Zollinger-Ellison Syndrome: A Clinical Overview

, Baylor College of Medicine and VAMC, Houston, Tex.

Disclosures
In This Article

Clinical Features of ZES

The estimated incidence of ZES in the United States is about 1 case per million individuals per year and about 0.1%-1% among patients with peptic ulcer disease.[6] The mean age at presentation is 45-50 years, and men are affected more often than women. Because of the rarity of the disease, the average interval between onset of symptoms and diagnosis is about 6 years. Most gastrinomas are malignant; therefore, a high index of suspicion remains key to proper and prompt management of the disease. Management is aimed at cure. ZES may present in one of several ways:

Peptic ulcer disease: This disease is present in 90%-95% of patients with gastrinomas. Patients who are Helicobacter pylori infection-negative and have no history of nonsteroidal anti-inflammatory drug use may have ZES. Peptic ulcers associated with ZES tend to be more persistent and less responsive to therapy than those not associated with ZES. Ulcers occurring in the second, third, or fourth portions of the duodenum or the jejunum should alert one to the possibility of ZES,[7] although a single ulcer in the duodenal bulb is the most common presentation. Gastroesophageal reflux disease complicated by ulcerations and strictures of the esophagus also tends to be more prevalent and more severe in patients with ZES.[8]

Diarrhea: This symptom may precede ulcer symptoms and is seen in over 30% of patients with gastrinoma.[9] Diarrhea results not only from gastric acid hypersecretion and subsequent activation of pepsinogens by the acid (which causes mucosal injury of the small intestine), but also from acid inactivation of pancreatic enzymes and the acid damage to enterocytes.

Steatorrhea: This defect occurs in part because inactivation of pancreatic lipase by intraluminal acid in the upper small intestine and the low pH environment render some primary bile acids insoluble, and thereby reduce the formation of micelles (which are necessary for fatty acid and monoglyceride absorption). In addition, patients often have blunted villi and, in rare cases, totally flat mucosa with resultant malabsorption.

Less frequently, ZES may present as:

Vitamin B12 malabsorption[10,11]: This deficiency may develop because low intraluminal intestinal pH interferes with intrinsic factor-facilitated active absorption of vitamin B12 by the distal ileum.[11]

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