Zollinger Ellison Syndrome

Zollinger-Ellison syndrome: causes, symptoms and treatment

Zollinger-Ellison syndrome (ZES) is a rare disease that is caused by hypergastrinemia (increased levels of the hormone gastrin) and is one of the causes of peptic ulcers of the stomach and duodenum. In this article we will look at the causes, symptoms and treatment of this disease.

Causes

ZES can occur with hyperplasia of G-cells of the antrum of the stomach (type I syndrome) or with the development of a tumor from D-cells of the islet apparatus of the pancreas, which produce gastrin (type II syndrome - gastrinoma). In type II syndrome, gastrinoma can be located outside the pancreas, most often in the wall of the duodenum. Approximately 60% of islet tumors are malignant.

Symptoms

The main symptoms of ZES are abdominal pain and diarrhea. Abdominal pain is typical for peptic ulcer disease, but it is very intense and does not respond to antacid therapy. Diarrhea is pathogenetically associated with hypersecretion of hydrochloric acid. The degree of secretion of hydrochloric acid exceeds the possibility of its neutralization in the duodenum; the acidic contents enter the jejunum and cause the development of enteritis. As a result of hypergastrinemia, intestinal motility increases and water absorption in it decreases. The course of peptic ulcer disease in ZES syndrome is very severe: ulcers are often complicated by bleeding and perforation; relapses of peptic ulcer occur even after surgical treatment. The localization of ulcers is very diverse: the descending part of the duodenum, even the jejunum.

Diagnostics

In the diagnosis of ZES, the study of gastric secretion is important, which has a number of features: in 12 hours, the secretion of gastric juice exceeds 1500 ml; the level of basal secretion exceeds 15 mmol/h (normal 2-3 mmol/h); after administration of the maximum dose of histamine, there is no increase in hydrochloric acid production. The most reliable diagnostic method is radioimmunochemical determination of gastritis in blood plasma. If in a healthy person the gastritis content in plasma is 50-200 pc/ml, then in ZES syndrome it can exceed 1000-5000 pc/ml.

Treatment

ZES treatment is aimed at reducing the secretion of hydrochloric acid and reducing the level of gastritis in the blood. This is achieved through the use of drugs that block histamine reuptake and block histamine receptors. These drugs include ranitidine, famotidine, nizatidine and others. Surgical treatment may also be performed to remove a tumor of the islet apparatus of the pancreas or ulcers of the stomach and duodenum.

However, with malignant gastrinoma, surgical treatment may not be sufficient, since the tumor can metastasize to other organs. In such cases, chemotherapy and radiation therapy may be used.

In general, the prognosis of ZES depends on the cause of the disease and its severity. With timely detection and treatment, the prognosis is usually favorable, but in the case of a malignant tumor, the prognosis may be poor.