Urine Hex4

Abbrev Code:HEX4U   
Order Code:LAB6085Order Name:HEX4, Urine
Order Instructions:Order test code 0097103 in ARUP Connect.
Synonyms:Urine Glucose Tetrasaccharide; Pompe Disease; Acid Maltase Deficiency; Urinary Hexose Tetrsaccharide
Methodology:Liquid chromatography/Tandam mass spectrometry (LC/MS/MS)
CPT Codes: 82570 x1, 83789 x1
Turnaround Time:Specimens are sent to reference laboratory Mon-Sat only; results are reported within 10-14 days.
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Urine Collection Procedure

Collection Instructions

Specimen:Urine, random collection
Optimal Volume:Submit entire collection; analysis requires 0.25 mL
Minimum\Peds Volume:0.25 mL
Container:Plastic leakproof container, sterile

Processing and Shipping

Specimen Processing:Aliquot 1 mL, 0.25 mL minimum. Freeze immediately.
Shipping Instructions:Ship on dry ice.
Test Performed at or Referral Lab Lab Sendouts  (Duke University Medical Center: Glycogen Storage Disease Laboratory via ARUP)
Referral Lab number:LAB6422


Reference Range:By report.
Use:As a monitoring tool, urine Hex4 can be used as an indirect measure of the degree of skeletal muscle glycogen clearance in patients with Pompe Disease receiving enzyme replacement therapy.

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