Wermer's syndrome
Key points
- Multiple Endocrine Neoplasia (MEN) I is a hereditary disorder in which one or more of the following glands develop excess normal tissue (hyperplasia) or adenoma (tumor): the parathyroid, the pancreas, the pituitary, and rarely, the adrenals and thyroid gland.
- Tumors of various glands appear in the same person, but not necessarily at the same time.
- Symptoms vary greatly from person to person and may be related to peptic ulcer disease, hypoglycemia, hypercalcemia, or pituitary dysfunction.
- Surgical removal of the affected gland is the treatment of choice, although therapy with a medication called bromocriptine can also be used for pituitary tumors which secrete prolactin.
Alternative names
Multiple Endocrine Neoplasia (MEN) I
Definition
Multiple Endocrine Neoplasia (MEN) I is a hereditary disorder in which one or more of the following glands develop excess normal tissue (hyperplasia) or adenoma (tumor): the parathyroid, the pancreas, the pituitary, and rarely, the adrenals and thyroid gland.
These are all endocrine glands, which produce and secrete hormones into the blood or lymph systems.
Causes, incidence, and risk factors
The cause of MEN I is genetic. Tumors of various glands appear in the same person, but not necessarily at the same time. The disorder is inherited, may occur at any age, and affects men and women equally.
Most people affected with this syndrome seek medical treatment because of one of the following: peptic ulcer disease, symptoms related to low blood sugar, symptoms related to high serum calcium levels or kidney stones, or symptoms related to pituitary problems such as headache.
Risk factors are a family history of this disorder, a previous pituitary tumor, and a history of Zollinger-Ellison syndrome.
Symptoms
Symptoms vary greatly from person to person and may be related to peptic ulcer disease, hypoglycemia, hypercalcemia, or pituitary dysfunction.
- Abdominal pain
- Burning, aching, or hunger discomfort in the upper abdomen or lower chest that is relieved by antacids, milk, or food
- Black, tarry stools
- Nausea and vomiting
- Bloated feeling after meals
- Weakness
- Headache
- Hunger
- Vision problems
- Loss of coordination
- Anxiety
- Mental changes or confusion
- Coma if hypoglycemia is untreated
- Loss of appetite
- Muscle pain
- Fatigue
- Sensitivity to the cold
- Weight loss
- Low blood pressure
- Loss of axillary or pubic hair
- In women, cessation of menses, infertility, or failure to lactate
- In men, decreased sexual interest, loss of body or facial hair
Diagnostic tests are used to evaluate the function of each endocrine gland.
Written and revised by
Andrey Nailor, M.D.
Our doctors write and review articles for accuracy and keep them up to date.
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