Zollinger-Ellison Syndrome

Definition

Zollinger-Ellison syndrome is a medical condition characterized by excessive levels of gastrin, a hormone. Usually tumors located in the pancreas or small bowel are responsible for excess gastrin production. The symptoms of Zollinger-Ellison syndrome occurs due to the presence of excess gastrin which is responsible for increased acid secretion by the cells of the stomach leading to peptic ulceration. Common symptoms are diarrhea, nausea, vomiting and abdominal pain.

The disease is rare and usually affects people between the age of 30 and 50 years. Zollinger-Ellison syndrome can occur as a sporadic disease or may occur as a part of hereditary multiple endocrine neoplasia 1 (MEN1) where tumors are present in other hormone-producing organs like the pituitary or parathyroid glands. Treatment options include surgical removal of the tumors along with use of acid-suppressing drugs. Not all tumors can be removed with surgery in certain instances and treatment should focus on minimizing the development of complications such as ulcers.

Symptoms

Common symptoms include :

  • Upper abdominal pain along with discomfort. This is the most common symptom associated with Zollinger-Ellison syndrome. It usually occurs more commonly in male patients and people with sporadic cases of Zollinger-Ellison syndrome.
  • Diarrhea is the next most common symptom of Zollinger-Ellison syndrome after abdominal pain. This symptom is more common among females and people with Zollinger Ellison syndrome as a part of MEN 1.
  • Heartburn is the third most common symptom that occurs as a result of gastric acid irritating the esophagus.

Other common digestive symptoms include nausea, vomiting, bleeding in the digestive tract characterized by blood vomiting or bloody diarrhea and unintentional weight loss. Patients with MEN1 syndrome present with other symptoms like kidney stones due to raised calcium level and so on. About 5% people with Zollinger-Ellison syndrome develop a number of complications like perforation in the digestive tract because of acidity, narrowing of the esophagus (esophageal stricture) and blockage of the stomach outlet (gastric outlet obstruction).

Causes

Zollinger-Ellison syndrome is a medical condition characterized by the presence of a gastrin-secreting tumor in the pancreas. Raised gastrin levels are responsible for the symptoms of Zollinger-Ellison syndrome. Gastrin stimulates as well as increases the number of acid-secreting gastric parietal cells, ultimately leading to raised levels of gastric (stomach) acid. This leads to ulceration in the stomach and in the upper part of the duodenum, diarrhea and malabsorption which contributes to weight loss.

Malabsorption also occurs because of damage to the gastric mucosal lining and neutralization of the alkaline pancreatic enzymes essential for digestion of food. Zollinger-Ellison syndrome can occur in two forms :

  • sporadic when there is only gastrin0producing tumor (gastrinoma), or
  • associated with tumors of pituitary glands, parathyroid and pancreas (MEN 1) syndrome.

Treatment

Treatment options include surgical removal of tumors and treatment of stomach ulcers. Often due to presence of multiple tumors complete surgical removal of the tumors are not possible. Therefore treatment has to focus on limiting excess stomach acid with the use of acid-suppressing drugs like proton pump inhibitors (omeprazole). Any other factors that may contribute to raised gastric acid secretion should be minimized or prevented.

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