SHARES
We learnt yesterday that Malaysia-born actress and TV host, Tiffany Leong, passed away after battling a hereditary disorder – MEN. But do we know anything about what caused her end? Read on to find out…
1.MEN?? What does that mean??
MEN stands for Multiple Endocrinal Neoplasia – a group of ailments that affect the endocrine system (the human body’s own network of hormone-producing glands).
- Hormones are chemical messengers that travel through the bloodstream and control the functioning of cells and tissues throughout the body.
- Multiple endocrine neoplasia typically involves tumours (neoplasia) in at least two endocrine glands; tumours can also develop in other organs and tissues. These growths may be benign (noncancerous) or malignant (cancerous). If the tumours become cancerous, the condition can be fatal.
MEN syndromes can be said to be a condition where increased activity and enlargement of a few endocrine glands is seen. MEN syndromes are typically (but not always) inherited. Multiple endocrine neoplasia (MEN) type I is a hereditary disease – that which is passed down through families, and in which one or more of the endocrine glands are overactive or form a tumour.
The endocrine glands that are most commonly involved include:
- Pancreas
- Parathyroid
- Pituitary
2. What are the types of MEN syndromes?
There are three main types of multiple endocrine neoplasia (MEN) –MEN 1, MEN 2a and MEN 2b. Each type of MEN is associated with a specific cluster of illnesses. Physicians use the term “syndrome” when clinical diseases come in groups of three. Each of the three types of MEN syndromes, therefore, has three diseases. The main health problems in each of the MEN syndromes are:
- MEN 1 = Parathyroid tumours, pituitary tumours and pancreatic
- MEN 2a = Medullary thyroid cancers (MTC), parathyroid tumours and
- MEN 2b = Medullary thyroid cancers, neuromas and pheochromocytoma.
There are specific genetic causes for each of the three types of MEN. A family with a specific MEN will have only one type of the syndrome – either MEN 1, MEN 2a or MEN 2b. Therefore, families with one type of MEN do not have an increased risk of developing another type of MEN.
3. What does it mean to be a Genetic Disease?
Each cell in the body is made up of several thousand genes which regulate every aspect of body function. Every person is made up of the genes they receive from their parents at the time of conception. Half of a person’s genes come from the mother and half from the father. People with MEN inherit an abnormality (mutation) in one specific gene (that is why they only get one type of MEN syndrome, and their children are at risk only for that same MEN syndrome). Only people who inherit the abnormality in one of the specific MEN genes can develop MEN.
4. What causes MEN?
MEN I is caused by a defect in a gene that carries the code for a protein called menin. The condition causes tumours of various glands to appear in the same person, but not necessarily at the same time.
The disorder may occur at any age, and it affects men and women alike. A family history of this disorder increases the risk.
5. What are the symptoms of the syndromes?
Symptoms vary from person to person depending on which gland is involved. Some of the symptoms maybe:
- Abdominal pain
- Burning, aching, orhunger discomfort in the upper abdomen or lower chest that is relieved by antacids, milk, or food
- Anxiety
- Headache
- Loss of body or facial hair (in men)
- Muscle pain
- Weakness
- Nausea and vomiting
- Fatigue
- Lack of menstrual periods (in women)
- Mental changesor confusion
- Vision problems
- Loss of appetite
6. How common is multiple endocrine neoplasia?
MEN type 1 affects about 1 in 30,000 people; MEN type 2 affects about 1 in 35,000 people. Among the subtypes of type 2, type 2A is the most common form. Type 2B is relatively uncommon, accounting for about 5 % of all cases of type 2.
7. What is the treatment for MEN?
Surgery to remove the diseased gland is the treatment of choice. Medicine is available to reduce the excess acid production in the stomach caused by some tumours (gastrinomas), and also to reduce the risk of ulcers.
Hormone replacement therapy is done when the entire gland is removed or if the gland does not produce enough hormones.
Sources:
by Qian
Qian's interest in healthcare industry came about after her father was diagnosed with bladder cancer. This experience has led her to become a strong believer in empowering individuals to take charge of their own health. View all articles by Qian.
