 I0S6 (MPS 1 NRE), Serum/Plasma
3003573 I2S0 (MPS 2 NRE), Serum/Plasma
3005211 EER MPS 1/2 Serum/Plasma 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 total and NRE, Serum/Plasma
  • HS non reducing end assay
  • MPS 1
  • MPS 2
  • MPS I
  • MPS II
  • MPS Type 1
  • MPS Type 2
Mucopolysaccharidoses Type 1/2, Total Heparan Sulfate and NRE (Sensi-Pro(R)) Quantitative, Serum or Plasma