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Zollinger-Ellison Syndrome

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Topic updated on 10/24/17 12:00pm

Snap Shot
  • A 42-year-old male executive complains of upper abdominal pain. He say he is under considerable stress at work and smokes a pack of cigarettes every day. He has no history of SOB, swelling of the feet, chest pain, or allergies. Further history reveals that the pain is worse at night and often wakens him from sleep. It occurs between meals and is appeased by drinking milk and eating a bland diet. On one occasion he vomited blood. Testing reveals a basal acid output (BA)) of 60 mEq (normal is <5 mEq and a serum gastrin level of 1000 pg/ml (normal is <300 pg/ml).
Introduction
  • Caused by a malignant islet cell tumor that secretes excessive amounts of gastrin
    • causes excessive secretion of acid
    • results in peptic ulcer disease
Presentation
  • Symptoms
    • similar to PUD
    • diarrhea
      • because the acidity in the duodenum inactivates pancreatic enzymes
Evaluation
  • Basal Acid Output test 
  • Secretin stimulation test
    • normal gastric G-cells are inhibited by secretin
    • secretin stimulates release of gastrin from gastrinoma cells  
    • positive test result is an increase in gastrin concentration
  • Elevated
    • insulin
    • glucagon
    • gastrin ( >1000 pg/mL)
Differential
  • GERD, CAD, gastritis, pancreatitis, cholecystitis, aortic aneurysm, and other causes of an acute abdomen
Treatment
  • Immediate treatment with proton blockers
  • Surgical resection of the tumor


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Qbank (1 Questions)

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(M2.GI.11) A 50-year-old man with a past medical history of parathyroid neoplasia and a pituitary tumor status post trans-sphenoidal resection presents with gnawing epigastric pain. He reports that the pain is persistent. He has been in so much pain that he has been unable to eat and has had 15 pounds of weight loss over the past month. Vital signs are stale. Physical examination is notable for tenderness on palpation of the epigastric region. Upon further diagnostic testing, which of the following might be expected? Topic Review Topic

1. Elevated fasting serum gastrin and elevated gastrin levels upon secretin administration
2. Elevated fasting serum gastrin and decreased gastrin levels upon secretin administration
3. Decreased fasting serum gastrin levels and elevated gastrin levels upon secretin administration
4. Decreased fasting serum gastrin levels and decreased gastrin levels upon secretin administration
5. Normal fasting gastrin levels and normal gastrin levels upon secretin administration

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