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  Zollinger Ellison Syndrome

Zollinger-Ellison Syndrome causes one or more tumors in the duodenum or the pancreas. The tumors are known as gastrinomas and secrete the hormone gastrin in large amounts. This hormone leads to extreme production of acid in the stomach eventually causing peptic ulcers. There usually are many ulcers in the area affected.

Zollinger Ellison Syndrome
Zollinger Ellison Syndrome


This is an extremely rare condition, affecting one in one million people in North America. The disease can begin at anytime, but patients are not usually diagnosed until later in life, around fifty years old.

Symptoms:

Symptoms for this disease are numerous ulcers in atypical areas of the stomach or intestine. These ulcers are extremely resistant to treatment compared to typical gastric ulcers; returning after treatment. Symptoms for Zollinger-Ellis Syndrome are diarrhea, nausea and vomiting, weight loss, and burning, aching or discomfort below the sternum. The symptoms are continuous and usually severe. Typical antacids provide no relief whatsoever.

Diagnosis:

Diagnosis for Zollinger-Ellison Syndrome is determined via physical exam, which includes a blood test, endoscopy and imaging. A blood test is used to determine if increased gastrin levels are present in the blood stream. Higher levels of gastrin can be an indicator of tumors on the pancreas or duodenum. An upper gastrointestinal endoscopy or Upper GI, is a procedure in which a flexible tube with a camera and light are inserted down the throat to the stomach and duodenum. The procedure gives the physician the opportunity to examine the affected area closely and possibly remove tissue (biopsy) for pathological evaluation. The tissue can be examined to determine if it is gastrin producing. Radioactive tracers can be used to detect the location of the tumors. The patient ingests the tracer before a CT scan or MRI (magnetic resonance imaging) is performed.

An endoscopic ultra sound is an ultrasound that is performed within the digestive tract. An endoscope (flexible tube) with an ultrasound probe is inserted through the mouth and down to the stomach and duodenum. A biopsy can also be taken while performing this diagnostic procedure.

Causes:

This syndrome is caused by tumor or tumors growing in the pancreas or duodenum, the reason they begin to grow is unknown. The pancreas is situated below and behind the stomach and next to the duodenum. This organ creates hormones. Insulin is a hormone that regulates blood sugar levels. Gastrin is a hormone that regulates the production of stomach acid. In the duodenum (the small intestine just after the stomach) digestive enzymes and juices from the gallbladder, liver and pancreas mix to digest the food. The tumors in Zollinger-Ellison syndrome create extreme amounts of gastrin causing the stomach to produce more acid than needed. The acid creates peptic ulcers and diarrhea.

The tumors may be cancerous or malignant. Thought the tumors are slow growing, the cancer may spread to other organs, such as the lymph nodes or liver. This disease could also be associated with multiple endocrine neoplasia type I, also known as MEN I. Patients who have MEN I have tumors through out the endocrine system, pancreas, parathyroid glands and possible pituitary gland. Patients with gastrinomas have a twenty five percent chance of having the MEN I condition.

Treatment:

In treating Zollinger-Ellison syndrome, doctors focus first on the tumors and then on the ulcers. Tumor treatment requires great skill because the tumors are numerous, small and difficult to locate. If there are tumors that have spread to the liver, surgery may not be recommended. Sometimes just the largest tumor may be removed.

Treatment to control the growth of tumors may include: thinning out especially larger tumors in the liver, embolization or destroying the blood supply to the tumor, radio frequency ablation, chemotherapy and drugs injected directly into the tumor to control symptoms. Radio frequency ablation is a procedure that used heat to kill cancer cells.

The next phase of treatment is directed at the ulcers. Excess acid production is controlled using proton pump inhibitors and acid blockers. Proton pump inhibitors have the most successful results for Zollinger-Ellison syndrome patients. These drugs reduce the amount of acid in the stomach by jamming the tiny pumps in acid-secreting cells. Acid blockers are also called histamine or H-2 blockers. The acid blockers prevent histamines from reaching the histamine receptors in the stomach – which then prevents the signal to produce more acid from being processed. These drugs do not work as well as the proton pump inhibitors. Peptic ulcers may also need to be treated with surgery to either stop bleeding, clear up an obstruction or close a hole that perforates the stomach or duodenum.

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