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Test Code IDS Iduronate sulfatase (MPS II; Hunter)

Clinical System Name

Iduronate Sulfatase

Synonyms

Hunter

MPS II

 

Description

Fluorometric enzyme test for iduronate sulfatase for diagnosis of MPS II (Hunter).

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

 

 

 

 

Reference Range

 

Specimen Type Normal Range (nmol/4hr/mL)

Plasma

155 - 1082

 

Methodology

Method: 4 MU Fluorometric Assay

Analytical Volume:

Limitations:

CPT Codes

82657

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.