Ordering Recommendation

Evaluate patients with suspected porphyria presenting with neurologic/psychiatric, abdominal, and/or cutaneous symptoms.

Mnemonic
PORUFPBGU
Methodology

High Performance Liquid Chromatography/Ion Exchange Chromatography/Quantitative Spectrophotometry

Performed

Mon-Fri

Reported

2-5 days

New York DOH Approval Status
This test is New York DOH approved.
Specimen Required
Patient Preparation
Collect

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

Specimen Preparation

Protect from light. Transfer 8 mL aliquot to an ARUP Amber Transport Tube. (Min: 4 mL) Record total volume and collection time interval on transport tube and test request form.

Storage/Transport Temperature

Frozen.

Unacceptable Conditions

Body fluids other than urine.

Remarks
Stability

Ambient: Unacceptable; Refrigerated: 4 days; Frozen: 1 month

Reference Interval
Test Number
Components
Reference Interval
Uroporphyrin 0-4 µmol/mol crt
Heptacarboxylate Porphyrin 0-2 µmol/mol crt
Coproporphyrin I 0-6 µmol/mol crt
Coproporphyrin III 0-14 µmol/mol crt
Porphobilinogen, Urine 0.0-8.8 µmol/L
Porphobilinogen, Urine (24-hour) 0.0-11.0 µmol/d
0020473 Creatinine, Urine - per 24h
Age
Male
Female
3-8 years 140-700 mg/d 140-700 mg/d
9-12 years 300-1300 mg/d 300-1300 mg/d
13-17 years 500-2300 mg/d 400-1600 mg/d
18-50 years 1000-2500 mg/d 700-1600 mg/d
51-80 years 800-2100 mg/d 500-1400 mg/d
81 years and older 600-2000 mg/d 400-1300 mg/d

Interpretive Data

Results are normalized to creatinine concentration and reported as a ratio of amounts (micromoles of porphyrin/moles of creatinine).

Compliance Category

Laboratory Developed Test (LDT)

Note

Urine porphyrins are useful for the evaluation of cutaneous photosensitivity to exclude porphyria cutanea tarda (PCT). Urine porphobilinogen (PBG) is useful for the evaluation of neurologic and/or psychiatric symptoms to exclude acute porphyrias such as acute intermittent porphyria (AIP).

Hotline History
N/A
CPT Codes

84120; 84110

Components
Component Test Code* Component Chart Name LOINC
0020207 Creatinine, Urine - per volume 2161-8
0020208 Creatinine, Urine - per 24h 2162-6
0080262 Porphobilinogen (PBG), Urine -per volume 2811-8
0080365 Uroporphyrin - ratio to CRT 32043-2
0080366 Heptacarboxylate - ratio to CRT 38175-6
0080369 Porphobilinogen (PBG), Urine -per 24h 14882-5
0080459 Porphyrin Urine Interpretation 49292-6
0097110 Total Volume 19153-6
0097111 Hours Collected 30211-7
2002059 Coproporphyrin I - ratio to CRT 45313-4
2002061 Coproporphyrin III - ratio to CRT 45315-9
* 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
  • Coproporphyrin
  • Heptacarboxyl Porphyrin
  • Hexacarboxyl Porphyrin
  • PBG
  • Pentacarboxyl Porphyrin
  • Porphobilinogen
  • Porphyrins, Quantitative, 24-Hour Urine
  • Urine Quantitative
  • Uroporphyrin
Porphyrins and Porphobilinogen (PBG), Urine