zollinger-ellison%20syndrome
ZOLLINGER-ELLISON SYNDROME
Zollinger-Ellison syndrome (ZES) is a disease entity which refers to the triad of severe peptic ulcer disease (PUD), gastric acid hypersecretion and non-beta cell gastrin-secreting tumor primarily of the pancreas and duodenum (gastrinoma).
Approximately two-thirds of patients have sporadic ZES while the rest is part of multiple endocrine neoplasia type 1.
ZES should be considered in the differential diagnoses of patients who present with abdominal pain, malabsorption and chronic watery diarrhea.
A high index of clinical awareness is necessary to correctly diagnose ZES.

Introduction

  • Zollinger-Ellison Syndrome (ZES) is a disease entity which refers to the triad of severe peptic ulcer disease (PUD), gastric acid hypersecretion and non-beta cell gastrin-secreting tumor primarily of the pancreas and duodenum (gastrinoma)
  • Approximately two-thirds of patients have sporadic ZES while the rest is part of multiple endocrine neoplasia type 1 (MEN-1)
    • Gastrinomas that occur in relation with MEN-1 are associated with a lower chance of metastases compared to gastrinomas in sporadic ZES

Epidemiology

  • Estimated incidence of ZES is 0.1-3 patients per million population
  • Most patients are diagnosed between the ages of 20 and 65, but as old as 90 and as young as 7 have been identified
    • Mean age of onset is 41 years, with a slight male predominance
    • Mean delay in the diagnosis is 5-6 years; the widespread use of PPIs contributes significantly to the delay in diagnosis

Signs and Symptoms

ZES should be considered in the differential diagnoses of patients who present with abdominal pain, malabsorption and chronic watery diarrhea

Abdominal Pain

  • Most common symptom, typically felt in the epigastric area and may mimic PUD
  • Pancreatitis, which may cause abdominal pain and malabsorption, may occur concomitantly with ZES

Diarrhea

  • Occurs secondary to malabsorption that results from large amounts of gastric acid that go into the small intestine, causing inactivation of pancreatic digestive enzymes and damage to the gut mucosa
  • Malabsorption leading to diarrhea (and sometimes steatorrhea) may also be caused by precipitation of bile salts
  • Diarrhea is seen less frequently in patients with MEN-1-associated ZES than in those with sporadic ZES
  • May occur together with abdominal pain in about half of patients with ZES

Heartburn

  • May mimic gastroesophageal reflux disease (GERD)

GI Bleeding

  • Often develops as a consequence of peptic ulceration

Other Symptoms

  • Nausea and vomiting (N/V), weight loss

Other Clinical Features Suspicious of ZES

  • Multiple duodenal and/or jejunal ulcers; postbulbar duodenal ulcer
  • PUD that is poorly responsive to medical therapy
  • PUD associated with chronic diarrhea
  • PUD not associated with Helicobacter pylori or nonsteroidal anti-inflammatory drugs (NSAIDs) use
  • Family history of PUD and hypercalcemia
  • In patients in whom MEN-1-associated ZES is considered, symptoms and/or a family history of associated disorders ie hypercalcemia, nephrolithiasis, pituitary disorders and hyperparathyroidism should be elicited
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31 Jan 2019
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