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Test Code LAB1948 Oligosaccharide Screen, Random, Urine

Important Note

This test has replaced our in-house Oligosaccharide Screen by Thin Layer Chromatography, effective 10/3/2020.

Useful For

Screening for selected oligosaccharidosis

Method Name

Matrix-Assisted Laser Desorption Ionization Time-of-Flight Mass Spectrometry (MALDI-TOF MS)

Reporting Name

Oligosaccharide Screen, U
Seattle Children's Hospital Note:

Clinical System Name: High Resolution Oligosaccharides, Urine

Specimen Type


Ordering Guidance

This is the recommended test when clinical features are suggestive of, or when molecular testing results suggest, an oligosaccharidosis disorder identified by this test.


The recommended screening test for the initial workup of a suspected lysosomal storage disorder, particularly when clinical features are nonspecific, is LSDS / Lysosomal Storage Disorders Screen, Random, Urine.

Necessary Information

1. Patient's age is required.

2. Biochemical Genetics Patient Information (T602) in Special Instructions is recommended. This information aids in providing a more thorough interpretation of results. Send information with specimen.

Specimen Required

Supplies: Urine Tubes, 10 mL (T068)

Collection Container/Tube: Plastic, 10-mL urine tube

Specimen Volume: 8 mL

Pediatric Volume: 2 mL

Collection Instructions:

1. Collect a random urine specimen.

2. No preservative

3. Immediately freeze specimen.

Specimen Minimum Volume

2.5 mL

Specimen Stability Information

Specimen Type Temperature Time Special Container
Urine Frozen (preferred) 365 days
  Refrigerated  15 days
  Ambient  7 days

Reject Due To

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

Reference Values

An interpretive report will be provided.

Method Description

Urine samples are extracted using Oasis HLB and carbograph columns and lyophilized overnight. Oligosaccharides are permethylated, replacing all hydroxy groups (-OH) with methoxy groups (-OCH3) and esterifies carboxyl groups (-COOH to -COOCH3). After permethylation, the tubes are centrifuged, and the supernatant removed from the sodium hydroxide pellet. The supernatant is quenched, neutralized, extracted onto an Oasis HLB column, eluted, and lyophilized again overnight. Specimens are resuspended, mixed with a matrix solution containing 2,5-dihydroxybenzoic acid, spotted onto a MALDI plate, and allowed to air dry. The plate is then analyzed using a matrix-assisted laser desorption/ionization tandem time-of-flight (MALDI TOF/TOF) 5800 Analyzer.(Xia B, Asif G, Arthur L, et al: Oligosaccharide analysis in urine by MALDI-TOF mass spectrometry for the diagnosis of lysosomal storage diseases. Clin Chem. 2013 Sep;59[9]:1357-1368)


Day(s) Performed


Seattle Children's Hospital Note:

TAT: 10 - 17 d

Report Available

8 to 15 days

Performing Laboratory

Mayo Clinic Laboratories in Rochester

Test Classification

This test was developed, and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. This test has not been cleared or approved by the US Food and Drug Administration.

CPT Code Information


LOINC Code Information

Test ID Test Order Name Order LOINC Value
OLIGU Oligosaccharide Screen, U 49284-3


Result ID Test Result Name Result LOINC Value
64889 Oligosaccharide Screen, U 49284-3

Testing Algorithm

Oligosaccharide analysis may be considered in the workup of unexplained refractory epilepsy. For more information see:

-Epilepsy: Unexplained Refractory and/or Familial Testing Algorithm

-Congenital Disorders of Glycosylation: Screening Algorithm.

Seattle Children's Hospital Additional Information:

Aliases: Alpha-Mannosidosis; Alpha-Fucosidosis; Alpha-N-Acetylgalactosaminidase Deficiency; Aspartylglycosaminuria; Beta-galactosidase; Beta-Mannosidosis; CDG IIb; Galactosialidosis; GM1 gangliosidosis; GM2 gangliosidosis; I-Cell Disease; MOGS-CDG; Mucolipidosis II; Mucolipidosis III; Mucopolysaccharidosis IVB; NGLY1-CDG; Oligosaccharidoses; Oligosaccharidosis; OLIGO; Pompe Disease; Pseudo-Hurler Polydystrophy; Sandhoff Disease; Schindler Disease; Sialidosis; UHiRsOlig; Urineoligos