Zollinger Ellison and pseudoZollinger Ellison syndromes

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

  • BAO) is generally more than 10 mEq/h. The secretin provocative test (2units/kg, intravenously) has a high diagnostic value; in adults, a rise in the serum gastrin level of 200 pg/ml above the fasting concentration is considered a positive diagnosis. Endoscopic findings are characterized by multiple peptic ulcers, most of which are localized in the duodenum, but the stomach and jejunum are involved less commonly. Recent developments of both radiological and nuclear medicine studies have increased the capability for identifying neoplastic lesions in the majority of patients with ZES. Recommended imaging procedures are computed tomography (CT), magnetic resonance imaging (MRI) and somatostatin receptor scintigra-phy (octreoscan); more recently, endoscopic ultrasound has been proposed as the most sensitive imaging modality.37
  • PZES' is usually used for two distinct entities: antral G-cell hyperplasia, described as an increased number of G cells, and antral G-cell hyperfunction, in which hypergastrinemia occurs in the absence of detectable G-cell hyperplasia.38 The clinical manifestation of PZES varies from vague features, such as non-specific abdominal pain, vertigo, anemia and occult bleeding, to severe gastrointestinal bleeding. The differentiation between ZES and PZES is based on the response to provocative tests. PZES is characterized by exaggerated serum gastrin release in response to a feeding test, and unchanged or even depressed serum gastrin values after injection of secretin.

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