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Active acromegaly must be distinguished from familial coarse features, large hands and feet, and isolated prognathism and from inactive ( burned-out ) acromegaly in which there has been a spontaneous remission due to infarction of the pituitary adenoma GH-induced gigantism must be differentiated from familial tall stature and from aromatase deficiency (See Osteoporosis) Misleadingly high serum GH levels can be caused by exercise or eating just prior to the test; acute illness or agitation; hepatic or renal failure; malnourishment; diabetes mellitus; or concurrent treatment with estrogens, -blockers, or clonidine During normal adolescence, serum IGF-I is usually elevated and GH may fail to be suppressed
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Complications include hypopituitarism, hypertension, glucose intolerance or frank diabetes mellitus, cardiac enlargement, and cardiac failure Carpal tunnel syndrome may cause thumb weakness and thenar atrophy Arthritis of hips, knees, and spine can be troublesome Cord compression may be seen Visual field defects may be severe and progressive Acute loss of vision or cranial nerve palsy may occur if the tumor undergoes spontaneous hemorrhage and necrosis (pituitary apoplexy) Colon polyps are more likely to develop in patients with acromegaly
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Endoscopic transnasal, transsphenoidal pituitary microsurgery removes the adenoma while preserving anterior pituitary function in most patients Surgical remission is achieved in about 70% of patients followed over 3 years GH levels fall immediately; diaphoresis and carpal tunnel syndrome often improve within a day after surgery Transsphenoidal surgery is usually well tolerated, but complications occur in about 10% of patients, including infection, cerebrospinal fluid leak, and hypopituitarism Transsphenoidal pituitary surgery may be difficult in patients with McCune Albright syndrome because of fibrous dysplasia of the skull base Hyponatremia can occur 4 13 days postoperatively and is manifested by nausea, vomiting, headache, malaise, or seizure It is prudent to monitor serum sodium levels postoperatively Dietary salt supplements for 2 weeks postoperatively may prevent this complication Corticosteroids are administered perioperatively and tapered to replacement doses over 1 week; hydrocortisone is discontinued and cosyntropin stimulation test is performed about 6 weeks after surgery At that time, a serum T4 can be checked (to screen for secondary hypothyroidism) and the patient is screened for secondary hypogonadism (see above)
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tension In acromegalic diabetics, hypoglycemic drugs are reduced to avoid hypoglycemia during pegvisomant therapy The effectiveness of pegvisomant is reduced by coadministration of opioids or propoxyphene Pegvisomant is extraordinarily expensive Acromegalic patients who have not had a complete remission with transsphenoidal surgery or medical therapy may be treated with stereotactic radiosurgery administered by gamma knife, heavy particle radiation, or adapted linear accelerator Some medical centers are using pituitary gamma knife radiosurgery as the initial treatment with reported success rates of 20 90% Radiosurgery precisely radiates the pituitary tumor in a single session and reduces radiation to the normal brain However, it cannot be used for pituitary tumors with suprasellar extension due to the risk of damaging the optic chiasm Radiosurgery can be used for pituitary tumors invading the cavernous sinus, since cranial nerves III, IV, V, and VI are less susceptible to radiation damage Radiosurgery can also be used for patients who have not responded to conventional radiation therapy
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Castinetti F et al Outcome of gamma knife radiosurgery in 82 patients with acromegaly: correlation with initial hypersecretion J Clin Endocrinol Metab 2005 Aug;90(8):4483 8 [PMID: 15899958] Feenstra J et al Combined therapy with somatostatin analogues and weekly pegvisomant in active acromegaly Lancet 2005 May 7 13;365(9471):1644 6 [PMID: 15885297] Galland F et al McCune-Albright syndrome and acromegaly: Effects of hypothalamopituitary radiotherapy and/or pegvisomant in somatostatin analog-resistant patients J Clin Endocrinol Metab 2006 Dec;91(12):4957 61 [PMID: 16984995] Melmed S Acromegaly N Engl J Med 2006 Dec 14;355(24): 2558 73 [PMID: 17167139]
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