Zollinger–Ellison syndrome

Zollinger–Ellison syndrome 
First described in 1955 by Robert Zollinger and Edwin Ellison, surgeons at The Ohio State University.
Main features of this syndrome:

  • Gastric acid hypersecretion
  • Severe peptic ulceration
  • Non-beta cell islet tumor of pancreas
In this syndrome increased levels of the hormone Gastrin are produced, causing the stomach to produce excess hydrochloric acid resulting to severe peptic ulceration. The ulcers are formed in the stomach and in the duodenum. The hormone Gastrin is produced by the Non-beta cell tumor in pancreas. At times this tumorcalled Gastrinoma may be located in the duodenum.Gastrin acts on the parietal cells of the stomach and causes the hyperplasia of the parietal cells resulting to increase in the number of the acid secreting cells. It also acts by causing the increase in the output of the acid by the parietal cells. All this results to increase in the production of acid in the stomach and causes the multiple ulcers in the stomach and the duodenum.Gastrinomas may be single of multiple tumors. They may be malignant tumors and spread to Liver, lymphnode in the abdomen and in the small intestine.Nearly 25 percent of patients with gastrinomas have multiple tumors. Such patients have tumors in their pituitary gland and parathyroid glands in addition to tumors of the pancreas.
Symptoms of this condition consist of: Pain in epigastrium, upper abdomen. Diarrhea, Melina (Blood in stool). Vomiting, Weight Loss.This condition is suspected when the symptoms do not resolve with routine treatment.Tests which help in diagnosing this disease are

  • Upper Gastro intestinal Endoscopy, Measurement of Gastric acid secretion. Normal basal gastric acid secretion is less than 10 mEq/hour, while in Zollinger–Ellison syndrome it is usually more than 15 mEq/hour.
  • Level of hormone Gastrin, especially in fasting state.
  • Secretin stimulation test. This measures the level of gastrin after stimulation.

Secretin is a hormone that controls the secretions into the duodenum, and also water homeostasis  in the body. It is produced in the S cells of the duodenum in the crypts of Lieberkühn. Its effect is to regulate the pH of the duodenal contents via the control of gastric acid secretion.

  • Ultrasound, CT Scans and MRI help in diagnosis.
Treatment consists of Proton Pump inhibitors such as omeprazole, rabiprazole, and H2 receptor antagonists such as famotidine and ranitidine.Tumors have to be removed surgically and chemotherapy may be needed.
 Diseases & Conditions
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