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Epic Code LAB1231992 Porphyrins, Fractionated, Quantitative and Porphobilinogen, 24-Hour Urine

Important Note

  • Must protect from light for entire collection.

Test Name Alias

LAB1231258 | PorPBGU  | Quest: 16105 

Interface Order Alias

1231992

Clinical Information

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

 

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.

Specimen Requirements

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.

 

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)

Specimen Transport Temperature

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

Rejection Criteria

  • Not protected from light.
  • pH <4.0 (acidified urine)
  • Specimens not collected and processed as indicated. 

Specimen Stability

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.

Test Frequency

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 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

 

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

 

Performing Department

Send Outs

Performing Department Laboratory Location

Sent to Quest Diagnostics- Wood Dale, IL

Methodology

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

CPT

84120, 84110.

LOINC

Unavailable

Reviewed Date

11/8/2025

Orderable Regions

Send Outs