Ordering Recommendation

Aids in the diagnosis and monitoring of patients with MPS Type 4A or Type 6.

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




Specimen Preparation

Transfer 0.25 mL urine to polypropylene sample tube and freeze immediately. (Min 0.15 mL)

Storage/Transport Temperature

Critical Frozen

Unacceptable Conditions

Specimens containing preservatives or heparin




Ambient: Unacceptable; Refrigerated: Unacceptable; Frozen: 1 year (3 freeze thaw cycles are acceptable)


Liquid Chromatography-Tandem Mass Spectrometry




14-21 days

Reference Interval

By report

Interpretive Data

Refer to report.
Total Chondroitin Sulfate and Dermatan Sulfate, Urine is a sum of the internal disaccharides D0a0, D0a4, and D0a6.
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
3003540 MPS 4A/6 Creatinine, Urine
3003541 MPS 4A/6 Urine Interpretation
3003546 Chondroitin and Dermatan Sulfate, Urine
3003548 a4 (MPS 6 NRE), Urine
3003550 a6 (MPS 4A NRE), Urine
3003551 a4/a6 Ratio, Urine
3005209 EER MPS 4A/6 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.


  • CS non-reducing end assay
  • DS non-reducing end assay
  • MPS 4a
  • MPS 6
  • MPS type 4a
  • MPS type 6
  • Mucopolysaccharides
  • Total Chondroitin Sulfate
  • Total Dermatan Sulfate
Mucopolysaccharidoses Type 4A/6 Total Chondroitin Sulfate and Dermatan Sulfate with NRE (Sensi-Pro(R)) Quantitative, Urine