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).

Mnemonic
A-I LEUK
Methodology

Quantitative Fluorometry

Performed

Varies

Reported

3-10 days

New York DOH Approval Status
This test is New York DOH approved.
Specimen Required
Patient Preparation
Collect

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.

Remarks

Additional information is required: Clinical Indication for testing.

Stability

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

Reference Interval

12 - 65 nmol hydrolyzed/hr/mg protein

Interpretive Data

Refer to report.

Compliance Category

Laboratory Developed Test (LDT)

Note
Hotline History
N/A
CPT Codes

82657

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