Zollinger Ellison syndrome is a condition in which there is an elevated level of a hormone called gastrin. It is due to overproduction of the hormone. Gastrinoma which is a tumour of the pancreas or small intestine is responsible for the overproduction of the gastrin.
Causes: Gastrinoma is a tumour responsible for the production of gastrin. These tumours are found in the head of the pancreas and the upper part of the small intestine. It may occur singly or as multiple tumours. Gastrin stimulates production of acid in the stomach. Therefore high levels of gastrin in the blood causes increased production of acid in the stomach. Half to two thirds of the single gastrinomas are malignant and can metastasise to the liver and nearby lymph nodes. A patient having multiple tumours falls in the MEN type 1 condition. MEN type 1 stands for multiple endocrine neoplasia type 1. In this condition the patient has multiple tumours in the pituitary gland which is present in the skull, parathyroid gland and pancreas. Thus patients having tumours in all these locations are said to be suffering from MEN type 1.
Almost 80 % of the tumours located in the gastrinoma triangle, which is bordered by the neck of the pancreas, the confluence of the cystic and common bile ducts and the third part of the duodenum.
Symptoms of Zollinger Ellison syndrome: Patients complain of abdominal pain varying from mild to severe degree, vomiting, gastroesophageal reflux disease and diarrhoea. Occasionally patients may even have haematemesis. The patient suffers from severe acid peptic disease. It must be suspected in patients having severe acid peptic disease and are refractory to therapy. They may have steatorrhoea. As a part of MEN 1 the patient may have hypercalcaemia, nephrolithiasis, hyperprolactinaemia, or hypopituitarism. Men are more commonly affected with Zollinger Ellison syndrome than females and the mean age of presentation is 45 to 50 years.
Signs and diagnostic tests for Zollinger Ellison syndrome: On examination, patients may have tenderness in the epigastrium or hypochondriac regions. The amount of tenderness elicited would depend on the severity of the condition. Patients have high fasting serum gastrin levels and show an exaggerated response to intravenous bolus of secretin and basal acid hypersecretion. Another method to establish the diagnosis is the presence of hypergastrinemia is to measure the basal acid output over 1 hour. This is done by aspiration of the gastric contents with the help of a nasogastric tube. Another test is the secretin provocative test and the levels of gastrin are measured after the administration of an Intravenous bolus of secretin. Abdominal CT scan, MRI studies are other tests which must be done in order to localise the tumour. If further confirmation of the location of the tumour is required the patient may need to undergo laparotomy or intraoperative endoscopy.
Treatment of zollinger Ellison syndrome: Proton Pump inhibitors (PPI’s) are widely used nowadays to treat acid peptic disease with good results. PPI’s like omeprazole, pantoprazole, lansoprazole are widely used. These help control the production of acid in the stomach and help in healing the ulcers in the stomach and relieve the symptoms. Some patients may undergo surgery to excise the gastrinoma’s, provided it has not metastasised.
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