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HelpTest ID BAIPD Bile Acids for Peroxisomal Disorders, Serum
Useful For
Biomarker for peroxisomal biogenesis disorders, such as Zellweger spectrum disorder and single enzyme defects of bile acid synthesis, including D-bifunctional protein deficiency and alpha methyl CoA racemases
Monitoring patients receiving bile acid therapy, such as cholic acid, for liver disease due to peroxisomal biogenesis disorders or single enzyme defects in bile acid synthesis
Method Name
Liquid Chromatography Tandem Mass Spectrometry (LC-MS/MS)
Reporting Name
Bile Acids for Peroxisomal D/O, SSpecimen Type
SerumOrdering Guidance
For assessment of general liver dysfunction in adults or diagnosis or monitoring of intrahepatic cholestasis of pregnancy, order BAFS / Bile Acids, Fractionated and Total, Serum.
Specimen Required
Patient Preparation: Patient must be fasting for 12 to 14 hours.
Collection Container/Tube:
Preferred: Serum gel
Acceptable: Red top
Submission Container/Tube: Plastic vial
Specimen Volume: 0.5 mL
Collection Instructions: Centrifuge and aliquot serum into a plastic vial.
Specimen Minimum Volume
0.3 mL
Specimen Stability Information
| Specimen Type | Temperature | Time | Special Container |
|---|---|---|---|
| Serum | Refrigerated (preferred) | 90 days | |
| Ambient | 90 days | ||
| Frozen | 90 days |
Reference Values
Dihydroxycholestanoic acid: ≤0.10 nmol/mL
Trihydroxycholestanoic acid: ≤1.30 nmol/mL
Total cholic acid: ≤5.00 nmol/mL
Total chenodeoxycholic acid: ≤6.00 nmol/mL
Total ursodeoxycholic acid: ≤2.00 nmol/mL
Total bile acids: ≤19.00 nmol/mL
Day(s) Performed
Monday through Friday
Report Available
3 to 5 daysPerforming 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. 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 |
|---|---|---|
| BAIPD | Bile Acids for Peroxisomal D/O, S | 43130-4 |
| Result ID | Test Result Name | Result LOINC Value |
|---|---|---|
| 41446 | Dihydroxycholestanoic Acid | 53479-2 |
| 41447 | Trihydroxycholestanoic Acid | 38188-9 |
| 41448 | Total Cholic Acid | 30518-5 |
| 41449 | Total Chenodeoxycholic Acid | 30519-3 |
| 41450 | Total Ursodeoxycholic Acid | 55159-8 |
| 41451 | Total Bile Acids | 14628-2 |
Special Instructions
Forms
If not ordering electronically, complete, print, and send a Biochemical Genetics Test Request (T798) with the specimen.