Test Code OXYBS Oxysterols, Blood Spot
Useful For
Investigating a possible diagnosis of Niemann-Pick disease types A, B, or C using blood spot specimens
Monitoring of individuals with Niemann-Pick disease type C
This test is not useful for the identification of carriers.
Special Instructions
Method Name
Liquid Chromatography Tandem Mass Spectrometry (LC-MS/MS)
Reporting Name
Oxysterols, BSSpecimen Type
Whole bloodOrdering Guidance
This test's clinical sensitivity and specificity for the identification of Niemann-Pick type C (NPC) is 75% and 89%, respectively. If NPC is strongly suspected, the recommended test is OXNP / Oxysterols, Plasma.
This test is also available as a part of a panel; see HSMBS / Hepatosplenomegaly Panel, Blood Spot. If this test (OXYBS) is ordered with either GPSY / Glucopsychosine, Blood Spot or CTXBS / Cerebrotendinous Xanthomatosis, Blood Spot, the individual tests will be canceled and HSMBS ordered.
Specimen Required
Supplies:
-Card-Blood Spot Collection (Filter Paper) (T493)
-Card-Postmortem Screening (Filter Paper) (T525)
Container/Tube:
Preferred: Blood Spot Collection Card (Filter Paper)
Acceptable: Whatman Protein Saver 903 filter paper, PerkinElmer 226 filter paper, Munktell filter paper, Postmortem Screening Card or collected with EDTA, sodium heparin, lithium heparin, or ACD B-containing devices
Specimen Volume: 2 Blood spots
Collection Instructions:
1. Let blood dry completely on the filter paper at ambient temperature in a horizontal position for a minimum of 3 hours.
2. At least 1 spot should be complete, (ie, unpunched).
3. Do not expose specimen to heat or direct sunlight.
4. Do not stack wet specimens.
5. Keep specimen dry.
Additional Information:
1. For collection instructions, see Blood Spot Collection Instructions.
2. For collection instructions in Spanish, see Blood Spot Collection Card-Spanish Instructions (T777).
3. For collection instructions in Chinese, see Blood Spot Collection Card-Chinese Instructions (T800).
Specimen Minimum Volume
1 Blood spot
Specimen Stability Information
Specimen Type | Temperature | Time | Special Container |
---|---|---|---|
Whole blood | Refrigerated (preferred) | 10 days | FILTER PAPER |
Frozen | 59 days | FILTER PAPER | |
Ambient | 10 days | FILTER PAPER |
Reference Values
CHOLESTANE-3-BETA,5-ALPHA,6-BETA-TRIOL
Cutoff: ≤0.800 nmol/mL
LYSO-SPHINGOMYELIN
Cutoff: ≤0.100 nmol/mL
Day(s) Performed
Tuesday
Report Available
3 to 9 daysPerforming Laboratory
Mayo Clinic Laboratories in RochesterTest Classification
This test was developed and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. It has not been cleared or approved by the US Food and Drug Administration.CPT Code Information
82542
LOINC Code Information
Test ID | Test Order Name | Order LOINC Value |
---|---|---|
OXYBS | Oxysterols, BS | 92741-8 |
Result ID | Test Result Name | Result LOINC Value |
---|---|---|
36762 | Interpretation (OXYBS) | 59462-2 |
36760 | Cholestane-3beta,5alpha,6beta-triol | 92757-4 |
36761 | Lyso-sphingomyelin | 92749-1 |
36763 | Reviewed By | 18771-6 |
Testing Algorithm
If the patient has abnormal newborn screening results for Niemann- Pick disease, refer to the appropriate American College of Medical Genetics and Genomics Newborn Screening ACT Sheet.(1)
For more information seeNewborn Screen Follow-up for Acid Sphingomyelinase Deficiency
Forms
If not ordering electronically, complete, print, and send a Biochemical Genetics Test Request (T798) with the specimen.