Zollinger-Ellison syndrome

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Zollinger-Ellison syndrome is  one of the severe cause of peptic ulcer disease (PUD) due to pancreatic adenoma gastrin-mediated gastric acid hypersecrection. ZES may occur at the age of 50 with a part of an autosomal dominant familial syndrome called multiple endocrine neoplasia type 1(MEN type1). Rarely, adenomas are located in the stomach and duodenum .50-60% is malignant, 10% are multiple and 30% or cases are associated with MEN type 1.

What is the pathophysiolgy of Zollinger-Ellison syndrome?

Zollinger-Ellison syndrome is caused by gastrinomas (tumor) which release the gastrin hormone.  Gastrin resulting in excess gastric acid secretion. The excess acid cause many ulcers in the stomach and duodenum.

What are the symptoms of ZES?

1. Burning abdominal pain especially in the epigastric region.
2. Diarrhea and weight loss in 40% of patients
3. Nausea and vomiting
4. Gastro intestinal bleeding
5. History of Nephrolithiasis, hyperparathyroidism, and gastrinoma also may be present if ZES is due to MEN type 1.

How to diagnosed Zollinger-Ellison syndrome?

ZES should be considered in any patients present with

1. PUD refractory to treatment for helicobacter pylori and convention dose of H2 blocker or omeprazole.
2. Recurrent, multiple, or atypically located peptic ulcer.
3. Peptic Ulcer disease with diarrhea.

Diagnosis is confirmed by lab test, endoscopy and imaging studies.

Lab tests:

1. A fasting serum gastric level of 100 pg/ml of greater and a basal gastric acid output (BAO) of 15 mEq/hour.
2. Increased gastrin levels (>200 pg/mL over base line) in response to a secretin stimulation test.

Endoscopy: Endoscopy shows multiple ulcers in stomach and duodenum.

Imaging studies: Imaging studies includes angiograph, ultrasonography and computerized tomography (CT).

How to managed Zollinger-Ellison syndrome?

A patient of ZES is managed with H2-histamine receptor antagonist like (cimentidine) or Omeprazole.  Surgical removal of tumor is the only cure of ZES. If a gastrinoma cannot be localized intraoperative, a parietal cell vagotomy may be preformed. Surgical debulking of metastatic or unrespectable primary gastrinoma and patients with ZES and MEN 1 facilitates medical treatment and prolongs life expectancy.

Filed Under: Gastroenterology

Tags: featured, gastrinoma, peptic ulcer disease, PUD, ZES, Zollinger-Ellison syndrome