Medication Summary
Inhibition of gastric acid secretion with proton pump inhibitors (PPIs) is mandatory to prevent complications of gastric acid hypersecretion in patients with Zollinger-Ellison syndrome (ZES). PPIs have proven to be safe, without side effects even after long-term use. The goal is to reduce basal acid output (BAO) to levels less than 10 mEq/h 1 hour before the next dose in patients without gastric acid–reducing surgery and less than 5 mEq/h in patients with previous acid-reducing gastric surgery.
Proton Pump Inhibitors
Class Summary
Proton pump inhibitors (PPIs) are used to reduce gastric acid hypersecretion. Dosing should be adequate to achieve a basal acid output (BAO) of less than 10 mEq/h. Dosing may vary in each patient. Children may require relatively higher doses per kilogram than adults.
Lansoprazole (Prevacid)
Lansoprazole is a substituted benzimidazole that covalently and irreversibly binds the hydrogen potassium/ATPase, thereby inhibiting acid secretion. This agent is available as an intravenous (IV) formulation, oral (PO) caps, or solutabs. Strawberry-flavored solutabs can be dissolved in water for easy administration to children. Dissolve the 15-mg solutab in 4 mL of water and the 30-mg solutab in 10 mL of water.
Omeprazole (Prilosec)
Omeprazole is a substituted benzimidazole that suppresses acid secretion by specific inhibition of hydrogen potassium/ATPase at the secretory surface of the parietal cell.
Pantoprazole (Protonix)
Pantoprazole is a substituted benzimidazole that suppresses acid secretion by specific inhibition of hydrogen potassium/ATPase at the secretory surface of the parietal cell.
Somatostatin Analogues
Class Summary
Somatostatin analogues are synthetic analogues of somatostatin that inhibit growth (GH) secretion, thereby leading to a decrease in chloride secretion, sodium absorption, and decreased fluid losses. These agents are used to treat secretory diarrhea in Zollinger-Ellison syndrome. [26]
Octreotide (Sandostatin)
Octreotide acts primarily on somatostatin receptor subtypes II and V. This agent inhibits growth hormone (GH) secretion and has a multitude of other endocrine and nonendocrine effects, including inhibition of glucagon, vasoactive intestinal peptide (VIP), and gastrointestinal (GI) peptides. Octreotide controls diarrhea in 80% of patients, but progressive increases in dosage may be necessary. In adults, Mozell et al reported success with 100 mcg subcutaneously (SC) 3 times/wk for 1-48 months.
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Somatostatin receptor scintigraphy in a young patient with Zollinger-Ellison syndrome. Besides physiologic uptake in the kidneys and the spleen, 2 pathologic hot spots are present, one in the area of the duodenum and another next to the pancreas.
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Somatostatin receptor scintigraphy in a young patient with Zollinger-Ellison syndrome whose gastrinomas were resected. The LEFT (big) tumor was a periduodenal lymph node gastrinoma; the RIGHT tumor was a pancreatic gastrinoma. Despite negative findings on the scan, the patient remained hypergastrinemic, probably because of microscopic disease that escaped diagnostic imaging and surgical exploration.
