Zollinger ellison syndrome

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1. Zollinger-Ellison syndrome ALI NAJAT JABBAR KIRKUK MEDICAL COLLAGE

2. INTRODUCTION Rare disorder characterized by the triad of sever peptic ulceration, gastric acid hypersecretion and neuroendocrine tumour of the pancreas or duodenum. It accounts for about 0.1%of all cases of duodenal ulceration. It occurs in either sex at any age. Most commonly between 30 and 50 years of age.

3. Pathophysiology The tumour secretes gastrin, which stimulate acid secretion to its maximal capacity and increases the parietal cell mass three to six fold. The acid output may be so great that it reaches the upper small intestine, reducing the luminal PH to 2 or less (normally 5.5-7). Pancreatic lipase inactivated (because of hyperacidity) and bile acid precipitate  diarrhea and steatorrhea result. Between 20%-60% of patients have multiple endocrine neoplasia type 1 (MEN1)

4. Clinical features Multiple peptic ulcers Poor response to standard ulcer therapy Bleeding Perforation Nausea Diarrhea Loss of appetite

5. Investigations Serum gastrin elevated (10-100 fold). Normally, injection of hormone secretin causes no change or slight decrease in circulating gastrin concentration, but in Zollinger syndrome it produces a paradoxical and dramatic increase in gastrin. Tumour localization is best achieved by a combination CT and EUS. Radio-labelled somatostatin receptor scintigraphy and gallium DOTATATE PET scanning may also be used.

6. Management Surgery: for single and localized tumour but many are multifocal and metastasized in which circumstances surgery is inappropriate unless there is complication such as GIT obstruction Medications: omeprazole or other PPI can be used in healing ulcers and alleviating diarrhea. Octreotide given by subcutaneous injection, reduces gastrin secretion