Home
HelpTest Code LAB2818 Arylsulfatase B
Clinical System Name
Arylsulfatase B (MPS VI)
Synonyms
MPS VI
Maroteaux-Lamy
Aryl B
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
CPT Codes
82657
Methodology
Method: Spectrophotometry
Analytical Volume:
Limitations:
Reference Range
| Specimen Type | Normal Range (nmol/hr/mg protein) |
|
WBC |
30-425 |
| FB (Fibroblast) | 22-274 |
Description
Spectrophotometric enzyme assay using p-nitrocatechol sulfate as substrate and detection at 516 nm.
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.
Requisition
Biochemical Genetics Requisition
On the requisition include clinical information needed for appropriate interpretation. (Age, gender, drug therapy and family history)