GIGANTISME DAN AKROMEGALI PDF

pergelangan tangan, dan pergelangan kaki dan dengan berkeringat banyak (ak ́ ́ro-meg ́ua-le) a condition gigantisme. akromegali (Ak”ro-meg’ua-le) suatu. Komplikasi akromegali / gigantisme dapat berupa hipopituitarisme, Stimulasi kardiomiosit yang disebabkan oleh GH dan IGF-I dikaitkan dengan status. Acromegaly is a disorder that results from excess growth hormone (GH) after the growth plates .. Kasper, Dennis; Fauci, Anthony; Hauser, Stephen; Longo, Dan; J. Jameson; Loscalzo, Joseph. (April 8, ). Harrison’s Principles of Internal.

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Acromegaly is a disorder that results from excess growth hormone GH after the growth plates have closed. Acromegaly is typically due to the pituitary gland producing too much growth hormone. Treatment options include surgery to remove the tumor, medications, and radiation therapy.

Acromegaly affects about 6 perpeople. In some cases, they may compress the optic nerves. Expansion of the tumor may cause headaches and visual disturbances. In addition, compression of the surrounding normal pituitary tissue can alter production of other hormonesleading to changes in menstruation and breast discharge in women and impotence in men because of reduced testosterone production.

A marked variation in rates of GH production and the aggressiveness of the tumor occurs. Some adenomas grow slowly and symptoms of GH excess are often not noticed for many years. Other adenomas grow rapidly and invade surrounding brain areas or the sinuseswhich are located near the pituitary. In general, younger patients tend to have more aggressive tumors.

Most pituitary tumors arise spontaneously and are not genetically inherited. Many pituitary tumors arise from a genetic alteration in a single pituitary cell that leads to gigantism cell division and tumor formation. This genetic change, or mutationis not present at birth, but is acquired during life.

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The mutation occurs in a gene that regulates the transmission of chemical signals within pituitary cells; it permanently switches on the signal that tells the cell to divide and secrete growth hormones.

The events within the cell that cause disordered pituitary cell growth and GH oversecretion currently are the subject of intensive research. Pituitary adenomas and diffuse somatomammotroph hyperplasia may result from somatic activating mutations GNASwhich may be acquired or associated with McCune-Albright syndrome. In a few patients, acromegaly is caused not by pituitary tumors, but by tumors of the pancreaslungsand adrenal glands. These tumors also lead to an excess of GH, either because they produce GH themselves or, more frequently, because they produce GHRH growth hormone releasing hormonethe hormone that stimulates the pituitary to make GH.

In these patients, the excess GHRH can be measured in the blood and establishes that the cause of the acromegaly is not due to a pituitary defect. When these nonpituitary tumors are surgically removed, GH levels fall and the symptoms of acromegaly improve. In patients with GHRH-producing, non-pituitary tumors, the pituitary still may be enlarged and may be mistaken for a tumor. Therefore, it is important that physicians carefully analyze all “pituitary tumors” removed from patients with acromegaly so as to not overlook the possibility that a tumor elsewhere in the body is causing the disorder.

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, 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 GH is not useful in view of its pulsatality levels in the blood vary greatly even in healthy individuals.

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GH levels taken 2 hours after a or gram glucose tolerance test are helpful in the diagnosis: Other pituitary hormones must be assessed to address the secretory effects of the tumor, as well as the mass effect of the tumor on the normal pituitary gland.

An MRI of the brain focusing on the sella turcica after gadolinium administration allows for clear delineation of the pituitary and the hypothalamus and the location of the tumor.

A number of other overgrowth syndromes can result in similar problems. Pseudoacromegaly is a condition with the usual acromegaloid features, but without an increase in growth hormone and IGF It is frequently associated with insulin resistance. There is no known cure for acromegaly. The goals of treatment are to reduce GH production to normal levels, to relieve the pressure that the growing pituitary tumor exerts on the surrounding brain areas, to gigantizme normal pituitary function, and to reverse or ameliorate the aromegali of acromegaly.

Currently, treatment options include surgical removal of the tumor, drug therapy adn, and radiation therapy of the pituitary. The primary current medical treatment of acromegaly is to use somatostatin analogues — octreotide Sandostatin or lanreotide Somatuline.

These somatostatin analogues are synthetic forms of a brain hormone, somatostatinwhich stops GH production.

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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. In many gigntisme, GH levels fall within one hour and headaches improve within minutes after the injection. Octreotide and lanreotide are effective for long-term akromgeali.

Octreotide and lanreotide have also been used successfully to treat patients with acromegaly caused gigantiisme non-pituitary tumors. Because octreotide inhibits gastrointestinal and pancreatic function, long-term use causes digestive aakromegali such as loose stools, nausea, and gas in one third of patients.

In addition, approximately 25 percent of patients develop gallstoneswhich are usually asymptomatic. 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 agonistsbromocriptine or cabergoline.

As tablets rather than injections, they cost considerably less. These drugs can also be used as an adjunct to somatostatin analogue therapy. They are most effective in those whose pituitary tumours cosecrete prolactin. Side effects of these dopamine agonists include gastrointestinal upset, nausea, vomiting, light-headedness when standing, and nasal congestion.

These side effects can be reduced or eliminated if medication is started at a very low dose at bedtime, taken with food, and gradually increased to the full therapeutic dose. Bromocriptine lowers GH and IGF-1 levels and reduces tumor size in fewer than half of patients with acromegaly. Some patients report improvement in their symptoms giganisme their GH and IGF-1 levels still are elevated. The giganttisme development in the medical treatment of acromegaly is the use of akromeali hormone receptor gigantusme.

The only giantisme member of this family is pegvisomant Somavert. 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. Combinations of long-acting akronegali analogues and weekly injections of pegvisomant seem to be equally effective as daily injections of pegvisomant.

Surgery is a rapid and effective treatment, of which there are two alternative methods. The first method, a procedure known as endonasal transphenoidal surgery, involves the surgeon reaching the pituitary through an incision in the nasal cavity wall.

The wall is reached by passing through the nostrils with microsurgical instruments. The second method is transphenoidal surgery during which an incision is made into the gum beneath the upper lip. Further incisions are made to cut through the septum to reach the nasal cavity, where the pituitary is located. Endonasal transphenoidal surgery is a less invasive procedure with a shorter recovery time than the older method of transphenoidal surgery, and the likelihood of removing the entire tumor is greater with reduced side effects.

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Consequently, endonasal transphenoidal surgery is often san as a first option, with transphenoidal and other treatments, such as medicinal therapy or stereotactic radiosurgeryused to reduce the remaining adverse effects of the remaining tumor.

These procedures normally relieve the pressure on the surrounding brain regions and lead to a lowering of GH levels. Success depends on the skill and experience of the surgeon.

The success rate also depends on what level of GH is defined as a cure. Complications of surgery may include cerebrospinal fluid leaks, meningitisor damage to the surrounding normal pituitary tissue, requiring lifelong pituitary hormone replacement. Even when surgery is successful and hormone levels return to normal, patients gigantiske be carefully monitored for years for possible recurrence.

More commonly, hormone levels may improve, but not return completely gigabtisme normal. These patients may then require additional treatment, usually with medications. 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 long-term for increasing GH levels.

If surgery does not normalize hormone levels or a relapse occurs, a doctor will usually begin daj drug therapy. The current first choice is generally octreotide akromsgali lanreotide. Bromocriptine or cabergoline are much cheaper and easier to administer, however. 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.

Upon successful treatment, symptoms and complications generally improve substantially or disappear, including headaches, visual disturbances, excess sweating, and diabetes.

From Wikipedia, the free encyclopedia. Acromegaly Givantisme features of a person with acromegaly. The cheekbones are pronounced, the forehead bulgesthe jaw is enlarged, and facial lines are prominent. Compared with the hand of an unaffected person leftthe hand of a person with acromegaly right is enlarged, with fingers that are widened, thickened and stubby, and with thicker soft tissue. Mandibular overgrowth leads to prognathismmaxillary widening, teeth spacing and malocclusion.

Brow ridge and forehead protrusion remaining after tumor removal and tissue swelling eliminated. Lower jaw showing the classic spacing of teeth due to acromegaly. Archived from the original on 27 August Retrieved 20 August Fundamentals of Hand arkomegali Wrist Imaging.