Zollinger Ellson syndrome

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Overview

Zollinger-Ellison syndrome is a rare condition in which one or more tumors form in your pancreas or the upper part of your small intestine (duodenum). These tumors, called gastrinomas, secrete large amounts of the hormone gastrin, which causes your stomach to produce too much acid. The excess acid then leads to peptic ulcers, as well as to diarrhea and other symptoms.

Zollinger-Ellison syndrome may occur at any time in life, but people usually find out they're affected between ages 20 and 60. Medications to reduce stomach acid and heal the ulcers are the usual treatment for Zollinger-Ellison syndrome.

Symptoms

Symptoms are generally digestive in nature. The two most commonTrusted Source symptoms of ZES are abdominal pain and diarrhea. Other symptoms include:

bloating and burping

nausea

vomiting, which may have blood in it if peptic ulcers are present

weight loss for no apparent reason

loss of appetite

ZES symptoms are sometimes confused with the more common peptic ulcer disease or gastroesophageal reflux disease gastroesophageal reflux disease (GERD). GERD is a chronic condition in which stomach acid backwashes into the esophagus. ZES symptoms tend to be more severe and less responsive to standard therapies than these other disorders

Causes

The exact cause of Zollinger-Ellison syndrome remains unknown. But the sequence of events that occurs in Zollinger-Ellison syndrome is clear. The syndrome begins when one or more tumors (gastrinomas) form in your pancreas or duodenum or at other sites such as the lymph nodes adjacent to your pancreas.

Your pancreas sits behind and below your stomach. It produces enzymes that are essential to digesting food. The pancreas also produces several hormones including insulin, a hormone that helps to control your blood glucose.

Digestive juices from the pancreas, liver and gallbladder mix in the duodenum, the part of the small intestine next to your stomach. This is where digestion reaches its peak.

The tumors that occur with Zollinger-Ellison syndrome are made up of cells that secrete large amounts of the hormone gastrin. Increased gastrin makes the stomach produce far too much acid. The excess acid then leads to peptic ulcers and sometimes to diarrhea.

Besides causing excess acid production, the tumors are often cancerous (malignant). Although the tumors tend to grow slowly, the cancer can spread elsewhere — most commonly to nearby lymph nodes or your liver.

Association with MEN 1

Zollinger-Ellison syndrome may be caused by an inherited condition called multiple endocrine neoplasia, type 1 (MEN 1). People with MEN 1 also have tumors in the parathyroid glands and may have tumors in their pituitary glands.

About 25% of people who have gastrinomas have them as part of Men 1. They may also have tumors in the pancreas and other organs.

Risk factors

Just about anyone can get ZES. But, some people with the condition may have a genetic problem known as multiple endocrine neoplasia type 1 (MEN1). Children of adults with MEN1 are at an greater risk of getting the disease.

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Complications

Complications of Zollinger-Ellison syndrome are related to the overproduction of stomach acid and to the severity of the peptic ulcers. Severe peptic ulcers can: Penetrate to an artery, causing internal bleeding. Cause swelling, resulting in an obstruction (blockage) in the path of food leaving the stomach.

Prevention

People at higher risk for Zollinger-Ellison syndrome include males ages 25–50 and people who have a parent with the disorder. Zollinger-Ellison syndrome cannot be prevented.

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