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Acromegaly Pt2

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Pit-Pat


Pituitary Network


TayEndoweb

 

The text for this page was copied from a document produced by DR Koko Naing of Ninewells Hospital in Dundee.
I hope he does not mind my using it!
The full text and information on other endocrine conditions is available at tayendoweb

What is Acromegaly?

Acromegaly is a clinical condition caused by an excess of growth hormone (GH.) in the body of an adult. This excess is normally caused through a benign tumour of the pituitary gland. (Pituitary adenoma).

What is the Pituitary?

fantasyThe pituitary gland is a small -pea size- gland which is situated in a hollow, bony pouch, at the base of the brain just behind the bridge of the nose. It is the master gland of the endocrine system and controls the functions of the other endocrine glands.

Pituitary Adenoma?

An abnormal growth (Adenoma) can develop from the pituitary gland. This adenoma may, or may not activelyThe Thinker produce any hormones. It normally grows very slowly and does not spread throughout the body. The pituitary gland sits in a very limited space and is surrounded by some very important structures. Therefore, any enlargement or growth can have pressure effects upon the pituitary gland itself and also upon the surrounding area. This local pressure can cause headaches and visual disturbances and the disruption of hormone production can have subsequent systemic effects on the body.

What is Growth Hormone? (GH)

patternGrowth hormone is produced by the pituitary and has important effects on the various tissues of the body. In children, it is an essential element in achieving normal growth. In adults it is important for maintaining normal energy levels and for keeping body tissue (e.g. muscles and bones) healthy.

Normal control of GH production.

The Hypothalamus is that part of the brain which controls pituitary function. It releases hormones, which stimulate (GHRH), and Somatostatin, which blocks the release of GH and so acts against overproduction from the pituitary.

How common is Acromegaly?

FairyIt is a very rare condition, diagnosed in only four per million of the population each year.

How do I know that I have Acromegaly?

Symptoms, which are due to the increased levels of GH acting upon the tissues:

Appearance changes can be subtle and not always noticed by the patient or their family members:
Facial features coarsen as the skin becomes thicker and greasier.
There is an increase in size to the hands, feet, lower jaw, nose and ears (noticed as an increase in glove size, shoe size or a wedding ring that has become too tight.)
Sweating can be excessive and the voice deepens.
There is also muscle weakness, joint pain and a tingling sensation, which is due to nerves being trapped by enlarged tissues e.g. Carpal Tunnel Syndrome.
There is also a greater risk of high blood pressure and diabetes.

DiagramSymptoms, which are due to the enlarged pituitary affecting the surrounding structures:

These include headaches, visual disturbances and sinus problems.
Sleep apnoea can also be a symptom along with the resultant tiredness caused by disturbed sleep.
Underproduction of other pituitary hormones may cause, irregular periods, breast secretions or impotency.

Why is treatment needed?

Studies show that untreated Acromegalic patients are more likely to suffer from high blood pressure, diabetes, stroke and heart attack, with a reduced life expectancy when compared to the normal population. This returns to normal after successful treatment. The growth of the tumour can cause pressure effects on the surrounding structures with intolerable symptoms. Symptoms related to the effects of the excess GH on the different body tissue could also become intolerable.

Tests:

Normally, GH levels are very low and vary throughout the day.

  1. To confirm excessive GH production it is normal to perform an Oral Glucose Tolerance Test with Measurement of GH. This is performed after an overnight fast.
  2. Blood tests to check for normal pituitary hormone levels.
  3. Patients may be required to have a Complete Pituitary Function Test if there is any suspicion of pressure on the pituitary by the GH producing pituitary adenoma.
  4. Field of vision may be checked to make sure that the pituitary adenoma is not giving pressure to the major nerves, carrying information from the eye to the brain, which run very close to the normal pituitary.
  5. A Magnetic Resonance Imaging Scan (MRI) will be carried out to determine the size of the adenoma.

What treatments are normal?

Surgery

Removal of the GH producing pituitary adenoma using Trans Sphenoidal Surgery, (TSS), or very rarely, when tumours are very large, with Craniotomy.

External Radiotherapy.

In the case of a large tumour or, if the GH production remains in excess after the surgery then radiotherapy will be required.

Medical Treatment.

Medicines can be used to block the action of GH production. This is usually required whilst awaiting the effects of the radiotherapy, which may take a few years to complete its work. Sandostatin or Bromocriptine can be used to do this. The first is more commonly effective but requires injection and is expensive. Dose and frequency of injections is dependent on measured GH levels.

Hormone Replacement Therapy:

  1. Sometimes, part, or all, of the normal pituitary gland has to be removed during surgery, as it cannot always be possible to remove the tumour alone.
  2. Radiotherapy is also likely to cause under functioning of the whole pituitary gland after a few years.

In the above two situations, patients will require life long Hormone Replacement Therapy (HRT). This may include the following:
Hydrocortisone; Thyroxin; Antidiuretic Hormone (ADH); Testosterone; and the female hormones Oestrogen and Progestogen. (See also Hypopituitarism)

Tests following surgery.

  1. Reassessment of GH levels with a repeat of the Glucose Tolerance Test.
  2. A Complete Pituitary Function Test to monitor any changes to the other pituitary hormones. This is normally carried out six weeks after surgery.
  3. Blood tests to reflect the long term average GH levels (IGF 1).
  4. Repeated MRI scans to reassess the pituitary and the site of the tumour.
  5. If GH levels remain high then medical treatment will be required (e.g. Sandostatin). It is sometimes necessary to check the level of GH at different times of the day before adjusting the dosage of Sandostatin.

Additional points.

  1. Life expectancy after the successful treatment of Acromegaly returns to that of the normal population.
  2. If life long replacement therapy is required after surgery, the patient will be entitled to free prescriptions.
  3. If cortisol is required, the dose will need to be increased at times of acute illness. The patient should carry a steroid card (or SOS bracelet), which shows details of the drugs used. GPs should also be informed of any replacement therapy.
  4. Hydrocortisone is a steroid but as there may be little, or no steroid at all after surgery the patient is not in danger of the side effects associated with steroid misuse. Dosage is adjusted to keep within the normal steroid requirement of the body.
  5. It is very important to have regular check ups by the doctor so that the patients condition can be closely monitored.
  6. The patient's general appearance and the symptoms of excessive GH should recover after the treatment of Acromegaly, although this may take some time.
  7. Patients should inform the DVLC and his/her insurance companies of their condition and drug treatments.

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