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26456 Oligosaccharide Screen, Random, Urine (OLIGU)

Oligosaccharide Screen, Random, Urine (OLIGU)
Test Code: OLIGUSO
Synonyms/Keywords

Oligosaccharidosis

Oligosaccharidoses

OLIGO

Alpha-Mannosidosis

Alpha-Fucosidosis

Alpha-N-Acetylgalactosaminidase Deficiency

Aspartylglycosaminuria

Galactosialidosis

GM1 gangliosidosis

GM2 gangliosidosis

I-Cell Disease

Mucolipidosis II

Mucolipidosis III

Pompe Disease

Pseudo-Hurler Polydystrophy

Sandhoff Disease

Schindler Disease

Sialidosis

Beta-Mannosidosis

Mucopolysaccharidosis IVB

Urineoligos

Beta-galactosidase

MOGS-CDG

NGLY1-CDG

CDG IIb

Fucosidosis

Mannosidosis

Urine oligos

Urine oligosaccharides

Useful For

Screening for selected oligosaccharidosis.

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.

Specimen Requirements
Fasting RequiredSpecimen TypePreferred Container/TubeAcceptable Container/TubeSpecimen VolumeSpecimen Minimum Volume
(allows for 1 repeat)
Pediatric Minimum Volume
(no repeat)
​Urine, Random​Plastic, 10-mL urine tube​8 mL​2.5 mL​2 mL
Collection Processing Instructions

1. Collect a random urine specimen.

2. No preservative

3. Immediately freeze specimen.

Necessary Information

1. Patient's age is required.

2. Biochemical Genetics Patient Information (T602)

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

Specimen Stability Information
Specimen TypeTemperatureTime
​Urine, Random ​ ​​Frozen (preferred)​365 days
​Refrigerated​15 days
​Ambient​7 days
Rejection Criteria

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

Interference

This test may give false-negative results, especially in older patients with mild clinical presentations.

This test may give false-positive results for Pompe disease, especially in pediatric patients on infant formula.

Performing Laboratory Information
Performing LocationDay(s) Test PerformedAnalytical TimeMethodology/Instrumentation
Mayo Clinic Laboratories​​Monday​8 to 15 days​Matrix-Assisted Laser Desorption Ionization Time-of-Flight Mass Spectrometry (MALDI-TOF MS)
Reference Lab
Test Information

The oligosaccharidoses (glycoproteinoses) are a subset of lysosomal storage disorders (LSD) caused by the deficiency of any one of the lysosomal enzymes involved in the degradation of complex oligosaccharide chains. They are characterized by the abnormal accumulation of incompletely degraded oligosaccharides in cells and tissues and the corresponding increase of related free oligosaccharides in the urine. Clinical diagnosis can be difficult due to the similarity of clinical features across disorders and their variable severity. Clinical features can include bone abnormalities, coarse facial features, corneal cloudiness, organomegaly, muscle weakness, hypotonia, developmental delay, and ataxia. Age of onset ranges from early infancy to adult and can even present prenatally.

The oligosaccharidoses and other storage disorders detected by this assay include alpha-mannosidosis, beta-mannosidosis, aspartylglucosaminuria, fucosidosis, Schindler disease, GM1 gangliosidosis, Sandhoff disease, sialidosis, galactosialidosis, mucolipidoses types II and III, mucopolysaccharidosis IVA (Morquio A), mucopolysaccharidosis IVB (Morquio B), and Pompe disease (see table). Additional conditions that may be picked up by this test include other mucopolysaccharidoses, Gaucher disease, Krabbe disease, and some congenital disorders of glycosylation (PMM2, NGLY1, MOGS, ALG1, MAN1B1).

Genetics Test Information:

Oligosaccharidoses are characterized by the abnormal accumulation of incompletely degraded oligosaccharides in cells and tissues and the corresponding increase of related free oligosaccharides in the urine.

Clinical features of oligosaccharidoses often overlap; therefore, urine screening is an important tool in the initial workup for these disorders.

Enzyme or molecular analysis is required to make a definitive diagnosis.

Reference Range Information

An interpretive report will be provided.

Interpretation

This is a screening test; not all oligosaccharidoses are detected. The resulting excretion profile may be characteristic of a specific disorder; however, abnormal results require confirmation by enzyme assay or molecular genetic testing.

When abnormal results are detected with characteristic patterns, a detailed interpretation is given, including an overview of results and significance, a correlation to available clinical information, elements of differential diagnosis, recommendations for additional confirmatory studies (enzyme assay, molecular genetic analysis).

Outreach CPTs
CPTModifier
(if needed)
QuantityDescriptionComments
​84377​1Sugars, multiple qualitative, each specimen
Synonyms/Keywords

Oligosaccharidosis

Oligosaccharidoses

OLIGO

Alpha-Mannosidosis

Alpha-Fucosidosis

Alpha-N-Acetylgalactosaminidase Deficiency

Aspartylglycosaminuria

Galactosialidosis

GM1 gangliosidosis

GM2 gangliosidosis

I-Cell Disease

Mucolipidosis II

Mucolipidosis III

Pompe Disease

Pseudo-Hurler Polydystrophy

Sandhoff Disease

Schindler Disease

Sialidosis

Beta-Mannosidosis

Mucopolysaccharidosis IVB

Urineoligos

Beta-galactosidase

MOGS-CDG

NGLY1-CDG

CDG IIb

Fucosidosis

Mannosidosis

Urine oligos

Urine oligosaccharides

Ordering Applications
Ordering ApplicationDescription
COM​​Oligosaccharide Screen, Random, Urine (OLIGU)
​Cerner​Oligosaccharide Screen, Random, Urine (OLIGU)
If the ordering application you are looking for is not listed, contact your local laboratory for assistance.
Specimen Requirements
Fasting RequiredSpecimen TypePreferred Container/TubeAcceptable Container/TubeSpecimen VolumeSpecimen Minimum Volume
(allows for 1 repeat)
Pediatric Minimum Volume
(no repeat)
​Urine, Random​Plastic, 10-mL urine tube​8 mL​2.5 mL​2 mL
Collection Processing

1. Collect a random urine specimen.

2. No preservative

3. Immediately freeze specimen.

Necessary Information

1. Patient's age is required.

2. Biochemical Genetics Patient Information (T602)

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

Specimen Stability Information
Specimen TypeTemperatureTime
​Urine, Random ​ ​​Frozen (preferred)​365 days
​Refrigerated​15 days
​Ambient​7 days
Rejection Criteria

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

Interference

This test may give false-negative results, especially in older patients with mild clinical presentations.

This test may give false-positive results for Pompe disease, especially in pediatric patients on infant formula.

Useful For

Screening for selected oligosaccharidosis.

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.

Reference Range Information

An interpretive report will be provided.

Interpretation

This is a screening test; not all oligosaccharidoses are detected. The resulting excretion profile may be characteristic of a specific disorder; however, abnormal results require confirmation by enzyme assay or molecular genetic testing.

When abnormal results are detected with characteristic patterns, a detailed interpretation is given, including an overview of results and significance, a correlation to available clinical information, elements of differential diagnosis, recommendations for additional confirmatory studies (enzyme assay, molecular genetic analysis).

For more information visit:
Performing Laboratory Information
Performing LocationDay(s) Test PerformedAnalytical TimeMethodology/Instrumentation
Mayo Clinic Laboratories​​Monday​8 to 15 days​Matrix-Assisted Laser Desorption Ionization Time-of-Flight Mass Spectrometry (MALDI-TOF MS)
Reference Lab
For billing questions, see Contacts
Outreach CPTs
CPTModifier
(if needed)
QuantityDescriptionComments
​84377​1Sugars, multiple qualitative, each specimen
For most current information refer to the Marshfield Laboratory online reference manual.