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Pathology and Laboratory Medicine
Test Catalogue

​Biotinidase, Quantitative


​Specimen Requirements​

  • ​Serum or Plasma

​Collection Procedure
  • Centrifuge and aliquot serum/plasma within 1 hour of collection.
  • Send to SPH on an ice pack.
  • Freeze serum/plasma immediately upon receipt in lab.
​Required Volume​Optimal Volume: 4 mL whole blood
Minimum/Pediatric Volume: 2 mL whole blood
  • Store frozen serum/plasma at minimum – 20ºC or below
  • Send to referral lab on dry ice 
  • Frozen specimens are stable for 30 days
Grounds for Rejection​

Testing Information​ ​

  • Shipped weekly
Testing Site​
  • ​Referred out by St. Paul's Hospital Laboratory
Results Reporting​
  • Spectrophotmetry
​Clinical Interpretation
  • Results interpretation should be correlated with clinical and other laboratory findings
Alternate Test Names​
  • ​Biotin-amide amidohydrolase 
  • Biotinidase deficiency
​Additional Comments​This test is used to confirm abnormal biotinidase enzyme activities for screen positive newborns
Biotinidase activity level is reported
Individuals with profound biotinidase deficiency contain less than 10% of normal biotinidase activity; individuals with partial biotinidase deficiency contain 10-30% of normal biotinidase activity. Most patients are responsive to biotin and develop normally if treated with biotin before symptoms occur.
SHR LIS Test Code​
  • ​BIOQ (For Laboratory Use Only)

​ ​Test Ordering Requirements

​Forms Required

​ ​Patient History

​Relevant Clinical History
  • Family History
  • Must include diagnosis and/or patient symptoms on the requisition​


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Laboratory Controlled Document LSM-962 v1

Last Modified: Thursday, January 24, 2019 |
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