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HelpTest Code LAB2818 Arylsulfatase B
Clinical System Name
Arylsulfatase B (MPS VI)
Synonyms
MPS VI
Maroteaux-Lamy
Aryl B
Description
Spectrophotometric enzyme assay using p-nitrocatechol sulfate as substrate and detection at 516 nm.
Sample Requirements
Specimen: Whole Blood
Container(s): Yellow/ACD A or B, Dark Green/Sodium Heparin Tube
Preferred Vol:10.0 mL
Minimum Vol: 6.0 mL
Do NOT use gel separator tubes.
Note: Also acceptable: cultured cells from skin fibroblasts (2 confluent T-25 flasks).
Processing Instructions
Reject due to:
Spin: N
Aliquot:N
Temp:RT
Storage location: Biochemical Genetics Box- RT
Weekend Processing: Contact Chem West (x72565) on dayshift. If Chemistry team is unavailable, sample should be stored in RT Biochemical Genetics box.
Off-site collection: Do not spin! Keep at room temperature. Transport Mon-Thurs at room temperature via overnight shipping.
Stability
| Specimen Type | Temperature | Time |
|---|---|---|
| whole blood | Room temp |
≤24 hrs |
| Refrigerated | <24 hrs |
Samples received > 24 hr after blood collection will still be processsed and test performed if possible.
Availability
| STAT | Performed | TAT |
|---|---|---|
| N | Weekly | 7-10 days |
Contact the Biochemical Genetics Lab for requests outside of stated availability (206)987-2216.
Performing Laboratory
Seattle Children's Laboratory
Department
Department: Biochemical Genetics
Phone Number: 206-987-2216
Methodology
Method: Spectrophotometry
Analytical Volume:
Limitations:
Reference Range
| Specimen Type | Normal Range (nmol/hr/mg protein) |
|
WBC |
30-425 |
| FB (Fibroblast) | 22-274 |
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
For confirmation of Maroteaux-Lamy (MPS VI), an autosomal recessive MPS storage disorder characterized by elevated muopolysaccharide excretion with dermatan sulfate. The clinical manifestations include dysostosis multiplex and corneal clouding, often with normal intelligence.