Zollinger Ellison syndrome

What is Zollinger Ellison syndrome?

Zollinger Ellison syndrome (ZES) is characterised by the excessive production of the hormone gastrin due to one or more tumours in the pancreas or small intestine. These tumours secrete the hormone gastrin, which is responsible for the production of gastric acid in the stomach. The normal level of gastrin in a human body is needed to produce gastric acid for the breakdown of food in the stomach. The excessive gastric acid in ZES causes peptic ulcers in the stomach and other parts of the digestive tract.

What are its main signs and symptoms?

The symptoms of ZES are:

• Diarrhoea
• Vomiting
• Abdominal pain
• Ulcers in the stomach
• Bloating
• Weight loss
• Loss of appetite
• Burping
• Ulcers in the small intestine

Contact a doctor immediately if blood in stool or vomit is seen.

What are the main causes?

In most individuals, the cause of ZES remains hidden; however, in about 25% of the population with ZES, a genetic disorder called multiple endocrine neoplasia type 1 (MEN1) is seen to be the cause. MEN1 causes gastrinomas, hormone gastrin-producing tumours, which increases the production of acid in the stomach.

How is it diagnosed and treated?

The diagnosis of the condition is generally made in the following manner:

• Study of detailed medical history
• Physical examination
• Blood test to detect the level of the hormone gastrin
• Upper gastrointestinal endoscopy to check for inflammation and ulcers in the upper part of the digestive tract, i.e., food pipe, stomach, and small intestine
• Imaging tests like computed tomography (CT) scan and magnetic resonance imaging (MRI) to get a current picture of the digestive tract
• Determining the levels of stomach acid

The doctor may recommend the following treatment methods to get rid of ZES:

• Medicines: Proton pump inhibitors like pantoprazole, rabeprazole, esomeprazole, etc., are prescribed to inhibit the production of acid in the stomach. It provides relief from the pain, ulcer, and other symptoms of ZES.
• Chemotherapy: Chemotherapy drugs like doxorubicin are prescribed to treat ZES tumours, which cannot be cured by surgical procedures.
• Surgical procedure: Surgical removal of the gastrinomas helps in treating the syndrome.
• Diet: Follow the diet suggested by the doctor to prevent the re-occurrence of these tumours.

1. MedlinePlus Medical Encyclopedia: US National Library of Medicine; Zollinger-Ellison syndrome
2. National Organization for Rare Disorders [Internet], Zollinger-Ellison Syndrome
3. National Institute of Diabetes and Digestive and Kidney Diseases [internet]: US Department of Health and Human Services; Zollinger-Ellison Syndrome.
4. National Center for Advancing and Translational Sciences. Zollinger-Ellison syndrome. Genetic and Rare Diseases Information Center
5. Paola Tomassetti et al. Treatment of Zollinger-Ellison Syndrome. World J Gastroenterol. 2005 Sep 21; 11(35): 5423–5432. PMID: 16222731