MEN1

MEN-1 (Multiple Endocrine Neoplasia-1) is a complex condition that occurs in approximately 1 in 100,000 people. It is an inherited condition, with a 50:50 risk of occurring in the child of a parent with the disease. It may also occur in people without other affected family members, because of a problem with the gene that causes MEN-1 occuring after birth (known as an index case).

MEN-1 classically leads to problems in 3 endocrine glands:

Parathyroid glands (occurring in 95% of people with MEN-1), leading to hyperparathyroidism (overactivity of the parathyroid glands). This is often the first recognisable problem with MEN-1 and usually affects all four parathyroid glands. Surgical treatment most commonly consists of removal of all parathyroid glands.

Pancreatic endocrine gland tumours (occurring in 50-75% of people with MEN-1). Multiple tumours are common, and often require surgical removal.

Pituitary gland tumours (occurring in 30-55% of people with MEN-1), which may require surgical removal or medical treatment.

In addition to the above disease patterns, people with MEN-1 may also have a number of other conditions as part of the syndrome. These include tumours of the adrenal glands (adrenocortical tumours), gut carcinoid tumours, and problems with the tissues forming the skin and connective tissues.

Because of the complex nature of MEN-1, all people with this condition should be seen in a clinic run by specialist teams that can monitor all of these issues, and offer treatment when problems arise.

At University Hospital Aintree a specialist MEN clinic is provided. In addition to Mr Hardy, this clinic is run by doctors that specialise in endocrinology, medical genetics and paediatric endocrinology, allowing a multidisciplinary approach to monitoring and treatment.

University Hospital Aintree also has a national reputation for treatment of carcinoid tumours, and is on the same site as the Walton centre for Neurology and Neurosurgery. It is therefore well placed to offer a comprehensive service for people with MEN-1.

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