Glucotetrasaccharides, Urine GLUTETSACU

Synonyms

HEX4
Glucose tetrasacchrides
Hexose tetrasaccharides
Glc4

Allscripts (AEHR) Order Name

Glucotetrasaccharides, Urine

Sunrise Clinical Manager (SCM) Order Name

Glucotetrasaccharides, Random, Urine

EPIC Order Name

Random Urine, Glucotetrasaccharides

Clinical Info

Diagnosing Pompe disease, when used in conjunction with acid alpha-glucosidase enzyme activity assays and molecular genetic analysis of the GAA gene

Monitoring Pompe patients on enzyme replacement therapy
May support the diagnosis and monitoring of other glycogen storage disorders; however, glucotetrasaccharide (Glc4) excretion appears to be less consistently elevated in glycogen storage disorders other than Pompe disease
This test is not useful for carrier screening

Specimen Type

Urine

Container

Urine Tube

Collection Instructions

Container/Tube: Plastic Urine Collection Tube
Specimen: 3 mL random Urine (1mL min)
Transport Temperature: Refrigerated
Stability: 14 Days Room Temperature
28 Days Refrigerated
87 Days Frozen

Transport Instructions

Refrigerated

Specimen Stability

14 Days Room Temperature
28 Days Refrigerated
87 Days Frozen

Methodology

Liquid Chromatography-Tandem Mass Spectrometry (LC-MS/MS)

Days Performed

Performing Laboratory

Mayo Medical Laboratories

CPT

82542
82570

PDM

215299

Result Interpretation

< or =14 months: < or =14.9 mmol/mol Cr
> or =15 months: < or =4.0 mmol/mol Cr

Forms