Pediatric Multiple Endocrine Neoplasia Medication

  • Author: Alicia Diaz-Thomas, MD, MPH; Chief Editor: Stephen Kemp, MD, PhD   more...
 
Updated: Apr 19, 2012
 

Medication Summary

Pharmacologic therapy for multiple endocrine neoplasia (MEN) is directed toward specific endocrine syndromes associated with the disorder. Patients with type 1 MEN and ZES need lifelong acid inhibition with PPIs.

As previously mentioned, somatostatin therapy is indicated particularly in patients with acromegaly in whom surgery did not achieve complete tumor removal. One third of patients should experience tumor shrinkage, and up to two thirds of patients should achieve normalization of IGF-1 levels.

Although surgery is the therapy of choice for insulinomas, unresectable tumors are treated with the hyperglycemic agent diazoxide.

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Somatostatin analogs

Class Summary

Octreotide acetate acts similarly to the natural hormone somatostatin by suppressing peptide secretion from gastroenteropancreatic tumors.

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Octreotide acetate (Sandostatin)

 

Octreotide acetate acts primarily on somatostatin receptor subtypes II and V. It inhibits GH secretion and has other endocrine and nonendocrine effects, including inhibition of glucagon, VIP, and gastrointestinal (GI) peptides. Octreotide acetate controls diarrhea in 80% of patients. Progressive increases in dosage may be necessary.

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Proton Pump Inhibitors

Class Summary

Gastric acid secretion with PPIs is mandatory to prevent complications of gastric acid hypersecretion. PPIs are safe and cause no adverse effects, even after long-term use. The goal of therapy with these agents is to reduce the basal acid output to less than 10mEq/h 1 hour before the next dose in patients without previous acid-reducing gastric surgery, and to less than 5mEq/h in patients with previous acid-reducing gastric surgery.

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Omeprazole (Prilosec)

 

Omeprazole is a substituted benzimidazole that suppresses acid secretion by specifically inhibiting the H+/K+ adenosine triphosphatase (ATPase) at the secretory surface of the parietal cell.

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Hypoglycemia Antidotes

Class Summary

These agents inhibit insulin release from the tumor.

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Diazoxide (Proglycem)

 

Diazoxide binds to the sulfonylurea receptor (SUR1) of the pancreatic beta cell, inhibiting insulin secretion. The oral form opens K adenosine triphosphate (ATP) channels and inhibits insulin secretion. Diazoxide increases a patient's blood glucose level within 1 hour by inhibiting insulin release from the patient's insulinoma. Unlike rapid IV administration of diazoxide, oral administration of the drug is not antihypertensive.

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Contributor Information and Disclosures
Author

Alicia Diaz-Thomas, MD, MPH  Assistant Professor of Pediatrics, University of Tennessee Health Science Center, Memphis

Alicia Diaz-Thomas, MD, MPH is a member of the following medical societies: American Academy of Clinical Endocrinology, Endocrine Society, and Tennessee Medical Association

Disclosure: Nothing to disclose.

Chief Editor

Stephen Kemp, MD, PhD  Professor, Department of Pediatrics, Section of Pediatric Endocrinology, University of Arkansas for Medical Sciences College of Medicine, Arkansas Children's Hospital

Stephen Kemp, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Association of Clinical Endocrinologists, American Pediatric Society, Endocrine Society, Phi Beta Kappa, Southern Medical Association, and Southern Society for Pediatric Research

Disclosure: Nothing to disclose.

Additional Contributors

George P Chrousos, MD, FAAP, MACP, MACE, FRCP (London) Professor and Chair, First Department of Pediatrics, Athens University Medical School, Aghia Sophia Children's Hospital, Greece; UNESCO Chair on Adolescent Health Care, University of Athens, Athens, Greece

George P Chrousos, MD, FAAP, MACP, MACE, FRCP(London) is a member of the following medical societies: American Academy of Pediatrics, American College of Endocrinology, American College of Physicians, American Pediatric Society, American Society for Clinical Investigation, Association of American Physicians, Endocrine Society, Lawson-Wilkins Pediatric Endocrine Society, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Robert J Ferry Jr, MD, Le Bonheur Chair of Excellence in Endocrinology, Professor and Chief, Division of Pediatric Endocrinology and Metabolism, Department of Pediatrics, University of Tennessee Health Science Center

Robert J Ferry Jr, MD is a member of the following medical societies: American Academy of Pediatrics, American Diabetes Association, American Medical Association, Endocrine Society, Pediatric Endocrine Society, Society for Pediatric Research, and Texas Pediatric Society

Disclosure: Nothing to disclose.

Christian A Koch, MD, PhD, FACP, FACE Professor and Director, Division of Endocrinology, University of Mississippi Medical Center

Christian A Koch, MD, PhD, FACP, FACE is a member of the following medical societies: American Academy of Neurology, American Association of Clinical Endocrinologists, American College of Clinical Endocrinologists, American College of Physicians-American Society of Internal Medicine, American Society for Clinical Pharmacology and Therapeutics, American Society for Dermatologic Surgery, Endocrine Society, and German Diabetes Society

Disclosure: NovoNordisk Honoraria Consulting

Lynne Lipton Levitsky, MD Chief, Pediatric Endocrine Unit, Massachusetts General Hospital; Associate Professor of Pediatrics, Harvard Medical School

Lynne Lipton Levitsky, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Diabetes Association, American Pediatric Society, Endocrine Society, Lawson-Wilkins Pediatric Endocrine Society, and Society for Pediatric Research

Disclosure: Pfizer Grant/research funds P.I.; Tercica Grant/research funds Other; Eli Lily Grant/research funds PI; NovoNordisk Grant/research funds PI

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.

Arlan L Rosenbloom, MD Adjunct Distinguished Service Professor Emeritus of Pediatrics, University of Florida; Fellow of the American Academy of Pediatrics; Fellow of the American College of Epidemiology

Arlan L Rosenbloom, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Epidemiology, American Pediatric Society, Endocrine Society, Florida Pediatric Society, Lawson-Wilkins Pediatric Endocrine Society, and Society for Pediatric Research

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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