Test Catalog

Test ID: HEX4    
Glucotetrasaccharides, Random, Urine

Testing Algorithm Delineates situations when tests are added to the initial order. This includes reflex and additional tests.

See Newborn Screen Follow-up for Pompe Disease in Special Instructions.

Specimen Type Describes the specimen type validated for testing


Additional Testing Requirements

When requested for the diagnosis of Pompe disease (GSD II), glucotetrasaccharide concentrations in urine need to be interpreted in light of the clinical presentation and other laboratory tests, such as blood creatine kinase, alpha-glucosidase (GAA) activity, and GAA genotype.

Necessary Information

Patient's age and reason for referral are required.

Specimen Required Defines the optimal specimen required to perform the test and the preferred volume to complete testing

Supplies: Aliquot Tube, 5 mL (T465)

Container/Tube: Plastic, 5-mL urine tube

Specimen Volume: 3 mL

Collection Instructions:

1. Collect a random urine specimen.

2. No preservative.

Special Instructions Library of PDFs including pertinent information and forms related to the test


If not ordering electronically, complete, print, and send an Inborn Errors of Metabolism Test Request (T798) with the specimen.

Specimen Minimum Volume Defines the amount of sample necessary to provide a clinically relevant result as determined by the Testing Laboratory

1 mL

Reject Due To Identifies specimen types and conditions that may cause the specimen to be rejected

All specimens will be evaluated at Mayo Clinic Laboratories for test suitability.

Specimen Stability Information Provides a description of the temperatures required to transport a specimen to the performing laboratory, alternate acceptable temperatures are also included

Specimen TypeTemperatureTimeSpecial Container
UrineFrozen (preferred)87 days
 Refrigerated 28 days
 Ambient 14 days