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22252 Biotinidase, Serum (BIOTS)

Biotinidase, Serum (BIOTS)
Test Code: BIOTSSO
Synonyms/Keywords
Biotin-amide amidohydrolase, Biotinidase deficiency, BTD
Useful For
​Preferred test for diagnosing biotinidase deficiency
 
Follow-up testing for certain organic acidurias
Specimen Requirements
Specimen Type Preferred Container/Tube Acceptable Container/Tube Specimen Volume Specimen Minimum Volume
(allows for 1 repeat)		 Pediatric Minimum Volume
(no repeat)
​Serum ​Serum Separator Tube (SST)​ Red Top Tube (RTT) 1 mL​ 0.5 mL​ ​
Collection Processing Instructions

Centrifuge immediately and aliquot serum into plastic vial. 

Specimen Stability Information
Specimen Type Temperature Time
Serum​ ​ Frozen (preferred)​ 21 days​
Refrigerated ​ 5 days​
Rejection Criteria
Gross hemolysis
Interference

A diet high in biotin may result in normal clinical presentation even when the biotinidase level is low.

Assay interference may occur for specimens collected when the patient is being treated with a sulfa drug

Performing Laboratory Information
Performing Location Day(s) Test Performed Report Available Methodology/Instrumentation
Mayo Clinic Laboratories​
 Monday, Thursday​
4 to 8 days​
Colorimetric​
Reference Lab
Mayo Clinic Laboratories
Reference Range Information
3.5-13.8 U/L
Interpretation
​The reference range is 3.5 U/L to 13.8 U/L.
 
Partial deficiencies and carriers may occur at the low end of the reference range.
 
Values below 3.5 U/L are occasionally seen in specimens from unaffected patients.
Outreach CPTs
CPT Modifier
(if needed)		 Quantity Description Comments
​82261 ​ ​ ​ ​
Synonyms/Keywords
Biotin-amide amidohydrolase, Biotinidase deficiency, BTD
Ordering Applications
Ordering Application Description
If the ordering application you are looking for is not listed, contact your local laboratory for assistance.
Specimen Requirements
Specimen Type Preferred Container/Tube Acceptable Container/Tube Specimen Volume Specimen Minimum Volume
(allows for 1 repeat)		 Pediatric Minimum Volume
(no repeat)
​Serum ​Serum Separator Tube (SST)​ Red Top Tube (RTT) 1 mL​ 0.5 mL​ ​
Collection Processing

Centrifuge immediately and aliquot serum into plastic vial. 

Specimen Stability Information
Specimen Type Temperature Time
Serum​ ​ Frozen (preferred)​ 21 days​
Refrigerated ​ 5 days​
Rejection Criteria
Gross hemolysis
Interference

A diet high in biotin may result in normal clinical presentation even when the biotinidase level is low.

Assay interference may occur for specimens collected when the patient is being treated with a sulfa drug

Useful For
​Preferred test for diagnosing biotinidase deficiency
 
Follow-up testing for certain organic acidurias
Reference Range Information
3.5-13.8 U/L
Interpretation
​The reference range is 3.5 U/L to 13.8 U/L.
 
Partial deficiencies and carriers may occur at the low end of the reference range.
 
Values below 3.5 U/L are occasionally seen in specimens from unaffected patients.
For more information visit:
Performing Laboratory Information
Performing Location Day(s) Test Performed Report Available Methodology/Instrumentation
Mayo Clinic Laboratories​
 Monday, Thursday​
4 to 8 days​
Colorimetric​
Reference Lab
Mayo Clinic Laboratories
For billing questions, see Contacts
Outreach CPTs
CPT Modifier
(if needed)		 Quantity Description Comments
​82261 ​ ​ ​ ​
For most current information refer to the Marshfield Laboratory online reference manual.