Acromegaly

Acromegaly – General Information

The name Acromegaly comes from the Greek combination of akros which means “extreme” or “extremities” and megalos which means large, so that would sum up as extremities enlargement. This disease may appear as a result of hormonal disorder which arises when the pituitary gland is producing growth hormone (HGH) in excess. Usually it begins when a growth hormone is producing tumor which is derived from a cell type that is distinct (somatotrops) and is called by convention pituitary adenoma. This disorder usually affects middle-aged adults, and can lead to premature death or serious illness. Because it has a slow progression and an insidious pathogenesis, Acromegaly is hard to be diagnosed in early stages and can be mistaken for other diseases for many years. 90% of the patients who have this disease have a benign tumor which is causing the overproduction of growth hormones. As these tissues expand, they may suppress and surround brain tissues, like the optical nerve. These tissue expansions may cause visual disturbances and headaches, which can usually be associated with Acromegaly. If the tissue around the pituitary gland is compressed, then breast discharge and changes in menstruation may appear at women and impotence at men because of the reduced and insufficient testosterone secretion. A lot of pituitary tumors appear spontaneously and are not inherited generically. Many can also arise from alteration of a single pituitary cell which may lead to tumor and increased cell division.

Acromegaly – Symptoms

Features which may result from a raised level of growth hormones and or expanding tumor can be:

1. Soft tissue swelling in hands and feet
2. Protrusion of the lower jaw and brow
3. Enlarging hands
4. Enlarging feet
5. Carpal tunnel syndrome and arthritis
6. Macroglossia(the tongue is enlarged)
7. The space between the teeth increases
8. Failure of the kidney
9. Failure of the heart
10. Optical chiasm compression which may lead to vision loss in outer visual fields (bitemporal hemianopia)
11. Headaches
12. Diabetes mellitus
13. Hypertension
14. Heart enlargement
15. Face sebum production and increased palm sweating may indicate that the active growth hormone is producing pituitary tumors. Symptoms like these can also be utilized to view the activity of Acromegaly after surgery.

In some other patients, Acromegaly can also be caused by tumors or the adrenal glands, pancreas and lungs. These tumors also result in excessive growth hormone secretion, either caused by the fact that they also produce growth hormones, or by the fact that they produce GHRH, the hormone which helps the pituitary gland to produce growth hormone. GHRH excesses can be seen in blood analysis and doctors can deduce that the Acromegaly isn’t caused by a defect of the pituitary gland. When these other types of tumors are surgically removed from the body, the level of growth hormones drop and the symptoms of the disease are radically improved.

Acromegaly – Treatment

Acromegaly Treatment leads to the reducing growth hormone production to a normal level, to make the pressure which the pituitary tumor forces on surrounding brain areas disappear, to preserve a normal functionality of the pituitary gland and to ameliorate or reverse this disease’s symptoms. Treatment options in our days are surgery, and radiation or drug therapies.

Surgery is an effective and quick treatment, and has two alternative methods. The first method is known as Endonasal Transphenoidal surgery and it involves reaching the pituitary gland using an incision in the cavity wall of the nose. This wall can be reached if you pass through the nostrils using medical equipment such as microsurgical instruments. The second surgery method is called Transphenoidal surgery and involves an incision of the gum which is situated beneath the upper lip. Then the septum will be incised for the surgeon to reach the nasal cavity, where the pituitary gland can be found.
The first form of surgery is less invasive and it has a shorter time of recovery than the second one, and the probability of removing the whole tumor is better, and the side-effects are lessened.

The first drug is called bromocriptine (parlodel) and is divided into doses of 20 mg and results in the reduction of growth hormone production from some tumors which are pituitary. This drug’s side-effects can be gastrointestinal upset, vomiting, nasal congestion and light-headedness when you stand. The side-effects can be stopped if the medication is administered in a low dose before bedtime and is taken with food, and is slowly raised to standard dose. This method is attractive because bromocriptine must be taken orally as a primary drug. It can also be combined with other treatments.
The second drug which is used against Acromegaly is ocreotide (Sandostatin) with lanreotide (Somatulin).Both can be considered synthetic forms of somatostatin, a brain hormone which stops growth hormone production. The powerful and long-acting forms of these drugs must be injected once 2-4 weeks, depending on the patient, for an effective treatment. Most patients who have Acromegaly usually respond to this medication. The effect of this drug is that one hour after injection, growth hormone production levels get to normal and headaches almost disappear in minutes. Many studies have shown us that these drugs are effective in the long range and that they are also effective on non-pituitary tumor caused Acromegaly.

Radiation Therapycan be used as a primary treatment or it can be combined with drugs or surgery. It is usually used on patients who have tumor remnants after surgery. These patients also ingest drugs to reduce the growth hormone production. This type of treatment can be given in doses which are divided over 4 to 6 weeks and can reduce growth hormone secretion in about two to five years. Patients which have taken this treatment for more than 5 years show a reduction of growth hormone production of about 50 percent.