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HelpTest Code LAB3201 Iduronate sulfatase (MPS II; Hunter)
Clinical System Name
Iduronate Sulfatase
Synonyms
Hunter
MPS II
IDS
Sample Requirements
Specimen: Whole Blood
Container(s): Dark Green/Sodium Heparin Tube; Red top
Preferred Vol:1.0
Minimum Vol: 0.5 mL
Processing Instructions
Spin: Y
Aliquot:Y
Temp:Freeze
Storage location: BCG -20o C
Processing on weekends: Freeze serum or plasma at -20o C
Off-site collection:Spin and freeze plasma or serum. Ship frozen.
Stability
| Specimen Type | Temperature | Time |
|---|---|---|
| Whole Blood | Room temp/refrig |
2 days |
| Plasma | Room temp/refrig | 2 days |
| Plasma | Frozen | 1 year |
Availability
| STAT | Performed | TAT |
|---|---|---|
| N | Weekly |
7-10 days |
Performing Laboratory
Seattle Children's Laboratory
Department
Department: Biochemical Genetics
Phone Number: 206-987-2216
CPT Codes
82657
Methodology
Method: 4 MU Fluorometric Assay
Analytical Volume:
Limitations:
Reference Range
| Specimen Type | Normal Range (nmol/4hr/mL) |
|
Plasma |
155 - 1082 |
Description
Fluorometric enzyme test for iduronate sulfatase for diagnosis of MPS II (Hunter).
Requisition
Biochemical Genetics Requisition
On the requisition include clinical information needed for appropriate interpretation. (Age, gender, drug therapy and family history)
Clinical Utility
This test is used for work up of patients with a suspected mucopolysaccharide disorder, and elevated excretion of glycosaminoglycans (heparan and dermatan sulfates). A deficiency of iduronate sulfatase supports a diagnosis of MPS type II (Hunter), an X-linked MPS storage disorder. This test is not appropriate for determining carrier status or for prenatal diagnosis.