Biotinidase Serum
Test Mnemonic
BIOTIN
CPT Codes
- 82261 - QTY (1)
LOINC ®
1982-8
Performing Laboratory
Cleveland Clinic Laboratories
FDA Category
Laboratory Developed Test
Specimen Requirements
| Volume | Type | Container | Collect Temperature | Transport Temperature | Special Instructions |
|---|---|---|---|---|---|
| 1 mL | Serum | SST (Gold) | Centrifuge, aliquot and freeze ASAP. | Centrifuge and remove serum ASAP or within 30 minutes of collection. Freeze immediately. Transport frozen specimen to testing laboratory. |
Alternate Specimen Requirements
| Volume | Type | Container | Collect Temperature | Transport Temperature | Special Instructions |
|---|---|---|---|---|---|
| 1 mL | Plasma | Green LiHep Nogel | Centrifuge, aliquot and freeze ASAP. | Centrifuge and remove plasma ASAP or within 30 minutes of collection. Freeze immediately. Transport frozen specimen to testing laboratory. | |
| 1 mL | Serum | Red Plain | Centrifuge, aliquot and freeze ASAP. | Centrifuge and remove serum from clot ASAP and then freeze immediately. Transport frozen to testing laboratory. | |
| 1 mL | Plasma | Lithium heparin PST (Lt. Green) | Centrifuge, aliquot and freeze ASAP. | Centrifuge and remove plasma ASAP or within 30 minutes of collection. Freeze immediately. Transport frozen specimen to testing laboratory. | |
| 1 mL | Plasma | Sodium heparin (Green) | Centrifuge, aliquot and freeze ASAP. | Centrifuge and remove plasma ASAP or within 30 minutes of collection. Freeze immediately. Transport frozen specimen to testing laboratory. |
Minimum Specimen Requirements
| Volume | Type | Container | Collect Temperature | Transport Temperature | Special Instructions |
|---|---|---|---|---|---|
| 0.5 mL |
Stability
| Environmental Condition | Description |
|---|---|
| Ambient | 4 hours, after separation from cells |
| Refrigerated | 4 hours, after separation from cells |
| Frozen | 7 days, after separation from cells, at < -20 degrees Celsius |
Days Performed
Varies
Turnaround Time
1 - 7 days
Methodology
| Name | Description |
|---|---|
| Colorimetry |
Reference Range
| Biotinidase Serum | |||||
|---|---|---|---|---|---|
| Sex | Age From | Age To | Type | Range | Range Unit |
| Years | 99 Years | Normal | 4.4 - 12.0 | U/L |
Special Info
Centrifuge, remove from cells, aliquot and freeze ASAP. Transport frozen.
Clinical Info
Evaluation of individuals with: 1) unexplained seizures; 2) unexplained ataxia or marked clumsiness; 3) unexplained hearing loss, especially if on a sensorineural basis and especially if accompanied by neurological abnormalities and/or dermatitis; 4) unexplained dermatitis, especially if accompanied by epilepsy, ataxia, intellectual disability and/or hearing loss. Partial deficiencies and carriers may occur at the low end of the reference range; restesting of the biotinidase activity and/or determination of possible mutations of the biotinidase gene may be indicated if the reported enzyme activity is < 4.5 U/L, depending on the enzyme level and clinical context.
Clinical Limitation
With respect to the reference interval, neonates and infants younger than 6 months of age generally have activities that are lower than those of normal adults.