Wermer's Syndrome

Wermer's Syndrome

Contributed byMaulik P. Purohit MD MPHApr 01, 2018

The topic Wermer's Syndrome you are seeking is a synonym, or alternative name, or is closely related to the medical condition Multiple Endocrine Neoplasia (MEN) Type 1.

Quick Summary:

  • Multiple Endocrine Neoplasia Type 1 (MEN Type 1) is an inherited disorder of the endocrine glands
  • The disorder occurs in individuals, who are genetically predisposed to the condition, where the endocrine glands grow tumors, become overactive, release excess hormones, causing a set of symptoms and medical complications
  • The endocrine glands involved in MEN Type 1 syndrome are the parathyroid, pancreas, and the pituitary glands

Parathyroid:

  • The parathyroid gland consists of 4 small glands that are situated behind the thyroid gland in the neck
  • The gland secretes a hormone, called parathyroid hormone (PTH), which is responsible for calcium metabolism in the body
  • In MEN Syndrome Type 1, the parathyroid gland produces excess amounts of hormones, which results in high calcium levels in blood and urine, causing a thinning of the bones
  • The bones become weak and are consequently prone to fractures
  • High calcium levels in blood, causes an increased excretion of calcium through the kidneys, leading to kidney stone formation and kidney damage

Pancreas:

  • The pancreas is a gland that is located behind the stomach in the abdomen
  • It has a dual role - as an exocrine gland secreting digestive juices into the intestines, and as an endocrine gland secreting hormones. These hormones include:
    • Insulin - lowers blood sugar
    • Glucagon - increases blood sugar
    • Somatostatin - an inhibitory hormone
    • Gastrin - stimulates acid production in the stomach
    • Vasoactive intestinal peptide - secretes water into the intestine
  • The common hormone involved in MEN Type 1 disorder is gastrin. Numerous gastrin-producing tumors cause an increased production of acids in the stomach
  • This excess acid causes multiple gastric and duodenal (in the first part of the small intestine) ulcers; sometimes, even jejunal ulcers (second part of the small intestine) are observed
  • Gastrin-producing tumors may also be part of a disorder, called  Zollinger Ellison syndrome

Pituitary:

  • The pituitary gland is a small gland located in the brain. The hormones produced by this gland controls most of the basic body functions
  • The pituitary gland secretes various hormones, which include:
    • Thyroid stimulating hormone (TSH) - that stimulates the thyroid gland to produce thyroid hormones
    • Adrenocorticotropic hormone (ACTH) - that stimulates the adrenal glands to produce corticosteroids
    • Prolactin - that regulates milk production, influences fertility and bone strength
    • Growth hormone (GH) - regulates body growth
    • Luteinizing hormone (LH) - regulates the production of sex hormones
    • Follicle stimulating hormone (FSH) - regulates fertility in men and women
  • The commonly involved hormone in MEN Type 1 disorder is prolactin, and tumors secreting prolactin are called prolactinomas
  • This leads to excessive milk production, interferes with fertility in women, and reduces sex-drive in men
  • If the tumor is big, it can compress the visual pathway in the brain, causing vision problems

Please find comprehensive information on Multiple Endocrine Neoplasia (MEN) Type 1 regarding definition, distribution, risk factors, causes, signs & symptoms, diagnosis, complications, treatment, prevention, prognosis, and additional useful information HERE.

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Maulik P. Purohit MD MPH

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