| Laboratory: | Clinical Biochemistry (METABOLIC) |
| Test Name: |
GLUCOSE TETRASACCHARIDE - (U)
Test Code:
MIS8
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| 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: |
Preferred Device:
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| 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 |
| Referral: |
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 |
| Requisition: | |
| Reference Values: |
Method of Analysis: UPLC-MSMS
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| Availability: |
Within 3 Weeks
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| 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.
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