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Zollinger-Ellison syndrome

Zollinger-Ellison syndrome is a condition characterised by multiple ulcers in the GI tract secondary to a gastrinoma(s). 

The commonest location of the gastrinoma is the duodenum (75%) followed by the pancreas (25%).

Studies suggest between 20 and 65% of gastrinomas are malignant. 

25% of cases of Zollinger-Ellison syndrome are associated with MEN-1 

Presentation: 
  • Abdominal pain from ulcers 
  • Gastric reflux
  • Diarrhoea 
  • Steatorrhoea and malabsorption – due to inactivation of pancreatic enzymes and damage to enterocytes by acid

Diagnosis:
  • Raised fasting gastrin level 
  • However, hypochlorhydria (due to chronic atrophic gastritis or prolonged PPI use) will also cause a raised fasting gastrin level. To differentiate IV secretin can be administered – this will result in a raised gastrin level in Zollinger-Ellison syndrome but no rise in achlorhydria. 

Treatment: 
  • High dose PPI 
  • Ocreotide (somatostatin analague) – reduces gastrin secretion 
  • Possibly surgery

References:
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Abdelghafour

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