Zollinger-Ellison Syndrome- Monday Zebra

Condition- Zollinger-Ellison Syndrome

Pronunciation- Zoh-Ling-Ger  El-Lis-Son

Rarity- very rare

Difficulty in Diagnosis- Often tricky to diagnose due to the rarity and for that reason is often one of the last things checked for.  Blood testing for certain enzyme levels is the most common way it is diagnosed.

     Zollinger-Ellison Syndrome (ZES), first described in 1955 by Robert Zollinger and Edwin Ellison, surgeons at The Ohio State University, is caused by a gastrin-secreting micro-tumor which causes the stomach to "ramp up" acid secretion to "maximum" and is suspected when treatment for gastric ulcers that are not caused by h pylori (Helicobactor Pylori) is unsuccessful and physicians start to look for other causes.

     People with Zollinger–Ellison Syndrome may experience upper abdominal pain and diarrhea though not all patients have these symptoms. The diagnosis is also suspected in patients without symptoms who have severe ulceration of the stomach and small bowel, especially if they fail to respond to treatment and have a negative biopsy for h pylori (taken during an upper endoscopy exam).

     Gastrinomas may occur as single tumors or as multiple, small tumors which may not be visible during an upper endoscopy exam. About one-half to two-thirds of single gastrinomas are malignant tumors that most commonly spread to the liver and lymph nodes near the pancreas and small bowel. Nearly 25 percent of patients with gastrinomas have multiple tumors as part of a condition called multiple endocrine neoplasia type I (MEN I). 

     Symptoms for Zollinger-Ellison Syndrome include-

• chronic diarrhea 
• pain in the esophagus (especially after meals at night)
• nausea
• wheezing
• heartburn
• vomiting digested blood (looks like coffee grounds)
• malnutrition
• weight loss

     Testing for ZES is almost exclusively done through laboratory blood testing and includes-

• Secretin stimulation test, which measures evoked gastrin levels
• Fasting gastrin levels, on at least three separate occasions
• Gastric acid secretion and pH. Normal basal gastric acid secretion is less than 10 mEq/hour, while in Zollinger–Ellison syndrome it is usually more than 15 mEq/hour.
• Increased level of Chromogranin A is a common marker of endocrin tumors

     Treatment for ZES includes use of Proton pump inhibitors (such as omeprazole and lansoprazole) and histamine H2-receptor antagonists (such as famotidine or andranitidine) are used to slow down acid secretion. A cure is only possible if the tumors are surgically removed, or treated with chemotherapy.