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Zollinger-Ellison syndrome
Classification and external resources

Endoscopy image of multiple small ulcers in the distal duodenum in a patient with Zollinger-Ellison syndrome
ICD-10 E16.4
ICD-9 251.5
MedlinePlus 000325
eMedicine med/2437 ped/2472
MeSH D015043

Zollinger-Ellison syndrome is a disorder where increased levels of the hormone gastrin are produced, causing the stomach to produce excess hydrochloric acid. Often the cause is a tumor (gastrinoma) of the duodenum or pancreas producing the hormone gastrin. Gastrin then causes an excessive production of acid which can lead to peptic ulcers in almost 95% of patients.

Contents

Pathophysiology

Gastrin works on stomach parietal cells causing them to secrete more hydrogen ions into the stomach lumen. In addition, gastrin acts as a trophic factor for parietal cells, causing parietal cell hyperplasia. Thus there is an increase in the number of acid-secreting cells, and each of these cells produces acid at a higher rate. The increase in acidity contributes to the development of peptic ulcers in the stomach and duodenum. High acid levels lead to multiple ulcers in the stomach and small bowel.

Presentation

Patients with Zollinger-Ellison syndrome may experience abdominal pain and diarrhea. The diagnosis is also suspected in patients without symptoms who have severe ulceration of the stomach and small bowel, especially if they fail to respond to treatment.

Gastrinomas may occur as single tumors or as multiple, small tumors. About one-half to two-thirds of single gastrinomas are malignant tumors that most commonly spread to the liver and lymph nodes near the pancreas and small bowel. Nearly 25 percent of patients with gastrinomas have multiple tumors as part of a condition called multiple endocrine neoplasia type I (MEN I). MEN I patients have tumors in their pituitary gland and parathyroid glands in addition to tumors of the pancreas.

Symptoms

Diagnosis

Clinical suspicion of Zollinger-Ellison syndrome may be aroused when the above symptoms prove resistant to treatment, when the symptoms are especially suggestive of the syndrome, or endoscopy is suggestive. The diagnosis of Zollinger-Ellison syndrome is made by several laboratory tests and imaging studies.

  • Secretin stimulation test, which measures evoked gastrin levels
  • Fasting gastrin levels, on at least three separate occasions[1]
  • Gastric acid secretion and pH

In addition, the source of the increased gastrin production must be discovered. This is either done using MRI or somastatin receptor scintigraphy, the investigation of choice.[2]

Therapy

Proton pump inhibitors (such as omeprazole and lansoprazole) and histamine H2-receptor antagonists (such as famotidine and ranitidine) are used to slow down acid secretion. Cure is only possible if the tumours are surgically removed, or treated with chemotherapy. Octreotide can be used to alleviate symptoms.

History

This syndrome was first described in 1955 by Robert Zollinger and Edwin Ellison, surgeons at the Ohio State University.[3]

References

  1. ^ http://www.patient.co.uk/showdoc/40001304/
  2. ^ Jensen RT; Gastrinomas: advances in diagnosis and management. Neuroendocrinology. 2004;80 Suppl 1:23-7.
  3. ^ Zollinger RM, Ellison EH (1955). "Primary peptic ulcerations of the jejunum associated with islet cell tumors of the pancreas". Ann. Surg. 142 (4): 709–23; discussion, 724–8. doi:10.1097/00000658-195510000-00015. PMID 13259432.  
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Wiktionary

Up to date as of January 15, 2010

Definition from Wiktionary, a free dictionary

English

Noun

Wikipedia

Singular
Zollinger-Ellison syndrome

Plural
uncountable

Zollinger-Ellison syndrome (uncountable)

  1. (medicine) A disorder where increased levels of the hormone gastrin are produced, causing the stomach to produce excess hydrochloric acid; often caused by a tumor (gastrinoma) of the duodenum or pancreas, and resulting in peptic ulcers.

Derived terms

  • ZE (abbreviation)

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