| Zollinger-Ellison syndrome 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
top part of the small intestine.
The tumors are cancerous in 50 percent of cases. They secrete a
substance called gastrin that causes the stomach to produce too much acid,
which in turn causes the stomach and duodenal ulcers (peptic ulcers). The
ulcers caused by Zollinger-Ellison syndrome are more painful and less
responsive to treatment than ordinary peptic ulcers. What causes people
with Zollinger-Ellison syndrome to develop tumors is unknown, but the
cause may be an abnormal tumor suppressor gene.
Zollinger-Ellison syndrome usually occurs in people between ages 30 and
60. Symptoms of Zollinger-Ellison syndrome include signs of peptic ulcers: gnawing, burning pain in the
abdomen; diarrhea; nausea; vomiting; fatigue; weakness; and weight loss.
Physicians diagnose Zollinger-Ellison syndrome through blood tests to
measure levels of gastrin. They may check for ulcers by taking x-rays of
the stomach and duodenum or by doing an endoscopy, which involves looking
at the lining of these organs through a lighted tube.
Medications used to reduce stomach acid include cimetidine, ranitidine,
famotidine, and omeprazole. Surgery to treat peptic ulcers or to remove
tumors in the pancreas or duodenum are other treatment options. In serious
cases, surgery to remove the entire stomach may be necessary.
More information is available from
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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