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Multiple Endocrine Neoplasia MEN I - Medical Malpractice
Multiple endocrine neoplasia type I or MEN I is a genetic disorder that causes specific tumours in the endocrine glands and in the duodenum. It is also known as Wermer's syndrome after one of the doctors that first identified the disease. The disease affects both genders equally and occurs in one out of 30,000 live births. MEN I affects the endocrine glands, which are the glands that release hormones into the body to affect other areas of the body. Hormones are specific chemicals that bind to cells and affect certain cells of the body.
In MEN I, the affected glands include the pancreas, the parathyroid glands and the pituitary gland. The tumours that form often form at exactly the same time, making the diagnosis easier. The glands form tumours but do not tend to be overactive.
When the parathyroid glands are involved, they form hyperparathyroidism. The parathyroid glands are four glands buried beneath and within the thyroid gland. In MEN I, the disease forms parathyroid adenomas, releasing too much parathyroid hormone (PTH) into the bloodstream. This messes with the calcium in the blood and with the bones that receive the calcium. There is too much calcium in the blood when a person has MEN I because of adenomas of all four parathyroid glands. The glands are not cancerous-just overactive. The bones can lose calcium and can become weak. Most people with hyperparathyroidism from MEN I get the disease before the age of 50 but some can get it before the age of 20. Symptoms include fatigue, muscle or bony pain, weakness, constipation, kidney stones or indigestion. Osteoporosis can also come from this disorder. Treatment includes surgery to remove at least three if not all of the glands making up the parathyroid gland.
The pancreas is also affected by MEN I. The pancreas releases insulin, glucagon, somatostatin, vasoactive intestinal peptide and gastrin. In MEN I, it is the gastrin that is over secreted in a condition known as a gastrinoma of the pancreas. Gastrin causes too much stomach acid to be secreted and serious diarrheal symptoms. The person can develop ulcers of the stomach quite easily in an illness called Zollinger-Ellison syndrome. It is difficult to treat the ulcers caused by this syndrome. There are often many gastrinomas in the pancreas at the same time, making surgical correction of this aspect of the disorder very difficult. The treatment revolves around taking acid pump inhibitors like Prilosec in order to block the excess gastrin production. Insulinomas can also be a part of MEN I and the person often has low blood sugar or hypoglycaemia. About ten percent of people with MEN I go on to develop insulinomas. Even less common are gastrinomas which lead to diabetes mellitus. Tumours that secrete ACTH can also come out of the pancreas in MEN I. Rarely gonadotropin-releasing hormone tumours can be a problem in cases of MEN I.
The pituitary gland is a small gland at the base of the brain. It produces multiple hormones used in reproduction, metabolism and other body functions. About a fourth of patients develop prolactinomas, which secrete prolactin that affects milk production by the mammary glands. There can be an excessive amount of breast milk produced and it can interfere with fertility in both men and women.
There may need to be no treatment for prolactinomas except for dopamine agonist medication that can shrink the tumour and can result in a lower production of prolactin.
Less commonly, MEN I can cause tumours that secrete ACTH, which stimulates the adrenal glands to make too much cortisol. This results in pituitary-induced Cushing's syndrome. Other pituitary tumours less common in MEN I include growth hormone tumours.
The treatment of MEN I is directed at slowing the production of the various hormones secreted in the disease. It can be very difficult to treat, especially if there is no antidote for the hormone secreted. The glands can be surgically removed, with the exception of the pituitary gland.
Multiple Endocrine Neoplasia MEN II - Medical Malpractice
Multiple endocrine neoplasia type II is a rare inherited disease that yields three major endocrine problems. The three endocrine glands involved in MEN II are the parathyroid glands, the adrenal glands and the thyroid glands. It is a disease in which almost all patients with the disease develop medullary thyroid cancer at some point in their lives. There can be an overactive adrenal gland or glands and you can develop a pheochromocytoma (too much adrenaline produced in the adrenal gland). The parathyroid glands, located in the neck, can become overactive as well and can produce too much parathyroid hormone. Only the thyroid gland portion of the disease is actually cancerous.
Multiple endocrine neoplasia type II is very rare. It happens in about 1 in every 20,000 individuals who carry the gene for the disease. It is an autosomal dominant disorder, inherited to both men and women in all races. Half of all children of those with the disease have the potential to get the disease.
MEN II increases the activity of the parathyroid, thyroid and adrenal glands. They increase the number of certain hormones in the body so that the body does not act properly. The glands tend to enlarge as well as producing too much hormone.
Most people inherit the disease at birth but don't get symptoms of the disease until they reach the age of ten years or more. The endocrine glands tend to go bad at different times of the patient's life. This makes the diagnosis of MEN II somewhat complicated. It can be perceived that the person has two or more different endocrine diseases without the linkage made to the cohesive diagnosis of MEN II. The likelihood of getting the various endocrine disorders associated with MEN II increases with age. Most people have at least one overactive gland by the age of 30 with symptoms holding off until they are older than 30. If one gland is affected, doctors should check the other glands for abnormalities, even if there are no symptoms.
Patients who have the MEN II gene, which can be assessed at any age, should have their thyroid gland surgically removed while they are in their early to mid-teen years because of the high incidence of thyroid cancer. Those who should be tested for the MEN II gene are those with a parent with the disease. It doesn't certainly need to be tested for in everyone because it is so rare, even if the person has one gland affected by an adenoma. MEN I and MEN II are completely different disorders with different genes involved.
There is no cure for MEN II; however, with removal of the thyroid gland early in life, the risk of cancer of the thyroid can be eliminated. Because MEN II is hereditary, it can only be prevented in future generations by not having children if you have the disease. In the future, genetic enhancements can be done to change the way the MEN II gene works but research is a long way from that.
The diagnosis of MEN II is twofold. First you need a diagnosis to see if the person has the MEN II gene. Then you need tests for hyperactivity of the affected glands. The test for the MEN II gene is complex but can be done at specialized laboratories. Those with a strong family history of MEN II should be genetically tested for the disease.
If you are positive for the MEN II gene, you should have your parathyroid hormone level checked to make sure you don't have overactive parathyroid glands. If the hormone level is high, you need to consider having the parathyroid glands removed. They can also be removed at the time of the removal of the thyroid gland, because they are imbedded in the thyroid gland. You will have to have hormone replacement after that. The same is true of thyroid hormone, which will need to be removed in the early teens. Thyroid hormone replacement is cheap and easy to take and will normalize the thyroid hormone in the system. The test for a pheochromocytoma or overactive adrenal gland is a test for epinephrine or itsLEGAL HELPLINE: ☎ 855 804 7125
The author of the substantive medical writing on this website is Dr. Christine Traxler MD whose biography can be read here