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Test Code LAB11193 Biotinidase, Serum

Reporting Name

Biotinidase, S

Useful For

Preferred test for the diagnosis of biotinidase deficiency


Follow-up testing for certain organic acidurias

Performing Laboratory

Mayo Clinic Laboratories in Rochester

Specimen Type


Ordering Guidance

Second-tier molecular testing is available, see BTDZ / Biotinidase Deficiency, BTD Full Gene Analysis Varies.

Specimen Required

Collection Container/Tube:

Preferred: Red top

Acceptable: Serum gel

Submission Container/Tube: Plastic vial

Specimen Volume: 1 mL

Collection Instructions: Centrifuge immediately and aliquot serum into plastic vial.

Specimen Minimum Volume

0.5 mL

Specimen Stability Information

Specimen Type Temperature Time Special Container
Serum Frozen (preferred) 21 days
  Refrigerated  5 days

Reference Values

3.5-13.8 U/L

Day(s) Performed

Wednesday, Friday

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
BIOTS Biotinidase, S 1982-8


Result ID Test Result Name Result LOINC Value
50666 Specimen 31208-2
50667 Specimen ID 57723-9
50668 Source 31208-2
50669 Order Date 82785-7
50670 Reason For Referral 42349-1
50671 Method 85069-3
50672 Biotinidase, S 1982-8
50673 Interpretation 59462-2
50674 Amendment 48767-8
50675 Reviewed By 18771-6
50676 Release Date 82772-5

Report Available

4 to 8 days

Reject Due To

Gross hemolysis Reject
Gross lipemia OK
Gross icterus OK

Method Name



1. New York Clients-Informed consent is required. Document on the request form or electronic order that a copy is on file. The following documents are available in Special Instructions:

-Informed Consent for Genetic Testing (T576)

-Informed Consent for Genetic Testing-Spanish (T826)

2. Biochemical Genetics Patient Information (T602) in Special Instructions.

3. If not ordering electronically, complete, print, and send an Inborn Errors of Metabolism Test Request (T798) with the specimen.