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Zollinger-Ellison Syndrome

A group of symptoms that occur when a tumor called a gastrinoma forms. The tumor, which can be cancerous, releases large amounts of the hormone called gastrin. The gastrin causes too much acid in the duodenum, resulting in ulcers, bleeding, and perforation.

PubMed Health Glossary
(Source: NIH - National Institute of Diabetes and Digestive and Kidney Diseases)

About Zollinger-Ellison Syndrome

Zollinger-Ellison syndrome is a rare disorder that occurs when one or more tumors form in the pancreas and duodenum. The tumors, called gastrinomas, release large amounts of gastrin that cause the stomach to produce large amounts of acid. Normally, the body releases small amounts of gastrin after eating, which triggers the stomach to make gastric acid that helps break down food and liquid in the stomach. The extra acid causes peptic ulcers to form in the duodenum and elsewhere in the upper intestine.

The tumors seen with Zollinger-Ellison syndrome are sometimes cancerous and may spread to other areas of the body.

What are the stomach, duodenum, and pancreas?

The stomach, duodenum, and pancreas are digestive organs that break down food and liquid.

NIH - National Institute of Diabetes and Digestive and Kidney Diseases

What works? Research summarized

Evidence reviews

Drug Class Review: Proton Pump Inhibitors: Final Report Update 5 [Internet]

Proton pump inhibitors decrease secretion of gastric acid. They act by blocking the last enzyme in the system that actively transports acid from gastric parietal cells into the gastrointestinal lumen, hydrogen–potassium adenosine triphosphatase, also known as the proton pump. Omeprazole, the first drug in this class, was introduced in 1989. Since then, 4 other proton pump inhibitors have been introduced: lansoprazole (1995), rabeprazole (1999), pantoprazole (2000), and esomeprazole (2001). In 2003 omeprazole became available over-the-counter in the United States. The purpose of this review is to compare the benefits and harms of different PPIs.

Additional bedtime medication for the control of night‐time acid reflux from the stomach.

The inhibition of gastric acid secretion is an accepted treatment for diseases related to reflux of acid from the stomach. Some types of antacids, known as proton pump inhibitors (PPI), are considered to be the most effective medical treatment for people patients with acid‐related diseases such as peptic ulcer, gastro‐oesophageal reflux disease (GERD) and Zollinger‐Ellison syndrome, but they may not reduce gastric acid secretion sufficiently to prevent night‐time acid reflux symptoms. H2‐receptor antagonists (H2RAs) have also been used for the treatment of acid‐related diseases for more than a decade and might help to control night‐time acid reflux, if taken at bedtime along with a high dose of PPI. The results show that additional bedtime H2RAs can decrease the night‐time gastric acid breakthrough, but we believe that additional bedtime H2RAs to PPI should only be used as treatment an intervention in clinical trials until further evidence has been found.

Effect of the long‐term use of proton pump inhibitors on the rate of pre‐cancerous lesions in the stomach

PPIs are the most effective drugs used to reduce gastric acid secretion (called antacids) and they are commonly prescribed worldwide. Although generally safe, their effectiveness and safety for long‐term use remains unclear. It has been suggested that the long‐term use of PPIs could promote the development of pre‐cancerous lesions in the stomach, which might subsequently increase the occurrence of stomach cancer. Therefore, the safety issues of long‐term PPI treatment needs to be addressed.

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Summaries for consumers

Additional bedtime medication for the control of night‐time acid reflux from the stomach.

The inhibition of gastric acid secretion is an accepted treatment for diseases related to reflux of acid from the stomach. Some types of antacids, known as proton pump inhibitors (PPI), are considered to be the most effective medical treatment for people patients with acid‐related diseases such as peptic ulcer, gastro‐oesophageal reflux disease (GERD) and Zollinger‐Ellison syndrome, but they may not reduce gastric acid secretion sufficiently to prevent night‐time acid reflux symptoms. H2‐receptor antagonists (H2RAs) have also been used for the treatment of acid‐related diseases for more than a decade and might help to control night‐time acid reflux, if taken at bedtime along with a high dose of PPI. The results show that additional bedtime H2RAs can decrease the night‐time gastric acid breakthrough, but we believe that additional bedtime H2RAs to PPI should only be used as treatment an intervention in clinical trials until further evidence has been found.

Effect of the long‐term use of proton pump inhibitors on the rate of pre‐cancerous lesions in the stomach

PPIs are the most effective drugs used to reduce gastric acid secretion (called antacids) and they are commonly prescribed worldwide. Although generally safe, their effectiveness and safety for long‐term use remains unclear. It has been suggested that the long‐term use of PPIs could promote the development of pre‐cancerous lesions in the stomach, which might subsequently increase the occurrence of stomach cancer. Therefore, the safety issues of long‐term PPI treatment needs to be addressed.

Gastrointestinal Carcinoid Tumors Treatment (PDQ®): Patient Version

Expert-reviewed information summary about the treatment of gastrointestinal carcinoid tumors.

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Terms to know

Duodenum
The first part of the small intestine that connects to the stomach.
Endoscopy
A procedure that uses an endoscope to examine the inside of the body. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue to be checked under a microscope for signs of disease.
Gastrin
A hormone released from special cells in the lining of the stomach after eating. Gastrin causes the stomach to release an acid that helps digest food.
Gastrinoma
A tumor that causes overproduction of gastric acid.
Imaging Tests
A type of test that makes pictures of areas inside the body. Some examples of imaging tests are CT scans and MRIs. Also called imaging procedure.
Pancreas
An organ that makes insulin and enzymes for digestion. The pancreas is located behind the lower part of the stomach and is about the size of a hand.
Peptic Ulcer
A sore on the lining of your stomach or duodenum.
Perforation
A hole in the wall of an organ.

More about Zollinger-Ellison Syndrome

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Also called: Zollinger Ellison syndrome, ZE, ZES

Other terms to know: See all 8
Duodenum, Endoscopy, Gastrin

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