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Test Code LAB2841 Beta Glucuronidase

Clinical System Name

Beta Glucuronidase


B Glucuro


Sly Syndrome



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


Note: Also acceptable: cultured cells from skin fibroblasts (2 confluent T-25 flasks)

Do NOT use gel separator tubes.


Processing Instructions

Reject due to:

Spin: N



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.


Specimen Type Temperature Time
  Room temp

≤24 hrs

  Refrigerated < 24 hrs

Samples received > 24 hr after blood collection will still be processsed and test performed if possible.


STAT Performed TAT
N Weekly 7-10 days


Performing Laboratory

Seattle Children's Laboratory    


Department:  Biochemical Genetics

Phone Number: 206-987-2216



CPT Codes



Method: 4 MU fluorometric assay

Analytical Volume:


Reference Range


Specimen Type Normal Range (nmol/hr/mg protein)


165 - 585
FB (Fibroblast) 66 - 186



Fluorometric enzyme test for beta-glucuronidase for diagnosis of MPS VII (Sly syndrome).

Clinical Utility

Sly syndrome (MPS VII) is a lysosomal storage disorder caused by a deficiency of the enzyme beta-glucuronidase. Deficiency of the beta-glucuronidase enzyme leads to accumulation of heparan, chondroitin and dermatan sulfate in multiple tissues and can cause coarse facial features, corneal clouding, cardiac involvement, skeletal dysplasia, joint stiffness and developmental delay. Age of onset and clinical severity is variable.


This test is indicated for individuals with elevated excretion of glycosaminoglycan,  who have had MPS electrophoresis indicating excretion of heparan and dermatan sulfate. This test is not appropriate for determining carrier status or for prenatal diagnosis.


Biochemical Genetics Requisition


On the requisition include clinical information needed for appropriate interpretation. (Age, gender, drug therapy and family history)