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Transcript of Acromegaly
Acromegaly is caused by the pitutary gland overproducing growth hormone (GH) after puberty (the union of epiphysis) .
In adults, a tumor is the most common cause of too much GH production.
Most cases of acromegaly are caused by a noncancerous (benign) tumor (adenoma) of the pituitary gland. The tumor secretes excessive amounts of growth hormone.
In a few people with acromegaly, tumors in other parts of the body, such as the lungs, pancreas or adrenal glands, cause the disorder. Sometimes, these tumors actually secrete GH. In other cases, the tumors produce a hormone called growth hormone-releasing hormone (GH-RH), which stimulates the pituitary to make more GH.
Acromegaly is a hormonal disorder that develops when your pituitary gland produces too much growth hormone during adulthood. When this happens, your bones increase in size, including those of your hands, feet and face. Acromegaly usually affects middle-aged adults.
In children who are still growing, too much growth hormone can cause a condition called gigantism. These children have exaggerated bone growth and an abnormal increase in height.
Because acromegaly is uncommon and physical changes occur gradually, the condition often isn't recognized immediately; sometimes not for years. If not treated promptly, acromegaly can lead to serious illness and even become life-threatening. However, available treatments for acromegaly can reduce your risk of complications and significantly improve your symptoms, including the enlargement of your features.
Because Acromegaly tends to progress slowly, early signs may not be readily apparent for several years. Sometimes, people notice the condition only by comparing old photographs.
16. Increased chest size (barrel chest)
1. Enlarged hands and feet
2. Coarsened, enlarged facial features
3. Coarse, oily, thickened skin
4. Excessive sweating and body odor
5. Small outgrowths of skin tissue (skin tags)
6. Fatigue and muscle weakness
7. A deepened, husky voice due to enlarged vocal cords and sinuses
8. severe snoring due to obstruction of the upper airway
9. Impaired vision
11. Enlarged tongue
12. Pain and limited joint mobility
13. Menstrual cycle irregularities in women
14. Erectile dysfunction in men
15. Enlarged liver, heart, kidneys, spleen and other organs
Non Pharmacological Treatment
1) somatostatin analogues – octreotide (Sandostatin) or lanreotide (Somatuline).
2) Dopamine agonists(Bromocriptine (Parlodel) or (Cabergoline)
3) Growth hormone receptor antagonists:pegvisomant (Somavert)
5) Radiation therapy
These are somatostatin analogues , which stops GH production.
The long-acting forms of these drugs must be injected every 2 to 4 weeks for effective treatment. Most patients with acromegaly respond to this medication.
They are effective for long-term treatment.
They have also been used successfully to treat patients with acromegaly caused by non-pituitary tumors.
Somatostatin analogues are also sometimes used to shrink large tumors before surgery.
Somatostatin analogues – octreotide (Sandostatin) or lanreotide (Somatuline).
For those who are unresponsive to somatostatin analogues, or for whom they are otherwise contraindicated, it is possible to treat using one of the dopamine agonists,.
These have the advantage of being tablets rather than injections.
These drugs can also be used as an adjunct to somatostatin analogue therapy.
( Bromocriptine (Parlodel) or Cabergoline) )
The latest development in the medical treatment of acromegaly is the use of growth hormone receptor antagonists. By blocking the action of the endogenous growth hormone molecules, this compound is able to control disease activity of acromegaly in virtually all patients. Pegvisomant has to be administered subcutaneously by daily injections.
Growth hormone receptor antagonists : pegvisomant (Somavert)
Surgery is most successful in patients with blood GH levels below 40 ng/ml before the operation and with pituitary tumors no larger than 10 mm in diameter. Success depends on the skill and experience of the surgeon.
Complications of surgery may include cerebrospinal fluid leaks, meningitis, or damage to the surrounding normal pituitary tissue, requiring lifelong pituitary hormone replacement.
Radiation therapy has been used both as a primary treatment and combined with surgery or drugs.
It is usually reserved for patients who have tumor remaining after surgery. These patients often also receive medication to lower GH levels. Radiation therapy is given in divided doses over four to six weeks. This treatment lowers GH levels by about 50 percent over 2 to 5 years. Patients monitored for more than 5 years show significant further improvement. Radiation therapy causes a gradual loss of production of other pituitary hormones with time.
Loss of vision and brain injury, which have been reported, are very rare complications of radiation treatments
No single treatment is effective for all patients.
Treatment should be individualized depending on patient characteristics, such as age and tumor size.
If the tumor has not yet invaded surrounding brain tissues, removal of the pituitary adenoma by an experienced neurosurgeon is usually the first choice.
After surgery, a patient must be monitored for a long time for increasing GH levels. If surgery does not normalize hormone levels or a relapse occurs, a doctor will usually begin additional drug therapy. The current first choice is generally octreotide or lanreotide. However, bromocriptine or cabergoline are much cheaper and easier to administer. With both types of medication, long-term therapy is necessary because their withdrawal can lead to rising GH levels and tumor re-expansion.
Radiation therapy is generally used for patients whose tumors are not completely removed by surgery; for patients who are not good candidates for surgery because of other health problems; and for patients who do not respond adequately to surgery and medication.
Choice of treatment
No observed non pharmacological treatment for acromegaly
If acromegaly is suspected, medical imaging and medical laboratory investigations are generally used together to confirm or rule out the presence of this condition.
IGF1 provides the most sensitive lab test for the diagnosis of acromegaly(IGF1Insulin-like growth factor , also called somatomedin C )levels can be measured in the blood in 10-1000 ng/ml amounts. As levels do not fluctuate greatly throughout the day for an individual person, IGF-1 is used by physicians as a screening test for growth hormone deficiency and excess in acromegaly and gigantism, and a GH suppression test following an oral glucose load, which is a very specific lab test, will confirm the diagnosis following a positive screening test for IGF1. A single value of the growth hormone (GH) is not useful in view of its pulsatality (levels in the blood vary greatly even in healthy individuals).
Magnetic resonance image of a pituitary macroadenoma that caused acromegaly with compression of the optic chiasm.
GH levels taken 2 hours after a 75 or 100 gram glucose tolerance test are helpful in the diagnosis: GH levels are suppressed below 1 μg/L in normal people, and levels higher than this cutoff are confirmatory of acromegaly.
Other pituitary hormones have to be assessed to address the secretory effects of the tumor as well as the mass effect of the tumor on the normal pituitary gland. They include TSH (thyroid stimulating hormone), gonadotropic hormones (FSH, LH), ACTH (adrenocorticotropic hormone), prolactin.
Continue : Diagnosis
Prognosis in acromegaly is determined by persistent elevation of growth hormone levels above 5 mU/l (2•5 μg/l)
Elevated growth hormone is a cardinal feature of acromegaly from the biological view point. Growth hormone stimulates IGF-I (Insulin-like growth factor 1 ) secretion and that of its major binding protein IGFBP-3(Insulin-like growth factor-binding protein 3) . In these circumstances, where hyperinsulinaemia is present, IGFBP-1 (Insulin-like growth factor-binding protein 1) levels, which are inversely related to insulin, are suppressed.
Failure of suppression of growth hormone after oral glucose (>2 mU/1 (1 μg/l)) is the cardinal biochemical feature of acromegaly.
For monitoring purposes, growth hormone values, either basal or during the day are useful.
Done by :
Yara Ayman 480
Yasmeen Elsaid 481
Yasmeen Gamal 482
Yasmeen Sobhy 483
Yasmeen Amr 484
Yasmeen Mohamed 485
Yasmeen Magdy 486
Yasmeen Mostafa 487
Yasmeen Mamdouh 488
Yomna Ahmed Sakr 489
Yomna Shereif 490
Youstina todry 491