Alias

EPIC-6156; ARUP-0080260

Collect

Random or 24-hour urine. Refrigerate 24-hour specimens during collection.

Container


ARUP Amber Transport Tube
Institute Instructions
Amery, Hudson,
Lakeview,Regions,
Westfields
ARUP Amber Transport Tube (ARUP supply #13654) available online through eSupply using ARUP Connect
HealthPartners
Clinics
Lawson/Warehouse Supply Item: ARUP Amber Transport Tube, Lawson 5008712

Specimen Preparation

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.

Unacceptable Conditions

Body fluids other than urine.

Storage/Transport Temperature

Frozen.

Stability (from collection to initiation)

Ambient: Unacceptable; Refrigerated: 1 week; Frozen: 1 month

Performed

Sun-Sat

Reported

1-5 days

Ordering Recommendations

Essential first-line test for suspected acute porphyria in adults. Use in determining metabolic response to IV hematin.

Notes

Appropriate test to rule out acute intermittent porphyria (AIP) and other acute attack types of porphyrias associated with neurologic and/or psychiatric symptoms.

Referral Lab

Reference Interval

Components
Reference Interval
Creatinine, Urine - per 24h
Age
Male (mg/d)
Female (mg/d)
3-8 years140-700140-700
9-12 years300-1300300-1300
13-17 years500-2300400-1600
18-50 years1000-2500700-1600
51-80 years800-2100500-1400
81 years and older600-2000400-1300

Porphobilinogen, Urine - per 24h0.4 - 1.5 µmol/d
Porphobilinogen, Urine - ratio to CRT0.0 - 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.

Methodology

Quantitative High Performance Liquid Chromatography-Tandem Mass Spectrometry

CPT Codes

84110

Performing Lab

ARUP Laboratories
Collection

Alias

EPIC-6156; ARUP-0080260

Collect

Random or 24-hour urine. Refrigerate 24-hour specimens during collection.

Container


ARUP Amber Transport Tube
Institute Instructions
Amery, Hudson,
Lakeview,Regions,
Westfields
ARUP Amber Transport Tube (ARUP supply #13654) available online through eSupply using ARUP Connect
HealthPartners
Clinics
Lawson/Warehouse Supply Item: ARUP Amber Transport Tube, Lawson 5008712

Specimen Preparation

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.

Unacceptable Conditions

Body fluids other than urine.

Storage/Transport Temperature

Frozen.

Stability (from collection to initiation)

Ambient: Unacceptable; Refrigerated: 1 week; Frozen: 1 month

Performed

Sun-Sat

Reported

1-5 days

Ordering Recommendations

Essential first-line test for suspected acute porphyria in adults. Use in determining metabolic response to IV hematin.

Notes

Appropriate test to rule out acute intermittent porphyria (AIP) and other acute attack types of porphyrias associated with neurologic and/or psychiatric symptoms.

Referral Lab

Result Interpretation

Reference Interval

Components
Reference Interval
Creatinine, Urine - per 24h
Age
Male (mg/d)
Female (mg/d)
3-8 years140-700140-700
9-12 years300-1300300-1300
13-17 years500-2300400-1600
18-50 years1000-2500700-1600
51-80 years800-2100500-1400
81 years and older600-2000400-1300

Porphobilinogen, Urine - per 24h0.4 - 1.5 µmol/d
Porphobilinogen, Urine - ratio to CRT0.0 - 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.

Methodology

Quantitative High Performance Liquid Chromatography-Tandem Mass Spectrometry
Administrative

CPT Codes

84110

Performing Lab

ARUP Laboratories

ARUP Consult®

Disease Topics