Multiple Endocrine Neoplasia (MEN) I
Alternative names:
Wermer's syndrome
Definition:
A hereditary disorder in which two or more of the following glands develop hyperplasia or adenoma (tumor): the parathyroid, the pancreas, the pituitary, and rarely, the adrenals and thyroid gland.
Causes, incidence, and risk factors:
The cause of multiple endocrine neoplasia 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. The incidence is 1 out of 100,000 people; it is extremely rare in children.
|