Urine Hex4
| Sunquest Code: | HEX4U | ||
| Epic Code: | LAB6085 | Epic 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) | ||
| 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) |
| 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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