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MEN I is an inherited condition that results in tumors in the endocrine system and in the duodenum. It is sometimes called Wermer's syndrome or endocrine adenomatosis. It is a relatively rare condition, occurring in about one out of 30,000 individuals. It is equally prevalent in men and women and in all races. In MEN I, the disease affects the pancreas, the parathyroid glands, and/or the pituitary gland. The end result is over activity of the glands. At least two of the three glands must be affected for to be MEN I.

The most commonly affected glands in MEN I are the parathyroid glands. They release parathyroid hormone into the bloodstream and result in alterations in calcium in the blood and bones. There is too much calcium in the blood that can last for many years before it is detected-often by chance. It can cause calcium-containing kidney stones in some individuals. The calcium is preferentially in the blood and is less in the bones, so the bones can weaken due to a lack of calcium. Hyperparathyroidism tends to occur by the age of 20 but is definitely present in those who have MEN I by the age of 50.

The treatment of hyperparathyroidism is often to remove three and half of the parathyroid glands, located in the neck and imbedded in the thyroid gland. If at least half of one gland is still present, the individual can maintain normal levels of PTH and calcium. The blood should be regularly tested for calcium levels so that it can be declared normal over time.

The pancreas is also involved in MEN I. The pancreas secretes insulin to reduce blood sugar, somatostatin, which inhibits secretion of some other hormones, glucagon, which increases blood pressure, gastrin, for stomach digestion, and vasoactive intestinal peptide or VIP, which causes water to be secreted into the intestinal space. In MEN I, gastrin can be secreted in excess so that too much stomach acid is released. Diarrhea can ensue as well as ulcers. About 30 to 60 percent of individuals with MEN I develop a gastrinoma. The ulcers formed by gastrinomas can be severe and fatal.

Rarely, those with MEN I can get an insulinoma. The blood sugar is secondarily too low and the person suffers from symptoms of low blood sugar. There can be tumors resulting in an excess glucagon and tumors resulting in excess ACTH. Tumors of the pancreas can rarely form GnRH or gonadotropin releasing hormone as well.

The pituitary gland can form adenomas in MEN I. The primary pituitary tumors include prolactin (prolactinomas), growth hormone excesses, ACTH-secreting tumours, thyroid stimulating hormone tumors, FSH-secreting tumours, and LH secreting tumours.

Prolactinomas occur in about 25 percent of MEN I cases. These secrete too much prolactin so that the breasts produce breast milk. Fertility of women can be affected and sex drive can be affected in both sexes. A dopamine agonist can be used to shrink the tumor and lessen the production of prolactin. Some prolactinomas require surgery, radiation or both.

Rarer types of pituitary problems in MEN I are those who have elevated ACTH levels. This makes the adrenal glands produce excess cortisol levels and you can get a secondary form of Cushing's syndrome. Growth hormone-producing tumours are also possible in MEN I.

The tumors associated with MEN I are not cancerous but still wreak havoc on the body. Prolactinomas particularly can become extremely large and may look like cancer. Lipomas or fatty tumors are common in those with MEN I and are also not cancerous. They can be treated with surgical excision. On the other hand, half of all people with MEN I will get a pancreatic cancer or carcinoid tumour of the intestinal tract or chest wall. Carcinoids are not particularly cancerous and don't require treatment if they occur in the stomach. Carcinoids may secrete hormones.

The treatment of MEN I depends on the type of adenomas found. Some can be very difficult to treat and hard to diagnose. Many times, no treatment is necessary and the patient can be monitored until they get symptomatic. In other cases, surgical excision of the adenomas is necessary and, in rare circumstances, radiation is necessary.

HELPLINE: ☎ 1800 633 634

Medical Negligence Solicitors

Our personal injury solicitors operate a specialist medical negligence compensation service. Our solicitors deal with Multiple Endocrine Neoplasia Type I (MEN I)claims using a no win no fee arrangement which means that if you don�t win then you don�t pay them their professional costs. If you would like legal advice at no cost with no further obligation just complete the contact form or email our MEN1 lawyers offices or use the helpline and a solicitor will review your medical negligence compensation claim and phone you immediately.

HELPLINE: ☎ 1800 633 634

The author of the substantive medical writing on this website is Dr. Christine Traxler MD whose biography can be read here