Urine Hex4
Abbrev Code: | HEX4U | ||
Order Code: | LAB6085 | Order 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. | ||
Associated Links: |
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 |
Interpretive
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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