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Pediatric Zollinger-Ellison Syndrome Medication

  • Author: Tiffany J Patton, MD; Chief Editor: Carmen Cuffari, MD  more...
 
Updated: Nov 19, 2015
 

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.

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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.

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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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Contributor Information and Disclosures
Author

Tiffany J Patton, MD Assistant Professor, Division of Pediatrics, Section of Pediatric Gastroenterology, Hepatology, and Nutrition, Comer Children's Hospital, University of Chicago Division of the Biological Sciences, The Pritzker School of Medicine

Tiffany J Patton, MD is a member of the following medical societies: American Academy of Pediatrics, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition

Disclosure: Nothing to disclose.

Chief Editor

Carmen Cuffari, MD Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine

Carmen Cuffari, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, Royal College of Physicians and Surgeons of Canada

Disclosure: Received honoraria from Prometheus Laboratories for speaking and teaching; Received honoraria from Abbott Nutritionals for speaking and teaching.

Acknowledgements

Jayant Deodhar, MD Associate Professor in Pediatrics, BJ Medical College, India; Honorary Consultant, Departments of Pediatrics and Neonatology, King Edward Memorial Hospital, India

Disclosure: Nothing to disclose.

Stefano Guandalini, MD Founder and Medical Director, Celiac Disease Center, Chief, Section of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, University of Chicago Medical Center; Professor, Department of Pediatrics, Section of Gastroenterology, Hepatology and Nutrition, University of Chicago Division of the Biological Sciences, The Pritzker School of Medicine

Stefano Guandalini, MD is a member of the following medical societies: American Gastroenterological Association, European Society for Paediatric Gastroenterology, Hepatology & Nutrition, North American Society for Pediatric Gastroenterology and Nutrition, and North American Society for the Study of Celiac Disease

Disclosure: Nothing to disclose.

Stacy A Kahn, MD Postdoctoral Fellow and Instructor, Department of Pediatrics, Section of Pediatric Gastroenterology, Hepatology and Nutrition, University of Chicago Division of the Biological Sciences, The Pritzker School of Medicine

Stacy A Kahn, MD is a member of the following medical societies: American Academy of Pediatrics, American Gastroenterological Assocation, and North American Society for Pediatric Gastroenterology and Nutrition

Disclosure: Nothing to disclose.

David A Piccoli, MD Chief of Pediatric Gastroenterology, Hepatology and Nutrition, The Children's Hospital of Philadelphia; Professor, University of Pennsylvania School of Medicine

David A Piccoli, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American Gastroenterological Association, and North American Society for Pediatric Gastroenterology and Nutrition

Disclosure: Nothing to disclose.

Klaus Radebold, MD, PhD Former Research Associate, Department of Surgery, Yale University School of Medicine

Disclosure: Nothing to disclose.

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

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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.
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.
 
 
 
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