Lab Test

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Porphyrins, Fractionated, Quantitative and Porphobilinogen, 24-Hour Urine

Test Codes

EPIC: LAB1231992, Beaker: PorPBGU, Quest: 17198

NOTE: Includes Porphyrins, Fractionated, Quantitative, 24-Hour Urine (Coproporphyrin I, Coproporphyrin III, Heptacarboxyporphyrin, Hexacarboxyporphyrin, Pentacarboxyporphyrin, Uroporphyrin I, Uroporphyrin III, Total Porphyrins), Porphobilinogen, Quantitative, 24-Hour Urine.

Department

Send Outs

Instructions

Specimen Collection Criteria

Collect (preferred specimen): 24-hour urine sample with no preservative.

  • Keep 24-hour urine specimen iced or refrigerated during collection.
  • Include start and end dates and times for the collection period on the specimen container.
  • All specimens must be protected from light.

Physician Office/Draw Specimen Preparation

Protect specimen from light. Maintain specimens refrigerated (2-8°C or 36-46) prior to transport..

Preparation for Courier Transport

Transport: Entire 24-hour urine collection, refrigerated (2-8°C or 36-46).

Rejection Criteria

Specimens not collected and processed as indicated. 

In-Lab Processing

Protect specimen from light. Measure total volume of 24-hour urine specimen. Record total volume and collection start and end dates and times in the LIS system. Aliquot 4.0 mL from the well-mixed 24-hour urine collection and refrigerate (2-8°C or 36-46). (Minimum: 2.0 mL)

Storage

Specimen Stability for Testing:

Room Temperature (20-26°C or 68-78.8°F): Unacceptable
Refrigerated (2-8°C or 36-46°F): 7 days
Frozen (-20°C/-4°F or below): 30 days

Specimen Storage in Department Prior to Disposal:

Specimen retention time is determined by the policy of the reference laboratory. Contact the Send Outs Laboratory with any questions.

Laboratory

Sent to Quest Diagnostics, Wood Dale, IL.

Performed

Information not provided.

Reference Range

Porphyrins, Fractionated, Quantitative, 24-Hour Urine
Uroporphyrin I                        4.1-22.4 mcg/24 h
Uroporphyrin III                      0.7-7.4 mcg/24 h
Heptacarboxyporphyrin       ≤3.3 mcg/24 h
Hexacarboxyporphyrin         ≤10.0 mcg/24 h
Pentacarboxyporphyrin        ≤4.6 mcg/24 h
Coproporphyrin I                    7.1-48.7 mcg/24 h
Coproporphyrin III                  11.0-148.5 mcg/24 h
Total Porphyrins                     35.0-210.7 mcg/24 h

Porphobilinogen, Quantitative, 24-Hour Urine
Pediatric                                   Not established.
Adult                                         <0.34 mg/24 h

Test Methodology

High Performance Liquid Chromatography (HPLC), Chromatography/Mass Spectrometry

  • Porphyrins, Fractionated, Quantitative, 24-Hour Urine (Coproporphyrin I, Coproporphyrin III, Heptacarboxyporphyrin, Hexacarboxyporphyrin, Pentacarboxyporphyrin, Uroporphyrin I, Uroporphyrin III, Total Porphyrins)
  • Porphobilinogen, Quantitative, 24-Hour Urine

Interpretation

By report.

Clinical Utility

Porphyrins, Fractionated, Quantitative and Porphobilinogen, 24-Hour Urine - Urinary Porphobilinogen is the first step in the diagnosis of acute porphyrias such as acute intermittent porphyria (AIP). AIP is an autosomal dominant disorder characterized by deficiency of porphobilinogen deaminase. An acute attack usually includes gastrointestinal disturbance and neuropsychiatric disorders.

CPT Codes

84120, 84110.

Contacts

Last Updated

11/19/2025

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