Acromegaly Symptoms
Acromegaly
Acromegaly is also known as Marie’s disease. Acromegaly is caused by primary adenoma which affect the anterior lobe.
Acromegaly occurs due to hypersecretion of growth hormone (GH) or increased amounts of insulin-like growth factor I (IGF-I). It is a chronic debilitating disease with an insidious onset.
The symptoms and signs of acromegaly are coarse features resulting from growth of soft
tissue, coarse, oily skin,hands and feet that are spadelike, fleshy, and moist , prognathism, which can give an underbite,Carpal tunnel syndrome, excessive sweating, arthralgias and severe osteoarthritis, history of increased hat, glove, and/or shoe size, hypertension, skin tag, muscle weakness and decreased exercise capacity, headache, often severe, diabetes mellitus and visual field defects.
There are few investigation tests that can be done. First screening test: measure serum IGF-I level. Direct measurement of the GH level is not as useful because it is secreted in a
pulsatile fashion and a random level may be falsely normal. Upper limits of a normal IGF-I level, depending on the assay: .380 ng/ml or 2.5 U/ml.
Failure to suppress serum GH to less than 2 ng/ml after 100 g oral glucose is considered
conclusive.Patients may show suppression of GH (paradoxic response). Patients will not suppress GH to 2 ng/ml or less (typical response in patients with acromegaly).GHRH level .300 ng/ml is indicative of an ectopic source of GHRH such as from carcinoid tumors of the pancreas or lung.
Laboratory investigation may reveal elevation of urine calcium, elevation of IGF-1 level and
elevated serum phosphate.Imaging studies of choice: MRI of the pituitary and hypothalamus. CT of the pituitary and hypothalamus sometimes used initially.
Acromegaly can be treated in few ways.Treatment of choice: transsphenoidal microsurgical adenomectomy. Surgical failure rate: approximately 13.3% for
microadenomas (tumors ,10 mm) and 11.1% for macroadenomas (tumors .10 mm
confined to the sella) Preoperative IGF-I level: indicator of surgical outcome with higher levels associated withsurgical failure.
Radiotherapy is usually reserved for tumor recurrence or persistence after surgery in patients with resistance to or intolerance of medical treatment
Major complication: hypopituitarism, which may occur in up to 50% of patients; this complication is more likely in patients who had surgery irradiation.
Medical therapy is indicated when patients have not responded to surgical therapy, when surgery is contraindicated, and in patients waiting for the effects of radiotherapy to begin.
Somastatin receptor ligands: octreotide, lanreotide is a useful medical treatment for acromegaly. Important in the preoperative shrinkage of pituitary tumors and softening of adenomatous tissue. Dopamine receptor agonists: Bromocriptine, cabergoline: can be used in addition to somastatin receptor ligands.
Pegvisomant is a growth hormone receptor antagonist that has shown promising results in the treatment of acromegaly. It is generally used in patients with resistance to or intolerance of somastatin analogues. It should be used in patients who do not have central compressive symptoms and those with resistant diabetes.
Combination of bromocriptine and octreotide may be synergistic, allowing a lower combination dosage than either alone.
Acromegaly is also known as Marie’s disease. Acromegaly is caused by primary adenoma which affect the anterior lobe.
Acromegaly occurs due to hypersecretion of growth hormone (GH) or increased amounts of insulin-like growth factor I (IGF-I). It is a chronic debilitating disease with an insidious onset.
The symptoms and signs of acromegaly are coarse features resulting from growth of soft
tissue, coarse, oily skin,hands and feet that are spadelike, fleshy, and moist , prognathism, which can give an underbite,Carpal tunnel syndrome, excessive sweating, arthralgias and severe osteoarthritis, history of increased hat, glove, and/or shoe size, hypertension, skin tag, muscle weakness and decreased exercise capacity, headache, often severe, diabetes mellitus and visual field defects.
There are few investigation tests that can be done. First screening test: measure serum IGF-I level. Direct measurement of the GH level is not as useful because it is secreted in a
pulsatile fashion and a random level may be falsely normal. Upper limits of a normal IGF-I level, depending on the assay: .380 ng/ml or 2.5 U/ml.
Failure to suppress serum GH to less than 2 ng/ml after 100 g oral glucose is considered
conclusive.Patients may show suppression of GH (paradoxic response). Patients will not suppress GH to 2 ng/ml or less (typical response in patients with acromegaly).GHRH level .300 ng/ml is indicative of an ectopic source of GHRH such as from carcinoid tumors of the pancreas or lung.
Laboratory investigation may reveal elevation of urine calcium, elevation of IGF-1 level and
elevated serum phosphate.Imaging studies of choice: MRI of the pituitary and hypothalamus. CT of the pituitary and hypothalamus sometimes used initially.
Acromegaly can be treated in few ways.Treatment of choice: transsphenoidal microsurgical adenomectomy. Surgical failure rate: approximately 13.3% for
microadenomas (tumors ,10 mm) and 11.1% for macroadenomas (tumors .10 mm
confined to the sella) Preoperative IGF-I level: indicator of surgical outcome with higher levels associated withsurgical failure.
Radiotherapy is usually reserved for tumor recurrence or persistence after surgery in patients with resistance to or intolerance of medical treatment
Major complication: hypopituitarism, which may occur in up to 50% of patients; this complication is more likely in patients who had surgery irradiation.
Medical therapy is indicated when patients have not responded to surgical therapy, when surgery is contraindicated, and in patients waiting for the effects of radiotherapy to begin.
Somastatin receptor ligands: octreotide, lanreotide is a useful medical treatment for acromegaly. Important in the preoperative shrinkage of pituitary tumors and softening of adenomatous tissue. Dopamine receptor agonists: Bromocriptine, cabergoline: can be used in addition to somastatin receptor ligands.
Pegvisomant is a growth hormone receptor antagonist that has shown promising results in the treatment of acromegaly. It is generally used in patients with resistance to or intolerance of somastatin analogues. It should be used in patients who do not have central compressive symptoms and those with resistant diabetes.
Combination of bromocriptine and octreotide may be synergistic, allowing a lower combination dosage than either alone.