Wermer Syndrome (MEN Type 1) Medication

  • Author: Irina Lendel, MD; Chief Editor: George T Griffing, MD   more...
 
Updated: Oct 8, 2010
 

Medication Summary

Because various syndromes may be expressed in patients with MEN 1, several medications may be indicated.

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Somatostatin analogues

Class Summary

These drugs suppress peptide secretion of gastroenteropancreatic tumors or treatment of acromegaly. They have also been reported to relieve pain from spinal metastasis.

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Octreotide (Sandostatin)

 

Acts primarily on somatostatin receptor subtypes II and V. Inhibits GH secretion and has multitude of other endocrine and nonendocrine effects, including inhibition of glucagon, VIP, and GI peptides.

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Proton pump inhibitors

Class Summary

Proton pump inhibitors inhibit gastric acid secretion by inhibition of the H+/K+ -ATP-ase enzyme system in the gastric parietal cells. Esomeprazole (Nexium), omeprazole (Prilosec), pantoprazole (Protonix), rabeprazole (Aciphex), and lansoprazole (Prevacid) are available.

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Omeprazole (Prilosec)

 

Proton pump inhibitors effectively block the H+, K+ -ATPase of the parietal cell at the secretory surface and inhibit acid secretion, which is required in MEN 1 associated with gastrinoma. The goal is to reduce the basal acid output to < 10 mEq/h 1 h prior to the next dose in patients without previous acid-reducing gastric surgery and to < 5 mEq/h in patients with previous acid-reducing gastric surgery.

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Esomeprazole (Nexium)

 

S-isomer of omeprazole. Inhibits gastric acid secretion by inhibiting H+/K+ ATPase enzyme system at secretory surface of gastric parietal cells.

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Dopamine agonists

Class Summary

Dopamine agonists are the treatment of choice in prolactinoma. In acromegaly, they are usually added to somatostatin analogues if complete remission has not been achieved. They have modest effects if used as a single agent for acromegaly. Bromocriptine (Parlodel) and cabergoline (Dostinex) are available.

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Bromocriptine (Parlodel)

 

Dopamine agonist reduces pituitary production of prolactin and may shrink prolactinoma. May be alternative for treatment of acromegaly, but adverse effects at high dose may limit applicability.

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Antihypoglycemic agents

Class Summary

These agents increase blood glucose by inhibiting pancreatic insulin release and possibly through an extrapancreatic effect.

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Diazoxide (Hyperstat, Proglycem)

 

Inhibitory effect of diazoxide in insulinoma may be effective in 90% of patients, the remaining 10% may not respond or tolerate drug. Treat adverse effects with hydrochlorothiazide. Hyperglycemic effect starts within 1 h and usually lasts a maximum of 8 h with normal renal function. In patients not responsive to diazoxide, somatostatin may be indicated.

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Ergot Alkaloid And Derivative

Class Summary

Dopaminergic activity of some of these agents may reduce prolactin secretion.

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Cabergoline (Dostinex)

 

Long-acting dopamine receptor agonist with high affinity for D2 receptors. Low affinity for D1 receptors. Inhibits prolactin secretion. Prolactin secretion by anterior pituitary predominates under hypothalamic inhibitory control exerted through dopamine.

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Contributor Information and Disclosures
Author

Irina Lendel, MD  Clinical Instructor in Endocrinology, Division of Endocrinology, Diabetes, and Metabolism, Milton S Hershey Medical Center

Disclosure: Nothing to disclose.

Coauthor(s)

James M Hammond, MD  Distinguished Professor of Medicine, Penn State University College of Medicine, Milton S Hershey Medical Center

James M Hammond, MD is a member of the following medical societies: Alpha Omega Alpha, American Diabetes Association, American Federation for Clinical Research, American Society for Clinical Investigation, Association of American Physicians, Endocrine Society, Phi Beta Kappa, and Society for the Study of Reproduction

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.

Specialty Editor Board

Frederick H Ziel, MD  Associate Professor of Medicine, David Geffen School of Medicine at UCLA; Physician-In-Charge, Endocrinology/Diabetes Center, Director of Medical Education, Kaiser Permanente Woodland Hills; Chair of Endocrinology, Co-Chair of Diabetes Complete Care Program, Southern California Permanente Medical Group

Frederick H Ziel, MD is a member of the following medical societies: American Association of Clinical Endocrinologists, American College of Endocrinology, American College of Physicians, American College of Physicians-American Society of Internal Medicine, American Diabetes Association, American Federation for Medical Research, American Medical Association, American Society for Bone and Mineral Research, California Medical Association, Endocrine Society, and International Society for Clinical Densitometry

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

Arthur B Chausmer, MD, PhD, FACP, FACE, FACN, CNS  Professor of Medicine (Endocrinology, Adj), Johns Hopkins School of Medicine; Affiliate Research Professor, Bioinformatics and Computational Biology Program, School of Computational Sciences, George Mason University; Principal, C/A Informatics, LLC

Arthur B Chausmer, MD, PhD, FACP, FACE, FACN, CNS is a member of the following medical societies: American Association of Clinical Endocrinologists, American College of Endocrinology, American College of Nutrition, American College of Physician Executives, American College of Physicians, American College of Physicians-American Society of Internal Medicine, American Medical Informatics Association, American Society for Bone and Mineral Research, American Society of Law, Medicine & Ethics, Endocrine Society, and International Society for Clinical Densitometry

Disclosure: Nothing to disclose.

Mark Cooper, MBBS, PhD, FRACP  Head, Diabetes & Metabolism Division, Baker Heart Research Institute, Professor of Medicine, Monash University

Disclosure: Nothing to disclose.

Chief Editor

George T Griffing, MD  Professor of Medicine, St Louis University School of Medicine

George T Griffing, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Medical Practice Executives, American College of Physician Executives, American College of Physicians, American Diabetes Association, American Federation for Medical Research, American Heart Association, Central Society for Clinical Research, Endocrine Society, International Society for Clinical Densitometry, and Southern Society for Clinical Investigation

Disclosure: Nothing to disclose.

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Sagittal (left image) and coronal (right image), T1-weighted magnetic resonance images of the brain in a patient with multiple endocrine neoplasia syndrome type 1 (MEN 1). These images show a pituitary macroadenoma (arrows).
Indium-111 (111In) octreotide scan in a patient with multiple endocrine neoplasia syndrome type 1 (MEN 1). These nuclear images demonstrate abnormal activity in the pituitary macroadenoma (curved arrow), parathyroid adenoma (straight arrow), and gastrinoma metastases throughout the abdomen (arrowheads).
Technetium-99m sestamibi scan (99mTc MIBI) in a patient with multiple endocrine neoplasia syndrome type 1 (MEN 1). These images demonstrate persistent abnormal activity of the inferior right parathyroid gland that is consistent with an adenoma.
Computed tomography (CT) scan of the pancreas in a patient with multiple endocrine neoplasia syndrome type 1 (MEN 1) and a gastrinoma. This image shows a pancreatic head mass (large, white arrow), as well as a low-attenuating lesion in the liver (small, black arrowhead) that indicates metastases. Note the calcifications of the right renal medullary pyramids (medullary nephrocalcinosis; black arrows) in this nonenhanced CT scan.
Endoscopic ultrasonogram in a patient with an insulinoma. The hypoechoic neoplasm (arrows) is seen in the body of the pancreas anterior to the splenic vein (SV). (From: Rosch T, Lightdale CJ, Botet JF, et al. Localization of pancreatic endocrine tumors by endoscopic ultrasonography. N Engl J Med. Jun 25 1992;326(26):1721-6.)
Computed tomography (CT) scan image with oral and intravenous contrast in a patient with biochemical evidence of insulinoma. The 3-cm contrast-enhancing neoplasm (arrow) is seen in the tail of the pancreas (P) posterior to the stomach (S) (From: Yeo CJ. Islet cell tumors of the pancreas. In: Niederhuber JE, ed. Current Therapy in Oncology. St. Louis, Mo: Mosby-Year Book; 1993: 272.)
Anteroposterior radiographic view of the right hand in a patient with multiple endocrine neoplasia syndrome type 1 (MEN 1) and primary hyperparathyroidism. This image shows subperiosteal bone resorption along the radial aspects of the middle phalanges (arrows).
Bilateral, anteroposterior radiographic views of the hands in a patient with multiple endocrine neoplasia syndrome type 1 (MEN 1) and primary hyperparathyroidism. These images show subperiosteal bone resorption along the radial aspects of the middle phalanges.
 
 
 
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