by Kimberly Allen, RN
Multiple endocrine neoplasia type I or MEN I is also known as Wermer’s syndrome. MEN I is an autosomal dominant inherited disorder that affects the glands of the endocrine system. It is considered a fairly rare disorder only occurring in 1 out of every 30,000 people. MEN I affects both males and females and shows no preference for ethnic, racial or geographical entities. Because MEN I is considered almost exclusively hereditary a person that has been diagnosed with MEN I has a 50% chance of having children with MEN I. usually all siblings and children as well as the parents of someone that has been diagnosed are tested to see if they carry the gene that causes it.
MEN I causes tumors to develop on the various glands of the endocrine system as well as in the stomach and upper part of the small intestine, known as the duodenum. Although these tumors can be either benign or malignant both can cause significant health issues. Because the glands of the endocrine system secrete a variety of powerful hormones into the bloodstream to regulate the function of tissues and cells throughout you body any tumors whether benign or malignant can have a significant impact on your health.
The most common place for tumors to develop in MEN I is the parathyroid glands. You have 4 parathyroid glands located next to the thyroid gland in the front of your neck. The parathyroid glands secrete the parathyroid hormone (PTH) which is responsible for maintaining the calcium levels in your bones and blood as well as your urine. In people with MEN I the parathyroid glands are usually hyperactive as the tumors that form secrete too much PTH. Too much PTH can lead to an over abundance of calcium in your bloodstream. Though a person can live for years with a high blood calcium level left untreated it can cause multiple problems including causing calcium to spill into your urine as well as lead to kidney stones and/or kidney damage. The bones can also lose calcium and weaken. Though hyperparathyroidism is frequently diagnosed in a person with MEN I by the age of 20 by 50 years of age almost all those with MEN I will have hyperparathyroidism. Also as many as 88% of those MEN I will develop angiofibromas which are small raised papuels that can be either pink, skin colored or light brown. These angifibromas are usually found on the central part or your face.
The symptoms of MEN I depend on the size and location of the tumors. The most common symptoms are associated with parahyperthyroidism including nausea and vomiting, constipation and dehydration as well as poor appetite and abdominal pain. Most also have increased lethargy, depression and confusion.
The standard treatment for MEN I is surgical removal of the tumors, however, this is controversial as there tend to be multiple tumors and the tumors can return even after removed. Therefore each patient is evaluated on an individual basis to determine the best approach to effectively treat their particular tumors. Many doctors prefer to “watch and wait” using non-surgical medical treatments when possible.
Kimberly Allen is a registered nurse with an AND in nursing. She has worked in ACF, LCF and psychiatric facilities, although she spent most of her career as a home health expert. She is now a regular contributor to HealthAndFitnessTalk.com, dispensing advice and knowledge about medical issues and questions. You can reach her with any comments or questions at firstname.lastname@example.org.