Multiple Endocrine Neoplasia Type I
What are the symptoms?
Many of the health problems associated with MEN I occur frequently in the general population such as stomach ulcers, kidney stones, tiredness, headaches, low blood sugar, and high calcium levels. MEN I may, therefore, go undetected in families. Individuals may have tumours which are asymptomatic. The majority of tumours are slow growing and 'benign.' However, some are cancerous and so accurate diagnosis and treatment are essential. With early detection and treatment the potential problems caused by MEN I can be greatly reduced.
The range and severity of features associated with MEN I depends upon the glands in which tumours are located in any one individual. The location of the tumours varies among individuals, even within the same families. In most individuals, tumours in the parathyroids glands are the first sign of MEN I. The parathyroid glands control calcium in the blood, bones and urine. Over-activity of the parathyroid gland (hyperparathyroidism) is associated with high levels of calcium in the blood (hyercalcemia). The medical problems associated with hyperparathyroidism include tiredness, weakness, muscle or bone pain, indigestion, kidney stones or thinning of the bones, poor memory, irritability, ulcers and bone fractures.
The pancreas releases digestive juices into the intestines. Tumours in the pancreatic islet cells are associated with the release of excessive amounts of hormones such as gastrin, glucagons or vasoactive intestine polypeptide. Normally, gastrin causes the stomach to secrete enough acid needed for digestion. Over-secretion of gastrin in MEN I is associated with the formation of severe ulcers in the stomach and small intestine which may cause severe vomiting with blood and/or diarrhoea. About one third of individuals with MEN I have gastrin-releasing tumours (otherwise known as gastrinomas). If left untreated, these may cause rupture of the stomach or intestine. In some cases, they are fatal.
The pituitary gland plays a critical role in regulating growth and development, metabolism and reproduction. It releases prolactin and other key hormones. Symptoms associated with tumours in the pituitary gland include a loss or irregularity of the menstrual cycle, headaches and eye problems.
Background
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