This book describes Zollinger-Ellison Syndrome, Diagnosis and Treatment and Related Diseases
Zollinger-Ellison syndrome (ZES) is an endocrine disorder featured by gastrin-secreting tumors, which produce multiple, refractory and repeat peptic ulcers in the distal duodenum and proximal jejunum.
There are two main types:
1. Sporadic (isolated).
2. ZES linked disorders with parathyroid and pituitary tumors as part of the genetic disorder multiple endocrine neoplasia type 1 (MEN1).
The tumor (gastrinoma) is normally in:
1. The duodenum (60-65%) or
2. The pancreas (30%).
Rarely, gastrinomas happen in other abdominal sites (e.g., the stomach, liver, bile duct, ovary) and also extra-abdominal sites (e.g., the heart, lung - small cell lung cancer).
Causes
Zollinger-Ellison syndrome is produced by tumors.
These tumors are most often present in the head of the pancreas and the upper small intestine.
The tumors are termed gastrinomas
Besides secreting high levels of gastrin, these tumors may secrete other hormones such as:
1. Adrenocorticotrophic hormone (ACTH),
2. Vasoactive intestinal polypeptide (VIP).
They can also form a range of peptides, such as:
1. Insulin,
2. Pancreatic polypeptide,
3. Glucagons,
4. Chromogranin A,
5. Neuron-specific enolase, and
6. The alpha and beta subunits of human chorionic gonadotrophin (hCG).
High levels of gastrin produce formation of too much stomach acid.
Symptoms
1. Abdominal pain
2. Diarrhea
3. Vomiting blood
Signs are ulcers in the stomach and small intestine.
Diagnosis:
It is very frequent for ZES to be treated early as a simple peptic ulcer.
The combination with pain with diarrhea may indicate the diagnosis.
Hyper-gastrinemia is described as fasting serum gastrin concentration >100 pg/ml and is seen often in medical disorders other than ZES such as:
1. Proton pump inhibitor (PPI) or H2-receptor antagonist therapy,
2. Post-vagotomy,
3. Renal failure and
4. Chronic atrophic gastritis.
If ZES is confirmed, it is important to determine MEN1, and calcium, parathyroid hormone and prolactin levels should be evaluated.
If the diagnosis is indicated, then more specific tests are needed - there is one algorithm from Liverpool:
Assessment of fasting gastrin level - levels >1000 pg/ml with acidic gastric juice (pH200 pg/ml 15 minutes after the dose is administered.
Treatment
Medicines called proton pump inhibitors (omeprazole, lansoprazole, and others) are given to decrease acid production by the stomach.
This reduction of acid helps the ulcers in the stomach and small intestine to heal.
In patients with liver metastases, surgery should be done followed by chemotherapy.

TABLE OF CONTENT
Introduction
Chapter 1 Zollinger-Ellison Syndrome
Chapter 2 Causes
Chapter 3 Symptoms
Chapter 4 Diagnosis
Chapter 5 Treatment
Chapter 6 Prognosis
Chapter 7 Barrett Esophagus
Chapter 8 Gastro-esophageal Reflux Disease
Epilogue