Ordering Recommendation

Use to diagnose patients for mucopolysaccharidosis type I (also known as MPS I, Hurler, Scheie, and Hurler-Scheie syndromes). To screen for all types of MPS, refer to Mucopolysaccharides Screen - Electrophoresis & Quantitation, Urine (0081352). To monitor glycosaminoglycans (GAGs) in patients previously diagnosed with MPS, refer to Mucopolysaccharides, Quantitative, Urine (0081357).


Quantitative Fluorometry




3-10 days

New York DOH Approval Status

This test is New York DOH approved.

Specimen Required

Patient Preparation

Yellow (ACD Solution B), Lavender (K2EDTA), or Lavender (K3EDTA).

Specimen Preparation

Transport 3 mL whole blood. (Min: 1 mL)

Storage/Transport Temperature

Room Temperature  (Preferred) or Refrigerated

Unacceptable Conditions

Grossly hemolyzed or heparinized specimens.


Additional information is required: Clinical Indication for testing.


Ambient: 3 days; Refrigerated: 3 days; Frozen: Unacceptable

Reference Interval

12 - 65 nmol hydrolyzed/hr/mg protein

Interpretive Data

Refer to report.

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
2011416 Alpha-Iduronidase Activity, Leukocytes 24057-2
2011417 Alpha-Iduronidase, Leukocytes, Interp 48767-8
* 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.


  • Hunter
  • Hunter-Scheie
  • Mucopolysaccharidosis Type I
  • Scheie
Alpha-Iduronidase Enzyme Activity in Leukocytes