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Laboratory: Clinical Biochemistry  (METABOLIC)
Test Name:
Test Code: MIS8
Clinical Information:
Alternate Name(s): 
Hex4 Glucotetrasaccharide
Hexose Tetrasaccharide
Pompe Urine Biomarker
Glycogen Storage Disease type II Biomarker
Description:  Measurement of urine hexose tetrasaccharides (Hex4) by UPLC-MSMS
Test Indications: The urine Hex4 assay is used to monitor patients with Pompe Disease (GSD II) on enzyme replacement therapy.
Test Approval Requirements: F260-11-02 Biochemistry Approval for Test Referral Form
Patient preparation instructions: Prepandrial or fasting samples are recommended to avoid dietary artifacts and enhance sensitivity.
Collection Devices:
Specimen Required:
Urine: 1.0 mL
Pediatric Urine: 0.25 mL

Collection Information: No preservative
Special Processing: Store at -20 C and ship frozen.
Specimen Stability:
Ambient: 4 hours
Refrigerated: 24 hours
Frozen: recommended

Shipping & Storage: Send frozen to Metabolic Lab at HSC with the original requisition or clear copy.
Referred Out Location: Biochemical Genetics Lab at Duke University
Test Code: LAB6422
Reference Values:
Method of Analysis: UPLC-MSMS
Within 3 Weeks
See Also:
More Information:
Additional Information: Hex4 may be elevated in a number of pathological and physiological conditions and is not specific to Pompe disease.