Zollinger-Ellison Syndrome

Zollinger-Ellison syndrome (ZES) is a rare disorder that causes tumors in the pancreas and duodenum and ulcers in the stomach and duodenum. The pancreas is a gland located behind the stomach. It produces enzymes that break down fat, protein, and carbohydrates from food, and hormones like insulin that break down sugar. The duodenum is the first part of the small intestine.

The tumors secrete a hormone called gastrin that causes the stomach to produce too much acid, which in turn causes stomach and duodenal ulcers (peptic ulcers). The ulcers caused by ZES are less responsive to treatment than ordinary peptic ulcers. What causes people with ZES to develop tumors is unknown, but approximately 25 percent of ZES cases are associated with a genetic disorder called multiple endocrine neoplasia type 1, which is associated with additional disorders.

The symptoms of ZES include signs of peptic ulcers: gnawing, burning pain in the abdomen; diarrhea; nausea; vomiting; fatigue; weakness; weight loss; and bleeding. Physicians diagnose ZES through blood tests to measure levels of gastrin and gastric acid secretion. They may check for ulcers by doing an endoscopy, which involves looking at the lining of the stomach and duodenum through a lighted tube.

The primary treatment for ZES is medication to reduce the production of stomach acid. Proton pump inhibitors that suppress acid production and promote healing are the first line of treatment and include lansoprazole, omeprazole, pantoprazole, and rabeprazole. H-2 blockers such as cimetidine, famotidine, and ranitidine may also be used, but are less effective in reducing stomach acid. Surgery to treat peptic ulcers or to remove tumors in the pancreas or duodenum are other treatment options. People who have been treated for ZES should be monitored in case the ulcers or tumors recur.

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For More Information

National Organization for Rare Disorders Inc. (NORD)
55 Kenosia Avenue
P.O. Box 1968
Danbury, CT 06813–1968
Phone: 1–800–999–6673 or 203–744–0100
Fax: 203–798–2291
Email: orphan@rarediseases.org
Internet: http://www.rarediseases.org/

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  Additional Information on Zollinger-Ellison Syndrome

The National Digestive Diseases Information Clearinghouse collects resource information on digestive diseases for National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) Reference Collection. This database provides titles, abstracts, and availability information for health information and health education resources. The NIDDK Reference Collection is a service of the National Institutes of Health.

To provide you with the most up-to-date resources, information specialists at the clearinghouse created an automatic search of the NIDDK Reference Collection. To obtain this information, you may view the results of the automatic search on Zollinger-Ellison Syndrome.

If you wish to perform your own search of the database, you may access and search the NIDDK Reference Collection database online.

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National Digestive Diseases Information Clearinghouse

2 Information Way
Bethesda, MD 20892–3570
Email: http://digestive.niddk.nih.gov/about/contact.htm

The National Digestive Diseases Information Clearinghouse (NDDIC) is a service of the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK). The NIDDK is part of the National Institutes of Health of the U.S. Department of Health and Human Services. Established in 1980, the Clearinghouse provides information about digestive diseases to people with digestive disorders and to their families, health care professionals, and the public. The NDDIC answers inquiries, develops and distributes publications, and works closely with professional and patient organizations and Government agencies to coordinate resources about digestive diseases.

Publications produced by the Clearinghouse are carefully reviewed by both NIDDK scientists and outside experts.

This publication is not copyrighted. The Clearinghouse encourages users of this publication to duplicate and distribute as many copies as desired.

NIH Publication No. 05–4332
November 2004

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