Mucopolysaccharides, Quant Urine

Test Code

MUCQNU

CPT Codes
82664,83864

Preferred Specimen

10 to 15 mL random urine (first morning sample preferred but not essential) or a 5 mL aliquot of 24 hour urine

Minimum Volume

3 to 5 mL random urine or an aliqot of 24 hour urine

Instructions

List clinical indication, and all medications from previous 48 hr.

Transport Container

Sterile urine container

Transport Temperature

Frozen

Specimen Stability

Room Temperature: Unacceptable
Refrigerated: 4 hours
Frozen:

Methodology

Colorimetric – quantitative urine mucopolysaccharides

Gel electrophoresis – qualitative analysis of urine mucopolysaccharides including chondroitin sulfate, dermatan sulfate, heparan sulfate, and keratan sulfate.

Setup Schedule

Set up every two weeks at OHSU Laboratories

Report Available

Report available in 5 to 14 days

Clinical Significance

Mucopolysaccharides (also known as glycosaminoglycans (GAGs)) are excreted in excessive amount and in unusual patterns in several lysosomal storage disorders involving mucopolysaccharide metabolism such as Hurler, Hunter, Sanfillipo, Morquio, or Maroteaux-Lamy syndromes. Individuals with these disorders may present with skeletal abnormalities, short stature, coarse facial features, hirsutism, corneal clouding, deafness, cardiac valve disease, hepatosplenomegaly, umbilical and inguinal hernias, developmental regression, and behavioral disturbances. This assay combines quantitative measurement of urinary mucopolysaccharide excretion and qualitative analysis of the types of mucopolysaccharides excreted to determine the most likely lysosomal storage disease in a patient.

The CPT Codes provided in this document are based on AMA guidelines and are for informational purposes only. CPT coding is the sole responsibility of the billing party. Please direct any questions regarding coding to the payor being billed. Any Profile/panel component may be ordered separately. Reflex tests are performed at an additional charge.