Pediatric Zollinger-Ellison Syndrome Medication

  • Author: Stefano Guandalini, MD; Chief Editor: Carmen Cuffari, MD   more...
 
Updated: Nov 4, 2011
 

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.

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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.

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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.

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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.[21]

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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

Stefano Guandalini, MD  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, and North American Society for Pediatric Gastroenterology and Nutrition

Disclosure: Nothing to disclose.

Coauthor(s)

Tiffany J Patton, MD  Fellow, Division of Pediatric Gastroenterology, Comer Children's Hospital, University of Chicago Medical Center

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

Disclosure: Nothing to disclose.

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

Klaus Radebold, MD, PhD is a member of the following medical societies: American Gastroenterological Association and New York Academy of Sciences

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, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Additional Contributors

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.

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.

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.

References

  1. Gibril F, Schumann M, Pace A, Jensen RT. Multiple endocrine neoplasia type 1 and Zollinger-Ellison syndrome: a prospective study of 107 cases and comparison with 1009 cases from the literature. Medicine (Baltimore). Jan 2004;83(1):43-83. [Medline].

  2. Imamura M, Komoto I, Ota S. Changing treatment strategy for gastrinoma in patients with Zollinger-Ellison syndrome. World J Surg. Jan 2006;30(1):1-11. [Medline].

  3. Zollinger RM, Ellison EH. Primary peptic ulcerations of the jejunum associated with islet cell tumors of the pancreas. Ann Surg. Oct 1955;142(4):709-23; discussion, 724-8. [Medline]. [Full Text].

  4. Passaro E, Howard TJ, Sawicki MP, et al. The origin of sporadic gastrinomas within the gastrinoma triangle: a theory. Arch Surg. Jan 1998;133(1):13-6; discussion 17. [Medline].

  5. Debelenko LV, Emmert-Buck MR, Zhuang Z, et al. The multiple endocrine neoplasia type I gene locus is involved in the pathogenesis of type II gastric carcinoids. Gastroenterology. Sep 1997;113(3):773-81. [Medline].

  6. Kulke MH, Mayer RJ. Carcinoid tumors. N Engl J Med. Mar 18 1999;340(11):858-68. [Medline].

  7. Evans JT, Nickles S, Hoffman BJ. Primary hepatic gastrinoma: an unusual case of zollinger-ellison syndrome. Gastroenterol Hepatol (N Y). Jan 2010;6(1):53-6. [Medline]. [Full Text].

  8. Norton JA, Fraker DL, Alexander HR, et al. Surgery to cure the Zollinger-Ellison syndrome. N Engl J Med. Aug 26 1999;341(9):635-44. [Medline].

  9. Nord KS, Joshi V, Hanna M, et al. Zollinger-Ellison syndrome associated with a renal gastrinoma in a child. J Pediatr Gastroenterol Nutr. Nov-Dec 1986;5(6):980-6. [Medline].

  10. Morrow EH, Norton JA. Surgical management of Zollinger-Ellison syndrome; state of the art. Surg Clin North Am. Oct 2009;89(5):1091-103. [Medline].

  11. Eire PF, Rodriguez Pereira C, Barca Rodriguez P, Varela Cives R. Uncommon case of gastrinoma in a child. Eur J Pediatr Surg. Jun 1996;6(3):173-4. [Medline].

  12. Ito T, Jensen RT. Primary hepatic gastrinoma: an unusual case of zollinger-ellison syndrome. Gastroenterol Hepatol (N Y). Jan 2010;6(1):57-9. [Medline]. [Full Text].

  13. Heikenen JB, Pohl JF, Werlin SL. Octreotide in pediatric patients. J Pediatr Gastroenterol Nutr. Nov 2002;35(5):600-9. [Medline].

  14. Berna MJ, Hoffmann KM, Serrano J, Gibril F, Jensen RT. Serum gastrin in Zollinger-Ellison syndrome: I. Prospective study of fasting serum gastrin in 309 patients from the National Institutes of Health and comparison with 2229 cases from the literature. Medicine (Baltimore). Nov 2006;85(6):295-330. [Medline].

  15. Berna MJ, Hoffmann KM, Long SH, Serrano J, Gibril F, Jensen RT. Serum gastrin in Zollinger-Ellison syndrome: II. Prospective study of gastrin provocative testing in 293 patients from the National Institutes of Health and comparison with 537 cases from the literature. evaluation of diagnostic criteria, proposal of new criteria, and correlations with clinical and tumoral features. Medicine (Baltimore). Nov 2006;85(6):331-64. [Medline].

  16. Proye C, Malvaux P, Pattou F, et al. Noninvasive imaging of insulinomas and gastrinomas with endoscopic ultrasonography and somatostatin receptor scintigraphy. Surgery. Dec 1998;124(6):1134-43; discussion 1143-4. [Medline].

  17. Norton JA, Melcher ML, Gibril F, Jensen RT. Gastric carcinoid tumors in multiple endocrine neoplasia-1 patients with Zollinger-Ellison syndrome can be symptomatic, demonstrate aggressive growth, and require surgical treatment. Surgery. Dec 2004;136(6):1267-74. [Medline].

  18. Corleto VD, Annibale B, Gibril F, et al. Does the widespread use of proton pump inhibitors mask, complicate and/or delay the diagnosis of Zollinger-Ellison syndrome?. Aliment Pharmacol Ther. Oct 2001;15(10):1555-61. [Medline].

  19. Jensen RT. Gastrinomas: Advances in Diagnosis and Management. Neuroendocrinology. 2004;80 Suppl 1:23-27. [Medline].

  20. Kwekkeboom DJ, de Herder WW, van Eijck CH, et al. Peptide receptor radionuclide therapy in patients with gastroenteropancreatic neuroendocrine tumors. Semin Nucl Med. Mar 2010;40(2):78-88. [Medline].

  21. Mozell EJ, Cramer AJ, O'Dorisio TM, Woltering EA. Long-term efficacy of octreotide in the treatment of Zollinger-Ellison syndrome. Arch Surg. Sep 1992;127(9):1019-24; discussion 1024-6. [Medline].

  22. Berna MJ, Annibale B, Marignani M, et al. A prospective study of gastric carcinoids and enterochromaffin-like cell changes in multiple endocrine neoplasia type 1 and Zollinger-Ellison syndrome: identification of risk factors. J Clin Endocrinol Metab. May 2008;93(5):1582-91. [Medline].

  23. Ellison EC, Johnson JA. The Zollinger-Ellison syndrome: a comprehensive review of historical, scientific, and clinical considerations. Curr Probl Surg. Jan 2009;46(1):13-106. [Medline].

  24. Gibril F, Jensen RT. Advances in evaluation and management of gastrinoma in patients with Zollinger-Ellison syndrome. Curr Gastroenterol Rep. May 2005;7(2):114-21. [Medline].

  25. Quatrini M, Castoldi L, Rossi G, et al. A follow-up study of patients with Zollinger-Ellison syndrome in the period 1966-2002: effects of surgical and medical treatments on long-term survival. J Clin Gastroenterol. May-Jun 2005;39(5):376-80. [Medline].

  26. Tomassetti P, Campana D, Piscitelli L, et al. Treatment of Zollinger-Ellison syndrome. World J Gastroenterol. Sep 21 2005;11(35):5423-32. [Medline]. [Full Text].

 
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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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