NIH Clinical Center
Dept. of Lab Medicine | Dept. of Transfusion Medicine | Anatomic Pathology Test Guide > Lab Test
 Lab Test
 
 Test Description
Panel Name:
Test Name:
Porphobilinogen, Urine
Panel Tests:
LIS Test Code:PORBN, PBNR3
Synonyms:
Department:Laboratory Medicine
Lab Area:Mayo Medical Labs
Telephone:301-496-5972
TAT:2-7 days
 Specimen Collection
Source:

Site:

Container:4L Fb
Yellow Conical

Micro. Container:


Collection Instructions:

PROTECT SPECIMEN FROM LIGHT!

Delivery Instructions:Send specimen to 2C324B
Specimen Type: Urine
Frequency / Restrictions:
 Expected Values/Results

Ranges and Interpretation:

Porphobilinogen, Random  mcmol/L
Performed at Mayo Medical Labs, Rochester MN
Effective 20Apr10 – present:  <=1.3

Porphobilinogen, 24 hour  mg/24hr  (SI:  mcmol/d = 4.42 x mg/24hr)
Performed at Quest Diagnostics/Nichols Institute, San Juan Capistrano CA
Effective 13Oct99 – present:   0 – 2.7

Organisms Reported:

Alert Limits:

 Additional Test Information

Comments:

Urinary porphobilinogen (PBG) is elevated during the acute phase of the neurologic porphyrias: acute intermittent porphyria (AIP), hereditary coproporphyria (HCP), and variegate porphyria (VP). Acute attacks may produce symptoms of cerebral dysfunction and damage (confusion, "psychiatric disease"), autonomic neuropathy, constipation, urinary retention, tachycardia, hypertension, and severe (sometimes chronic) abdominal pain. In the acute phase, these disorders can be life threatening. Between attacks patients have mild symptoms that may include psychoneuroses. Several studies indicate that approximately 1 in 300 psychiatric patients have AIP.

 The common symptoms of AIP, HCP, and VP mimic many other diseases from acute appendicitis to acute schizophrenia. Acute episodes usually begin with or include abdominal discomfort ranging from mild cramping to severe pain that suggests a need for surgical intervention. These symptoms and outward signs are common to a variety of medical problems and diagnosis usually depends on timely, accurate laboratory testing. Frequently, surgical intervention can be avoided if the laboratory returns an elevated level for urinary PBG.


Methodology:

Liquid Chromatography-Tandem Mass Spectrometry 
(LC-MS/MS) Stable Isotope Dilution Analysis


Cautions:

Ideally, specimen collection should occur during the acute phase. Porphobilinogen (PBG) may be normal when the patient is not exhibiting symptoms.

 PBG is susceptible to degradation at high temperatures, at pH <5.0, and on prolonged exposure to light. Specimens should be frozen immediately following collection and protected from light.

 
Archived Ranges:Historical Reference Ranges



 
 

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