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Acromegaly and Gigantism

Acromegaly and Gigantism

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Published by: nfacma on May 06, 2012
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Acromegaly and Gigantism

Overproduction of growth hormone causes excessive growth. In children, the condition is called gigantism. In adults, it is called acromegaly.

• • • • • •

Excessive growth hormone is almost always caused by a noncancerous (benign) pituitary tumor. Children develop great stature; adults develop deformed bones but do not grow taller. Heart failure, weakness, and vision problems are common. The diagnosis is based on blood tests and x-rays of the skull and hands. Other imaging tests are done to look for the cause.

A combination of surgery, radiation therapy, and drug therapy is used to treat the overproduction of growth hormone.
Growth hormone stimulates the growth of bones, muscles, and many internal organs. Excessive growth hormone, therefore, leads to abnormally robust growth of all of these tissues. Overproduction of growth hormone is almost always caused by a noncancerous (benign) pituitary tumor (adenoma). Certain rare tumors of the pancreas and lungs also can produce hormones that stimulate the pituitary to produce excessive amounts of growth hormone, with similar consequences.

If excessive growth hormone production starts before the growth plates have closed (that is, in children), the condition produces gigantism. The long bones grow enormously. A person grows to unusually great stature, and the arms and legs lengthen. Puberty may be delayed, and the genitals may not develop fully. In most cases, excessive production of growth hormone begins between the ages of 30 and 50, long after the growth plates of the bones have closed. Increased growth hormone in adults

particularly in the outer visual fields. they are usually not recognized for years. the abnormality of the extreme growth becomes clear. Serial photographs (those taken over many years) may help a doctor establish the diagnosis. The sebaceous and sweat glands in the skin enlarge. which typically darkens. Did You Know? • A woman with acromegaly can produce breast milk even if she is not breastfeeding. Nearly all women with acromegaly have irregular menstrual cycles. In adults. the tongue may enlarge and become more furrowed. Larger rings. Some women produce breast milk even though they are not breastfeeding (galactorrhea) because of either too much growth hormone or a related increase in prolactin. and its function may be so severely impaired that heart failure occurs. making the voice deep and husky. Diagnosis In children. increases as the skin thickens. acromegaly often is not diagnosed until many years after the first symptoms appear. crippling degenerative arthritis may occur. Coarse body hair. creating a barrel chest. and certain tumors. heart failure. In both gigantism and acromegaly. particularly affecting the large intestine. About one third of men who have acromegaly develop erectile dysfunction. Overgrowth of the jawbone (mandible) can cause the jaw to protrude (prognathism). The heart usually enlarges. producing excessive perspiration and often an offensive body odor. sleep apnea. rapid growth may not seem abnormal at first. because the changes induced by high levels of growth hormone occur slowly. Life expectancy is reduced in people with untreated acromegaly. high blood pressure (hypertension). Sometimes a person feels disturbing sensations and weakness in the arms and legs as enlarging tissues compress the nerves.produces acromegaly. in which the bones become deformed rather than elongated. There is also an increased likelihood of developing diabetes mellitus. shoes. after many years. Nerves that carry messages from the eyes to the brain may also be compressed. and hats are needed. however. Eventually. The person's facial features become coarse. Because changes occur slowly. An x-ray of . which may become cancerous. gloves. Cartilage in the voice box (larynx) may thicken. Joint pain is common. The pressure on the brain may also cause severe headaches. causing loss of vision. and the hands and feet swell. The ribs may thicken.

Radiation therapy involves the use of supervoltage irradiation. The diagnosis is confirmed by blood tests. thus. which usually show high levels of both growth hormone and insulin-like growth factor 1 (IGF-1). A computed tomography (CT) or magnetic resonance imaging (MRI) scan is usually done to look for abnormal growths in the pituitary gland. Because acromegaly is usually present for some years before being diagnosed. such as stereotactic radiosurgery. This test is not necessary when the clinical features of acromegaly are obvious. the IGF-1 level is high.bromocriptine and other drugs that act like dopamine are of some benefit. These drugs includeoctreotide . however. Blood sugar levels and blood pressure may be high. doctors may need to use a combination of surgery. a tumor is seen on these scans in most people. as normal tissue is often also affected. are those that are forms of somatostatin. particularly if a substantial amount of the tumor remains after surgery and acromegaly persists. or a tumor is seen in the pituitary on scanning. most often without causing deficiency of other pituitary hormones. and often results in later deficiencies of other pituitary hormones. a single high level of growth hormone in the blood is insufficient to make the diagnosis. which is less traumatic than surgery. More directed radiation therapy.the skull may show thickening of the bones and enlargement of the nasal sinuses. most commonly a glucose drink (the oral glucose tolerance test). Treatment Stopping or reducing the overproduction of growth hormone is not easy. Unfortunately. Doctors must give something that would normally suppress growth hormone levels. Because growth hormone is released in short bursts and the levels of growth hormone often fluctuate dramatically even in people without acromegaly. and surgery alone does not usually produce a cure. Occasionally. radiation therapy. It results in an immediate reduction in tumor size and growth hormone production. Radiation therapy is often used as a follow-up treatment. The most effective drugs. tumors are often large by the time they are found. Drug therapy can also be used to lower growth hormone levels. and show that normal suppression does not occur. and drug therapy. however. Surgery by an experienced surgeon is currently regarded as the best first treatment for most people with acromegaly caused by a tumor. This treatment may take several years to have its full effect. is being tried to speed results and spare the normal pituitary tissue. X-rays of the hands show thickening of the bones under the fingertips and swelling of the tissue around the bones. the hormone that normally blocks growth hormone production and secretion.

. These drugs are effective in controlling acromegaly in many people as long as they continue to be taken (they do not provide a cure). such aspegvisomant . This may change as such drugs become longer acting and more readily available. are now available and may be useful for people who do not respond to somatostatin-type drugs. Their use has been limited by the need to inject them and by their high cost. Several new growth hormone blocker drugs.and its newer long-acting analogs. which only have to be given about once a month.

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