Ordering Recommendation

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

Mnemonic
KS U MS
Methodology

Quantitative Liquid Chromatography/Tandem Mass Spectrometry

Performed

Varies

Reported

3-10 days

New York DOH Approval Status
Specimens from New York clients will be sent out to a New York DOH approved laboratory, if possible.
Specimen Required
Patient Preparation

Morning void is preferred.

Collect

Urine.

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.

Remarks
Stability

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

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).

Compliance Category

Laboratory Developed Test (LDT)

Note
Hotline History
N/A
CPT Codes

83864

Components
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
* 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.
Aliases
  • MPS IVa
Keratan Sulfate, Quantitative by LC-MS/MS, Urine