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Review Question - QID 104553

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QID 104553 (Type "104553" in App Search)
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?

Elevated fasting serum gastrin and elevated gastrin levels upon secretin administration

89%

74/83

Elevated fasting serum gastrin and decreased gastrin levels upon secretin administration

6%

5/83

Decreased fasting serum gastrin levels and elevated gastrin levels upon secretin administration

2%

2/83

Decreased fasting serum gastrin levels and decreased gastrin levels upon secretin administration

0%

0/83

Normal fasting gastrin levels and normal gastrin levels upon secretin administration

0%

0/83

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This patient has history concerning for Zollinger-Ellison syndrome (ZES) in the context of multiple endocrine neoplasia I (previous parathyroid and pituitary tumors). Gastrin is characteristically increased ZES and administration of secretin would also increase gastrin levels.

ZES occurs when a patient has a gastrinoma - a pancreatic islet cell tumor that secretes large amounts of gastrin which lead to gastric acid hypersecretion and ulcers. Over half of gastrinomas are malignant and 20% are associated with MEN I. Diagnosis begins with the history and physical, which often reveal features of peptic ulcer disease, diarrhea, weight loss, and abdominal pain. When suspicious of a gastrinoma, diagnostic workup continues with a fasting serum gastrin level (> 1000 pg/mL is diagnostic). If this test is equivocal, a secretin injection test can be performed. Normally, secretin inhibits gastrin secretion; however, in patients with ZES, gastrin levels increase substantially after secretin administration.

Ramakrishnan and Salinas discuss peptic ulcer disease (PUD), which commonly occurs in the stomach and the proximal duodenum. Predominant causes in the U.S. are infection with H. pylori and the use of NSAIDs. Symptoms include epigastric discomfort, loss of appetite, and weight loss. For young patients without alarming symptoms, a test-and-treat strategy based on the results of H. pylori testing is recommended.

Krampitz and Norton discuss management of ZES. A gastrinoma is the culprit, usually associated with symptoms of peptic ulcer disease, GERD, and diarrhea. The diagnosis is made by fasting levels of serum gastrin and the secretin stimulation test. Because of the high association of ZES and MEN I, hyperparathyroidism should be excluded by obtaining a serum calcium and parathyroid hormone level.

Illustration A depicts a gastrinoma (arrow). Illustration B shows the classic MEN syndromes and their trademarks.

Incorrect Answers:
Answer 2-5: In ZES, the classic results from the fasting gastrin test and secretin test will be elevated gastrin levels. These options are less consistent with a diagnosis of ZES.

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