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Biotinidase, Serum (with Paired Normal Control)
0093362
Ordering Recommendation

Initial biotinidase enzyme test to diagnose or rule out biotinidase deficiency.

Mnemonic
BTD ENZ
Methodology
Spectrophotometry
Performed
Tue, Fri
Reported
1-6 days
New York DOH Approval Status
This test is New York DOH approved.
ARUP Consult®
Disease Topics
Specimen Required
Patient Preparation
A control specimen needs to be sent with the patient specimen. The control specimen needs to be drawn from a normal, healthy individual who is not biologically related to the patient. Collect control specimen within 30 minutes of patient specimen. 
Collect
Plain red or serum separator tube (patient) AND plain red serum separator tube (control). 
Specimen Preparation
Separate serum from cells and freeze ASAP or within 2 hours of collection. Transfer 2 mL serum (patient) AND 2 mL serum (control) to individual ARUP Standard Transport Tubes. (Min: 0.2 mL (patient) AND 0.2 mL (control)). Label control tube as "Control for [patient name]". 
Storage/Transport Temperature
CRITICAL FROZEN. Separate specimens must be submitted when multiple tests are ordered. Ship patient and control specimens together. 
Unacceptable Conditions
Refrigerated or room temperature specimens. Specimens subjected to more than one freeze/thaw cycle. 
Remarks
 
Stability
After separation from cells: Ambient: 1 hour; Refrigerated: 1 hour; Frozen: 1 month 
Reference Interval
3.5-13.8 U/L
Interpretive Data


Compliance Statement B: This test was developed and its performance characteristics determined by ARUP Laboratories. The U.S. Food and Drug Administration has not approved or cleared this test; however, FDA clearance or approval is not currently required for clinical use. The results are not intended to be used as the sole means for clinical diagnosis or patient management decisions.

Components
Component Test Code*Component Chart NameLOINC
0093363Biotinidase, Paired Normal Control54457-7
0093368Biotinidase, Patient1982-8
* Component test codes cannot be used to order tests. The information provided here is not sufficient for interface builds; for a complete test mix, please click the sidebar link to access the Interface Map.
Aliases
  • Biotin-amide amidohydrolase
  • Biotinidase deficiency