Facts About Zollinger-Ellison Syndrome (ZES): Symptoms, Causes and Treatment

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Overview

Zollinger-Ellison syndrome is a rare condition wherein the stomach produces an excessive amount of gastric acid resulting in the ulceration of the tissues surrounding the stomach, small intestines and esophagus.

In extreme cases it may also result in the spread of gastrinomas - a gastrin secreting tumor that invade the pancreas and the upper part of the small intestines.

Epidemiology

The disorder may occur in early childhood. However, it is seen more often in patients between 20 and 50 years of age. Almost 2/3 of the tumors are likely to develop into cancer. Some person are more susceptible to the the tumor that cause the disease while in some places occurring at irregular intervals for unknown reasons.

Twenty five percent of ZES patient have a genetic syndrome known as multiple endocrine neoplasia type 1 or (MEN-1). Prognosis of the disease is related to tumor sizes and distance of the spread of the tumor or Metastasis. If left untreated it may develop into a malignant tumor.

This syndrome was first described in 1955 by Dr. Robert Zollinger and Dr. Edwin Ellison, surgeons at Ohio State University.

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What are the signs and symptoms?

The symptom is associated with a feeling of a burning sensation and hunger in the upper trunk of the abdomen. It would take up to one to three hours after meals especially felt in the middle of the night. Compare to peptic ulcer the symptoms associated with Zollinger-Ellison syndrome are generally more painful and less responsive to treatment. The symptoms were the following;

• Peptic ulcer, abdominal pain, diarrhea, nausea, vomiting, fatigue, weakness, weight loss
• Gastrointestinal bleeding such as epigastric pain, reflux esophagitis, diarrhea
• Gastrin secreting pancreatic tumors: excess stomach acidity, stomach ulcers, duodenal ulcers, benign stomach tumor 
• Malignant stomach tumor, malignant duodenal tumor, malignant mesenteric tumor, malignant spleen tumor, and malignant abdominal lymph node tumor
• Benign duodenal tumor, benign mesenteric tumor, benign spleen tumor, and benign abdominal lymph node tumor
• Hepatomegaly and steatorrhea
• Excessive fat in the feces/stools

Treatment and Laboratory Aid to Diagnosis

• The patient should undergo Endoscopy per physician advice.
• The patient should undergo Secretin stimulation test to measures gastrin levels.
• The patient needs to undergo Fasting gastrin levels test.
• The patient needs to undergo Gastric acid secretion and pH test.
• The patient needs to undergo MRI to identify the excessive sources of gastrin or somatostatin receptor.
• Gastrectomy or removal of stomach in severe cases as advice by physician.
• Intake of Octreotide to reduce symptoms.
• Cure is only possible after surgery or chemotherapy after surgery.

The drugs of choice

• Apo-Cimetidine

• Acid Reducer 200

• Acid Reducer Cimetidine

• Alti-Ranitidine

• Apo-Ranitidine

• Aciphex

• Acimax

• Enlon

• Famotidine-histamine receptorantagonist

• Heartburn 200

• Heartburn Relief 200

• Klasec

• Lanzoprazole-proton inhibitor

• Losec

• Maxor

• Novo-Cimetine

• Nu-Cimet

• Novo-Ranidine

• Nu-Ranit

• Omeprazole-proton inhibitor

• Prilosec

• Pantoprazole

• Peptol

• Protonix

• Panto IV

• Pantoloc

• Pantozol

• Pariet

• Probitor

• Ranitidine-histamine antagonist receptor

• Risek

• Rebeprazole

• Rabeprazole

• Somac Tagamet

• Tagamet HB 200

• Zantac

• Zantac-C

• Zantac 75

• Zantac 75 EFFERdose

• Zurcal

• Zoton

Nursing care:

• The patient to be schedules for surgery should be prepared psychologically in order to calm him.
• The patient should be told the type of surgery that is plan and the reason for it.
• A priest or clergy may be helpful to prepare the patient to learn to accept the outcome of the surgery.

Prevention

1. Keep away from excessive consumption of spicy food and coffee.
2. Take care of the body by having healthy lifestyles such as avoiding smoking and alcohol intake.
3. Reduce intake of NSAIDs and paracetamol.
4. Keep a normal weight.
5. Intake of appropriate prescription drugs to reduce the stomach acidity such as antagonist drugs like ranitidine (Zantac) and cimetidine (Tagamet).
6. Intakes of drugs such as the proton pump inhibitors to reduce the production of stomach acid.
7. Antibiotics

• Never take medicines that have been prescribed for someone else even if your symptoms are similar. Consult your physician or Health Care Professional for more advice.

References:

1. Zollinger RM, Ellison EH (1955). "Primary peptic ulcerations of the jejunum associated with islet cell tumors of the pancreas". Ann. Surg. 142 (4): 709–23; discussion, 724–8. doi:10.1097/00000658-195510000-00015. PMID 13259432.

2. Mamura M, Komoto I, Ota S. Changing Treatment Strategy for Gastrinoma in Patients with Zollinger-EllisonSyndrome. World J Surg. Dec 13 2005;[Medline].

3. Jensen RT (2004). "Gastrinomas: advances in diagnosis and management". Neuroendocrinology 80 Suppl 1: 23–7. doi:10.1159/000080736. PMID 15477712.