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.
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 Category

Laboratory Developed Test (LDT)

Note
Hotline History
N/A
CPT Codes

82261

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