Ordering Recommendation

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

New York DOH Approval Status

This test is New York state approved.

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.


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.


After separation from cells: Ambient: 1 hour; Refrigerated: 1 hour; Frozen: 1 month




Tue, Fri


1-6 days

Reference Interval

3.5-13.8 U/L

Interpretive Data

This test was developed and its performance characteristics determined by ARUP Laboratories. It has not been cleared or approved by the US Food and Drug Administration. This test was performed in a CLIA certified laboratory and is intended for clinical purposes.

Compliance Category

Laboratory Developed Test (LDT)


Hotline History


CPT Codes



Component Test Code* Component Chart Name LOINC
0093363 Biotinidase, Paired Normal Control 54457-7
0093368 Biotinidase, Patient 1982-8
* 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.


  • Biotin-amide amidohydrolase
  • Biotinidase deficiency
Biotinidase, Serum (with Paired Normal Control)