Ordering Recommendation

Screening and monitoring of patients with suspected Morquio syndrome (MPS IV), in conjunction with other mucopolysaccharides screen tests.

New York DOH Approval Status

This test is New York state approved.

Specimen Required

Patient Preparation

Morning void is preferred.



Specimen Preparation

Transfer 2 mL urine to ARUP Standard Transport Tube and freeze immediately. (Min: 1 mL)

Storage/Transport Temperature

CRITICAL FROZEN. Separate specimens must be submitted with multiple tests are ordered.

Unacceptable Conditions

Specimens containing preservatives.


Ambient: Unacceptable; Refrigerated: Unacceptable; Frozen: 1 month


Liquid Chromatography-Tandem Mass Spectrometry




3-10 days

Reference Interval

Age Total Keratan Sulfate (ug/mg CRT)
0-11 months 5.65 - 20.73
1 year - 5 years 1.75 - 6.81
6 years - 15 years 0.67 - 4.16
16 years and older 0.29 - 0.85

Interpretive Data

Refer to report.
Keratan Sulfate, Total is calculated as the sum of the two disaccharides, LacNAc1 and LacNAc2, per milligram of creatinine (CRT).

This test was developed and its performance characteristics determined by ARUP Laboratories. It has not been cleared or approved by the US Food and Drug Administration. This test was performed in a CLIA certified laboratory and is intended for clinical purposes.

Compliance Category

Laboratory Developed Test (LDT)


Hotline History


CPT Codes



Component Test Code* Component Chart Name LOINC
2012260 Creatinine, Urine 2161-8
2012261 LacNAc1 (S1 monosulfated), Urine
2012262 LacNAc2 (S2 disulfated), Urine
2012263 Keratan Sulfate, Total Urine
2012264 Keratan Sulfate, Urine Interpretation
3005207 EER Keratan Sulfate, Urine 11502-2
* Component test codes cannot be used to order tests. The information provided here is not sufficient for interface builds; for a complete test mix, please click the sidebar link to access the Interface Map.


  • MPS IVa
Keratan Sulfate, Quantitative by LC-MS/MS, Urine