Porphobilinogen (PBG), Urine
Ordering Recommendation
Essential first-line test for suspected acute porphyria in adults. Use in determining metabolic response to IV hematin.
New York DOH Approval Status
Specimen Required
Random or 24-hour urine. Refrigerate 24-hour specimens during collection.
Protect from light. Transfer 2 mL aliquot from a random or well-mixed 24-hour collection to ARUP amber transport tubes. (Min: 1.5 mL) Record total volume and collection time interval on transport tube and test request form.
Frozen.
Body fluids other than urine.
Ambient: Unacceptable; Refrigerated: 1 week; Frozen: 1 month
Methodology
Quantitative High Performance Liquid Chromatography-Tandem Mass Spectrometry
Performed
Sun-Sat
Reported
1-5 days
Reference Interval
Test Number |
Components |
Reference Interval |
|||||||||||||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Creatinine, Urine - per 24h |
|
||||||||||||||||||||||
Porphobilinogen, Urine - per 24h | 0.4 - 1.5 µmol/d | ||||||||||||||||||||||
Porphobilinogen, Urine - ratio to CRT | 0.0 - 0.2 mmol/mol CRT |
Interpretive Data
Porphobilinogen (PBG), Urine
Results for random urine specimens are normalized to creatinine (CRT) concentration and reported as a ratio of amounts (millimoles of PBG/mole of creatinine).
Porphobilinogen (PBG) in a random urine specimen is used to evaluate an attack of acute porphyria. Slight increases in urinary PBG are associated with acute porphyrias other than acute intermittent porphyria (AIP) and may indicate a resolving or treated acute porphyria.
Urinary PBG in excess of two times the upper reference limit is consistent with acute porphyria.
Per 24h calculations are provided to aid interpretation for collections with a duration of 24 hours and an average daily urine volume. For specimens with notable deviations in collection time or volume, ratios of analytes to a corresponding urine creatinine concentration may assist in result interpretation.
Laboratory Developed Test (LDT)
Note
Appropriate test to rule out acute intermittent porphyria (AIP) and other acute attack types of porphyrias associated with neurologic and/or psychiatric symptoms.
Hotline History
Hotline History
CPT Codes
84110
Components
Component Test Code* | Component Chart Name | LOINC |
---|---|---|
0020207 | Creatinine, Urine - per volume | 2161-8 |
0020208 | Creatinine, Urine - per 24h | 2162-6 |
0080261 | Porphobilinogen, Urine - per 24h | 14882-5 |
0080262 | Porphobilinogen (PBG), Urine -per volume | 2811-8 |
0097110 | Total Volume | 19153-6 |
0097111 | Hours Collected | 30211-7 |
3017599 | Porphobilinogen, Urine - ratio to CRT | 34357-4 |
3017600 | Porphobilinogen Interpretation | 93479-4 |
Aliases
- Acute Porphyria Screening Test
- PBG, Quantitative Porphobilinogen