Wermer Syndrome (MEN Type 1) Medication

Updated: Feb 18, 2015
  • Author: Laura Williams, MD; Chief Editor: George T Griffing, MD  more...
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Medication

Medication Summary

Many hormonal abnormalities may be expressed in patients with multiple endocrine neoplasia type 1 (MEN1). Medications that may be used are outlined below.

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Somatostatin analogues

Class Summary

These drugs suppress peptide secretion of gastroenteropancreatic tumors or growth hormone – producing tumors. They have also been reported to relieve pain from spinal metastasis.

Octreotide (Sandostatin)

Octreotide acts primarily on somatostatin receptor subtypes II and V. It inhibits growth hormone secretion and has a multitude of other endocrine and nonendocrine effects, including inhibition of glucagon, vasoactive intestinal peptide, 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.

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 MEN1-associated gastrinomas. The goal is to reduce the basal acid output to less than 10 mEq/h 1 hour prior to the next dose in patients without previous acid-reducing gastric surgery and to less than 5 mEq/h in patients with previous acid-reducing gastric surgery.

Esomeprazole (Nexium)

Esomeprazole is an S-isomer of omeprazole. It inhibits gastric acid secretion by inhibiting H+/K+-ATPase enzyme system at the secretory surface of gastric parietal cells.

Pantoprazole (Protonix)

Pantoprazole suppresses gastric acid secretion by specifically inhibiting the H+/K+-ATPase enzyme system at the secretory surface of gastric parietal cells. Use of the intravenous preparation has only been studied for short-term use (ie, 7-10 d).

Rabeprazole sodium (AcipHex)

Rabeprazole sodium suppresses gastric acid secretion by specifically inhibiting the H+/K+-ATPase enzyme system at the secretory surface of gastric parietal cells.

Lansoprazole (Prevacid)

Rabeprazole sodium suppresses gastric acid secretion by specifically inhibiting the H+/K+-ATPase enzyme system at the secretory surface of gastric parietal cells.

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Dopamine agonists

Class Summary

Dopamine agonists are the treatment of choice in prolactinomas. In growth hormone producing pituitary tumors, 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.

Bromocriptine (Parlodel)

Bromocriptine is a dopamine agonist that reduces pituitary production of prolactin and may shrink prolactinomas. It may be an alternative for treatment of acromegaly, but adverse effects at high dose may limit applicability.

Cabergoline (Dostinex)

Cabergoline is a long-acting dopamine receptor agonist with high affinity for D2 receptors and low affinity for D1 receptors. It inhibits prolactin secretion. Prolactin secretion by the anterior pituitary predominates under hypothalamic inhibitory control exerted through dopamine.

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Antihypoglycemic agents

Class Summary

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

Diazoxide (Hyperstat, Proglycem)

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

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