Hexose Tetrasaccharide (Hex4) Assay, Urine (Sendout)

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General Information

Lab Name
Lab Code
376
Description

Test Information: Urine Hex4 can be used as a biomarker for glycogen storage in skeletal and cardiac muscle. This test may be useful for aiding in the diagnosis of Pompe disease or monitoring patients on enzyme replacement therapy.

Ordering Requirements: A completed Duke Biochemical Biochemical Genetics Request Form should accompany all submissions.

Forms & Requisitions
Synonyms
Glc4, Glucose tetrasaccharide urine, hexose tetrasaccharide urine, Pompe urine biomarker
Components

Interpretation

Method

Ultra Performance Liquid chromatography-tandem mass spectrometry (UPLC-MS/MS)

Reference Range

Ordering & Collection

Specimen Type
Urine
Collection

Collect random urine in a screw-cap, preservative-free container.

Collection Note: Preprandial or fasting samples are preferred to avoid dietary artifacts and enhance sensitivity.

Forms & Requisitions
Handling Instructions

Outside Laboratories:

  • Transfer 1 mL urine to a separate aliquot and freeze at -20°C while awaiting shipment. Transport on dry ice.
  • Clients do not need to complete the Duke requisition.

Stability: Frozen: Required; Refrigerated: 24 hours; Ambient: 4 hours.

Quantity
Requested: 1 mL Urine
Minimum: 0.3 mL Urine

Processing

Receiving Instructions

Transfer 1 mL urine to a screw-capped plastic urine aliquot and freeze at -20°C.

Login: SEND1-;FREEZE

  • RSNDT1: DUKBGL
  • RSTYP1: URN
  • RTSRQ1: ;HEX4, Urine

Sendouts:

  • Order Duke Biochemical Genetics Test: HEX4.
  • Request form may be completed by Sendouts if not completed by the provider. Affix "Bill to" and "Send Results To" DLMP Account labels to the requisition.
  • Ship samples Monday - Thursday only with sufficient dry ice to ensure that they are frozen upon arrival at Duke.

Stability: Frozen: Required; Refrigerated: 24 hours; Ambient: 4 hours.

Misc Sendout

Performance

Lab Department
Frequency
Performed: Once a week.
Available STAT?
No
Performing Location(s)
Sendout Duke University Hospital Biochemical Genetics Laboratory
919-549-0445

801 Capitola Drive, Suite 6
Durham, NC 27713

Billing & Coding

Billing Comments

CPTs: 82570, 82542