Zollinger Ellison Syndrome
Test Protocol

For more information on testing for Z-E Syndrome please call

1-877-CRC-4888

ZE Information ZE Test Protocol

ZE Syndrome

 
ZE Test Protocol

To perform a Zollinger-Ellison Syndrome Test Follow this procedure:

 

  1. The patient should have fasted for at least 12 hours prior to beginning the test.
     

  2. Prior to injection of ChiRhoStim® (Human Secretin for injection), two blood samples are drawn for determination of fasting serum gastrin levels (baseline values).
     

  3. Subsequently, a test dose of ChiRhoStim® (Human Secretin for Injection) 0.2 mcg (0.1 mL) is injected intravenously to test for possible allergies.
     

  4. If no untoward reactions occur, ChiRhoStim® Human Secretin for injection) at a dose of 0.4 mcg/kg of body weight is injected intravenously over 1 minute;
     

  5. Post-injection blood samples are collected after 1, 2, 5, 10, and 30 minutes for determination of serum gastrin concentrations.
     

  6. Gastrinoma is strongly indicated in patients who show an increase in serum gastrin concentrations of at least 110 pg/mL over basal level on any of the post secretin injection samples.

 

Testing Labs for Gastrin, Serum:

 
bullet

Lab Corp: Test number 004390

bullet

Quest Diagnostics: Test number 784X

 

 

Testing Sample information:

 
bullet

CPT Code for Gastrin, Serum is 82941 (per specimen)

bullet

First collect Gastrin Serum in the Red-stopper tube or serum gel separator tube

bullet

Then transfer the serum into a lavender colored frozen tube.

bullet

The Specimen must be frozen when shipped to the testing labs

bullet

Test turn around time is 24-72 hours.

 

 

 

For more Information on ChiRhoStim® (Human Secretin for injection)

Please download the Package Insert.
 

 

Lab Core Gastrin, Serum Test and Specifications

    

 

Number

 

004390

CPT

 

82941 (per specimen)

Related Information

 

·  Pancreatic Polypeptide

Special Instructions

 

See the Endocrine Appendix for instructions on multiple specimen testing.

Specimen

 

Serum, frozen

Volume

 

0.5 mL

Minimum Volume

 

0.3 mL (Note: This volume does not allow for repeat testing.)

Container

 

Red-top tube or gel-barrier tube

Collection

 

Separate serum from cells. Transfer the serum into a LabCorp PP transpak frozen purple tube with screw cap (LabCorp No 49482). Freeze immediately and maintain frozen until tested. To avoid delays in turnaround time when requesting multiple tests on frozen samples, please submit separate frozen specimens for each test requested.

Storage Instructions

 

Freeze immediately.

Patient Preparation

 

The patient must be fasting overnight, 12-14 hours.

Causes for Rejection

 

Gross hemolysis; patient not fasting; specimen not received frozen; gross lipemia; plasma specimen

Reference Interval

 

Pediatrics1,2,3 and adults:

bullet0-1 month: 69-190 pg/mL
bullet2-22 months: 55-186 pg/mL
bullet22 months to 16 years: fasting 3-4 hours: 2-168 pg/mL, fasting 5-6 hours: 3-117 pg/mL, fasting >8 hours: 1-125 pg/mL
bulletOlder than 16 years: 0-115 pg/mL

Use

 

Diagnose Zollinger-Ellison (Z-E) syndrome; diagnose gastrinoma. Gastrin >1000 pg/mL with gastric acid hypersecretion (basal acid secretion >15 mmol/hour in a patient with peptic ulcer who has not had surgery) establishes unequivocally the diagnosis of the Zollinger-Ellison syndrome.4 Antral G-cell hyperplasia may relate to high gastrin levels and duodenal ulcer.

Limitations

 

Gastric hyperacidity must be documented. Gastric ulcer, chronic renal failure, hyperparathyroidism, pyloric obstruction, carcinoma of stomach,5 vagotomy without gastric resection, retained gastric antrum and short bowel syndrome have been reported with moderate elevations of gastrin levels. Gastrin levels are increased with pernicious anemia. H2-receptor blockers (cimetidine) may result in elevated levels. Overlap of serum gastrin values between gastrinoma and other states occurs. Up to 40% of Z-E patients have fasting gastrin values between 100 and 500 pg/mL, while a few patients with gastric or duodenal ulcer without gastrinoma, have results in this range. At least half of patients with the Z-E syndrome lack diagnostic serum gastrin levels, although in nearly all, fasting serum gastrin levels are increased.4 One report describes a patient with Z-E syndrome with a normal initial gastrin level.6

Methodology

 

Immunochemiluminometric assay (ICMA)

Additional Information

 

Gastrin is secreted by antral G cells and stimulates gastric acid production, antral motility, and secretion of pepsin and intrinsic factor. The principle forms of gastrin in blood are G-34 (big gastrin, half-life is 5 minutes) and G-14 (minigastrin, half-life is 5 minutes). Each of these polypeptides circulates in nonsulfated (I) or sulfated (II) forms. Instilling acid into the stomach normally inhibits gastrin secretion. Elevated gastrin levels should be interpreted in light of gastric acid secretion and other parameters. The neuroendocrine tumors associated with the Zollinger-Ellison syndrome are characterized by elevated rates of gastric HCl secretion and upper gastrointestinal ulcer disease. Gastrin levels >500-600 pg/mL in a patient with basal acid hypersecretion often indicate gastrinoma, but antral G-cell hyperplasia cases can have gastrin levels >500 pg/mL and hyperchlorhydria. If gastrinoma is likely but fasting gastrin level is not diagnostic, the secretin test is the provocative test of choice. Absolute increase in serum gastrin level above the basal figure is preferred to percent change.4 I.V. secretin normally diminishes gastrin, but serum gastrin increases in gastrinoma patients. Wolfe provides an explanation for this paradoxical effect.4 Calcium infusion also stimulates gastrin release but does not distinguish other causes of ulcer as well as the secretin test. Protocols for stimulation tests are published.7

Fifteen percent to 26% of Z-E patients have evidence of Werner syndrome (multiple endocrine neoplasia type 1). It may include hyperparathyroidism, islet cell tumors of the pancreas, pituitary tumors, Cushing syndrome (adrenal glands), and hyperparathyroidism.8 Gastrinoma are malignant in 62% of cases, and 44% of patients have metastases.

No consistent relationship has been established between Helicobacter pylori (Campylobacter pylori) and gastric acid secretion or serum gastrin levels.

Features of gastrinoma additional to those of peptic ulcer may include diarrhea and steatorrhea.

Gastrinomas are usually found in the pancreas but they may be primary in the duodenum. A few cases in which a gastrinoma was primary in the stomach have been reported. The morphology is that of foregut carcinoids.9

Footnotes

 
  1. Meites S, Buffone GJ, Cheng MH, et al, eds, Pediatric Clinical Chemistry, Reference (Normal) Values, 3rd ed, Washington, DC: AACC Press, 1989, 131.
  1. Sann L, Chayvialle AP, Bremond A, et al, “Serum Gastrin Level in Early Childhood,” Arch Dis Child, 1975, 50(10):782-5.
  1. Janik JS, Akbar AM, Burrington JD, et al, “Serum Gastrin Levels in Infants and Children,” Pediatrics, 1977, 60(1):60-4.
  1. Wolfe MM, “Diagnosis of Gastrinoma: Much Ado About Nothing?” Ann Intern Med, 1989, 111(9):697-9.
  1. Rakic S and Milicevic MN, “Serum Gastrin Level in Patients With Intestinal and Diffuse Type of Gastric Cancer,” Br J Cancer, 1991, 64(6):1189.
  1. Yanda RJ, Ostroff JW, Ashbaugh CD, et al, “Zollinger-Ellison Syndrome in a Patient With Normal Screening Gastrin Level,” Dig Dis Sci, 1989, 34(12):1929-32.
  1. Malagelada JR, Glanzman SL, and Go VL, “Laboratory Diagnosis of Gastrinoma. II. A Prospective Study of Gastrin Challenge Tests,” Mayo Clin Proc, 1982, 57(4):219-26.
  1. Jensen RT, Gardner JD, Raufman JP, et al, “Zollinger-Ellison Syndrome: Current Concepts and Management,” Ann Intern Med, 1983, 98(1):59-75 (review).
  1. Wilander E, “Endocrine Cell Tumours,” Gastrointestinal and Oesophageal Pathology, Whitehead R, ed, New York, NY: Churchill Livingstone, 1989, 629-41

References

 

Cherner JA, Doppman JL, Norton JA, et al, “Selective Venous Sampling for Gastrin to Localize Gastrinomas. A Prospective Assessment,” Ann Intern Med, 1986, 105(6):841-7.

Clain JE, “Diagnosis and Management of Gastrinoma (Zollinger-Ellison Syndrome),” Mayo Clin Proc, 1982, 57(4):265-7.

den Hartog G, van der Meer JW, Jansen JB, et al, “Decreased Gastrin Secretion in Patients With Late-Onset Hypogammaglobulinemia,” N Engl J Med, 1988, 318(24):1563-7.

Fraker DL and Norton JA, “The Role of Surgery in the Management of Islet Cell Tumors,” Gastroenterol Clin North Am, 1989, 18(4):805-30.

Friesen SR and Tomita T, “Pseudo-Zollinger-Ellison Syndrome, Hypergastrinemia, Hyperchlorhydria Without Tumor,” Ann Surg, 1981, 194(4):481-93.

Green DW, Gomez G, and Greeley GH Jr, “Gastrointestinal Peptides,” Gastroenterol Clin North Am, 1989, 18(4):695-733.

Malagelada JR, Davis CS, O'Fallon WM, et al, “Laboratory Diagnosis of Gastrinoma. I. A Prospective Evaluation of Gastric Analysis and Fasting Serum Gastrin Levels,” Mayo Clin Proc, 1982, 57(4):211-8.

McQuaid KR, “Much Ado About Gastrin,” J Clin Gastroenterol, 1991, 13(3):249-54.

Modlin IM, Jaffe BM, Sank A, et al, “The Early Diagnosis of Gastrinoma,” Ann Surg, 1982, 196(5):512-7.

Solcia E, Capella C, Fiocca R, et al, “The Gastroenteropancreatic Endocrine System and Related Tumors,” Gastroenterol Clin North Am, 1989, 18(4):671-93.

Warburton R and Close JR, “The In Vitro Stability of Gastrin in Serum and Whole Blood,” Ann Clin Biochem, 1987, 24(Pt 3):320-1.

Wolfe MM, Jain DK, and Edgerton JR, “Zollinger-Ellison Syndrome Associated With Persistently Normal Fasting Serum Gastrin Concentrations,” Ann Intern Med, 1985, 103(2):215-7.

 

 

 

 

 


Copyright © 2008-2009 ChiRhoClin, Inc. All rights reserved.