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Test ID KSQNU Keratan Sulfate Quantitative, Urine

Useful For

Monitoring the pharmacodynamic effects of enzyme replacement therapy for Morquio A (MPS IVA)


Morquio syndrome (MPSIV) is caused by a deficiency in the keratan degradation pathway and consists of types A and B.


Individuals with Morquio syndrome excrete keratan sulfate in their urine.


Enzyme replacement therapy (ERT) is available for Morquio A.


Measuring keratan sulfate in the urine of Morquio A patients undergoing ERT can be an appropriate tool for monitoring the pharmacodynamic effects of treatment

Method Name

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

Reporting Name

Keratan Sulfate Quantitative, U

Specimen Type


Necessary Information

Patient’s age is required.

Specimen Required

Supplies: Plastic, Urine Tubes, 10 mL (T068)

Specimen Volume: 1 mL

Collection Instructions:

1. Collect a random urine collection (early morning preferred).

2. Immediately freeze specimen.

Specimen Minimum Volume

0.5 mL

Specimen Stability Information

Specimen Type Temperature Time
Urine Frozen 365 days

Reference Values

0-12 months: ≤2.50 mg/mmol creatinine

13-24 months: ≤2.00 mg/mmol creatinine

25 months-4 years: ≤1.50 mg/mmol creatinine

5-10 years: ≤1.00 mg/mmol creatinine

11-18 years: ≤0.80 mg/mmol creatinine

>18 years: ≤ 0.50 mg/mmol creatinine

Day(s) and Time(s) Performed


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 U.S. Food and Drug Administration.

CPT Code Information


LOINC Code Information

Test ID Test Order Name Order LOINC Value
KSQNU Keratan Sulfate Quantitative, U 92806-9


Result ID Test Result Name Result LOINC Value
64712 Keratan Sulfate Quantitative, U 92806-9


Biochemical Genetics Patient Information (T602) in Special Instructions