It is intuitive that excessive acid production can result in multiple erosions and frank ulcers in the stomach, duodenum and jejunum. Although rare, this condition may arise from a gastrinoma (Zollinger-Ellison syndrome (ZES)) or from antral gastrin cell hyperfunction with or without hyperplasia (pseudo-Zollinger-Ellison syndrome (PZES)).
ZES is the most frequent ulcerogenic syndrome associated with increased gastrin levels and, in adults, represents the most common of the malignant islet cell tumors.35 ZES is characteristically associated with increased levels of serum gastrin (hypergastrinemia), which, in turn, causes an overproduction of gastric acid and results in complicated ulcer disease. As would be expected, the most common presenting signs are abdominal pain, nausea, vomiting, hematemesis and melena. Diarrhea occurs less commonly in childhood than in adulthood. ZES can be sporadic or associated with multiple endocrine neoplasia type I (MEN I). ZES-associated MEN-I patients often become symptomatic in childhood and at an earlier age than those with the sporadic form. Thus, if patients show severe peptic ulceration, kidney stones, watery diarrhea and malabsorption with a positive family history of endocrinopathy, MEN-I and nephrolithiasis, ZES should be strongly suspected. The diagnosis of ZES is made if the patient shows an elevated fasting serum gastrin level, gastric acid hypersecretion and either a positive secretin test or a histologically proven gastri-noma.36 Typically, patients have fasting serum gastin levels of > 100 pg/ml, even though adult patients usually show a level greater than 500 pg/ml, a level greater than 1000 pg/ml is nearly diagnostic of the disease. The basal acid output
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