Mucopolysaccharides Quantitative, Urine (Sendout)

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General Information

Lab Name
Lab Code
481
External Test Id
LAB1057
Description

Test Description: Quantitative DMB dye binding test used to screen individuals suspected of having a mucopolysaccharidosis.

Ordering Guidance: This test is suitable for initial assessments where elevated GAG levels along with low enzyme activities strongly suggest MPS. For monitoring of disease progression and treatment efficacy in patients with known MPS IV, refer to separate test Morquio Syndrome (MPS IV) Monitoring, Urine (Sendout).

Ordering Requirements: Include relevant clinical information (drug therapy, family history, etc.) in the Epic order comment to ensure proper interpretation.

References
Forms & Requisitions
Synonyms
Acid Mucopolysaccharides, GAG, Glycosaminoglycans, LAB1057, MPS Quant, U MPS QNT
Components

Interpretation

Method

Dimethylene Blue (DMB) Spectrophotometric Assay

Reference Range
Ref. Range Notes

Reference Ranges:

Age mg/G Creat mg/L
0 - 2 mo < 350 < 60
3 - 6 mo < 250 < 60
7 - 18 mo < 150 < 60
19-24 mo < 125 < 60
> 2 yr < 60 < 60
Interferences and Limitations

Limitations: Elevated protein and bilirubin in urine can cause false elevation.

References

Ordering & Collection

Specimen Type
Urine
Collection

Collect random urine in a sterile, preservative-free, screw-capped container.

Forms & Requisitions
Handling Instructions

Outside Laboratories:

  • Transfer 2 mL urine (1 mL minimum) to a separate, screw-cap urine aliquot, leaving room at the top for expansion.
  • Freeze urine at -20°C while awaiting shipment. Transport on dry ice.
  • Optional: Include relevant clinical information (diet/TPN therapy, drug therapy, family history) in the "Comments" section of the DLMP requisition.

Stability: Frozen (preferred): 3 weeks; Refrigerated: 2 days; Ambient: 24 hrs.

Quantity
Requested: 2 mL urine
Minimum: 1 mL urine

Processing

Receiving Instructions

Transfer 2 mL urine (1 mL minimum) to a separate, screw-cap urine aliquot vial, leaving room at the top for expansion. Freeze urine at -20°C.

Login: SEND1-;FREEZE URINE

  • RSNDTO: SEACHL
  • RSPTYP: URN
  • RTSTRQ: ;Mucopolysaccharides Quant, Urine (SEACHL Test LAB1057)

Sendouts:

  • Order SEACHL Test: LAB1057
  • Complete a SEACHL Biochemical Genetics requisition and select test, "Mucopolysaccharide, Quantitative Urine" under the "Urine" section.
  • If clinical information (drug therapy, family history) is included with the order (optional), print the Epic requisition and send it with the SEACHL form.
  • Send samples on dry ice via DLMP or DE courier.

Stability: Frozen (preferred): 3 weeks; Refrigerated: 2 days; Ambient: 24 hrs.

Misc Sendout

Performance

Lab Department
Frequency
Performed: Twice weekly. Turn Around Time: 7 days from sample receipt at Seattle Children's Hospital Lab.
Available STAT?
No
Performing Location(s)
Sendout Seattle Children's Hospital Laboratories
206-987-2617

4800 Sand Point Way NE
Seattle, WA 98105

Room Number: FB.2.441
Other Locations/Notes

Seattle Children's Hospital Laboratory, Biochemical Genetics: 206-987-2216

Billing & Coding

Billing Comments

CPT: 83864