Acromegaly: Difference between revisions
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*Growth hormone receptor agonists- blocks the GH signal for IGF-1 production. More effective for patients with higher levels of IGF-1, and demonstrated a more favorable influence on glycemic control.<ref name="Fleseriu">4.</ref> Adverse events often associated with growth hormone receptor agonists are compromised liver function and reactions at the injection site.<ref name="Fleseriu">4.</ref> | *Growth hormone receptor agonists- blocks the GH signal for IGF-1 production. More effective for patients with higher levels of IGF-1, and demonstrated a more favorable influence on glycemic control.<ref name="Fleseriu">4.</ref> Adverse events often associated with growth hormone receptor agonists are compromised liver function and reactions at the injection site.<ref name="Fleseriu">4.</ref> | ||
<ref name="Del Porto">7.Del Porto LA, Liubinas SV, Kaye AH. Treatment of persistent acromegaly. J Clin Neurosci 2011; 18:181-190.</ref> | |||
== Diagnostic Tests/Lab Tests/Lab Values == | == Diagnostic Tests/Lab Tests/Lab Values == | ||
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Magnetic Resonance Imaging (MRI)- the preferred imaging method when diagnosing acromegaly.<ref name="Cordero">2.</ref> An MRI of the pituitary gland is taken in order to look for any abnormal growth.<ref name="Merck">3.</ref> It can help determine the tumor size, as well as compression of surrounding structures.<ref name="Cordero">2.</ref> | Magnetic Resonance Imaging (MRI)- the preferred imaging method when diagnosing acromegaly.<ref name="Cordero">2.</ref> An MRI of the pituitary gland is taken in order to look for any abnormal growth.<ref name="Merck">3.</ref> It can help determine the tumor size, as well as compression of surrounding structures.<ref name="Cordero">2.</ref> | ||
Blood tests- blood may be used to measure serum levels of IGF-1 in addition to GH.<ref name="Cordero">2.</ref><ref name="Fleseriu">4.</ref><ref name="Shlomo">6.</ref> High levels of IGF-1 have been used as a marker for acromegaly; IGF-1 is correlated with GH levels as well as the clinical manifestations of acromegaly.<ref name="Cordero">2.</ref><ref name="Shlomo">6.</ref> IGF-1 levels also tend to remain stable throughout the day, making IGF-1 testing a fairly reliable diagnostic tool.<ref name="Cordero">2.</ref><ref name="Shlomo">4.</ref> Generally, a diagnosis of acromegaly can be excluded if IGF-1 levels are within the normal range. | Blood tests- blood may be used to measure serum levels of IGF-1 in addition to GH.<ref name="Cordero">2.</ref><ref name="Fleseriu">4.</ref><ref name="Shlomo">6.</ref> High levels of IGF-1 have been used as a marker for acromegaly; IGF-1 is correlated with GH levels as well as the clinical manifestations of acromegaly.<ref name="Cordero">2.</ref><ref name="Shlomo">6.</ref> IGF-1 levels also tend to remain stable throughout the day, making IGF-1 testing a fairly reliable diagnostic tool.<ref name="Cordero">2.</ref><ref name="Shlomo">4.</ref> Generally, a diagnosis of acromegaly can be excluded if IGF-1 levels are within the normal range.<ref name="Del Porto">7.</ref> | ||
== Etiology/Causes == | == Etiology/Causes == |
Revision as of 22:45, 27 March 2011
Original Editors - Alex Kent from Bellarmine University's Pathophysiology of Complex Patient Problems project.
Lead Editors - Your name will be added here if you are a lead editor on this page. Read more.
Definition/Description
[edit | edit source]
Acromegaly is a rare systemic disease which affects the entire body.[1] It is characterized by a hypersecretion of growth hormone (GH) which the body is unable to regulate.[2] GH facilitates growth of muscles, internal organs, and bones, as well as stimulating secretion of its target hormone insulin-like growth factor 1 (IGF-1).[1] ,[3] The extremely high levels of GH and IGF-1 which typify acromegaly cause tissue enlargement and metabolic changes that result in visible deformity as well as an increase in mortality.[2][4] The disease often begins between the ages of 30 and 50, and has an insidious onset and slow progression, often delaying diagnosis until later stages of the disease.[2][3]
Prevalence[edit | edit source]
- The prevalence of acromegaly is approximately 40-70 cases per million persons.[2][4]
- However, new research suggests that the prevalence may be as high as 77 cases per million persons.[4]
- The annual incidence of acromegaly is approximately is 3-4 new cases per million persons.[4]
Characteristics/Clinical Presentation[edit | edit source]
Acromegaly affects many of the body's systems, and various signs and symptoms develop over a period of several years.[5] They range from subtle changes to notable disfigurement.[2]
Clinical Presentation[2][3][5][6]:
- Hand and foot enlargement
- Hyperhydrosis- increased perspiration
- Increased skin thickness
- Darkening and thickening of body hair
- Frontal skull bossing- an abnormally heavy brow and prominent forehead
- Widening of the maxilla accompanied by separation of the teeth
- Jaw malocclusion and overbite
- Soft tissue enlargement
- Skeletal overgrowth and thickening causing many areas to appear swollen
- Deep and husky voice due to thickening of cartilage in the larynx
- Ventilatory dysfunction
- Weight gain
- Joint pain
- Sleep apnea
- Acne
- Vision problems
Associated Co-morbidities[edit | edit source]
IGF-1, the target molecule of GH, enables many of the growth-promoting actions of GH; GH itself is also a regulator of mineral, lipid, and carbohydrate metabolism.[6] Therefore the elevated levels of GH and IGF-1 which are characteristic of acromegaly excessive soft tissue growth, swelling of internal organs, and musculoskeletal, neurological, and metabolic comorbidities.[6]
- Hypertension
- Arrhythmias
- Valvulopathy
- Cardiomyopathy
- Hypertrophy (biventricular or asymmetric septal)
- Congestive heart failure
- Obstructive sleep apnea
- Macroglossia
- Upper airway obstruction
- Ventilatory dysfunction
- Upper airway obstruction
- Insulin resistance
- Impaired glucose metabolism
- Diabetes mellitus
- Organ enlargement
- Colon polyps
- Fluid retention
- Renal failure
Musculoskeletal:[2]
- Arthropathy/osteoarthritis
- Carpal tunnel syndrome
- Osteopenia
Medications[edit | edit source]
- Somatostatin analogs- somatostatin inhibits endocrine cells, including GH-secreting cells of the pituitary gland. Somatostatin analogs (SSAs) mimic the GH-suppressing effects of the body's own somatostatin.[4] SSAs are one of the most common medications prescribed for acromegaly. There are currently three SSAs approved in the US: short-acting octreotride, octreotride LAR, and Somatuline Depot.[4] Most common adverse events are glucose intolerance, and gallbladder and sludge stones.[4]
- Dopamine agonists- leads to GH suppression in a portion of acromegaly patients. Interestingly, this medication stimulates GH release in healthy patients.[4] Advantages of this medication are relatively low cost, oral administration, and no hypopituitarism associated with medication. However, the medication is only effective at lowering GH and IGF-1 to safe levels in approximately 10% of patients, and potentially causes cardiac valvular damage.[4]
- Growth hormone receptor agonists- blocks the GH signal for IGF-1 production. More effective for patients with higher levels of IGF-1, and demonstrated a more favorable influence on glycemic control.[4] Adverse events often associated with growth hormone receptor agonists are compromised liver function and reactions at the injection site.[4]
Diagnostic Tests/Lab Tests/Lab Values[edit | edit source]
Diagnostic Tests:
Oral Glucose Tolerance Test (OGTT)- glucose has a neuroendocrine suppressive signal that lowers GH.[6] In this test, 75 g of glucose is administered with GH measurements at different points over a period of 120 minutes.[2] The failure to suppress GH secretion to less than 1 microgram/liter is currently the standard for diagnosis of acromegaly.[6]
Magnetic Resonance Imaging (MRI)- the preferred imaging method when diagnosing acromegaly.[2] An MRI of the pituitary gland is taken in order to look for any abnormal growth.[3] It can help determine the tumor size, as well as compression of surrounding structures.[2]
Blood tests- blood may be used to measure serum levels of IGF-1 in addition to GH.[2][4][6] High levels of IGF-1 have been used as a marker for acromegaly; IGF-1 is correlated with GH levels as well as the clinical manifestations of acromegaly.[2][6] IGF-1 levels also tend to remain stable throughout the day, making IGF-1 testing a fairly reliable diagnostic tool.[2][6] Generally, a diagnosis of acromegaly can be excluded if IGF-1 levels are within the normal range.[7]
Etiology/Causes[edit | edit source]
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Systemic Involvement[edit | edit source]
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Medical Management (current best evidence)[edit | edit source]
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Physical Therapy Management (current best evidence)[edit | edit source]
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Alternative/Holistic Management (current best evidence)[edit | edit source]
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Differential Diagnosis[edit | edit source]
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Case Reports/ Case Studies[edit | edit source]
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Resources
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Recent Related Research (from Pubmed)[edit | edit source]
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References[edit | edit source]
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- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 1. Vance M. Acromegaly: a fascinating pituitary disorder. Neurosurg Focus 2010;29(4):1. Cite error: Invalid
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tag; name "Vance" defined multiple times with different content - ↑ 2.00 2.01 2.02 2.03 2.04 2.05 2.06 2.07 2.08 2.09 2.10 2.11 2.12 2.13 2.14 2.15 2. Cordero RA, Barkan AL. Current diagnosis of acromegaly. Rev Endocr Metab Disord 2008;9:13-19. Cite error: Invalid
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tag; name "Cordero" defined multiple times with different content - ↑ 3.0 3.1 3.2 3.3 3.Merck Manual Home Edition. Acromegaly and gigantism: pituitary gland disorders. http://www.merckmanuals.com/home/print/sec13/ch162e.html (Accessed March 11 2011). Cite error: Invalid
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tag; name "Merck" defined multiple times with different content - ↑ 4.00 4.01 4.02 4.03 4.04 4.05 4.06 4.07 4.08 4.09 4.10 4.11 4.Fleseriu M, Delashaw JB, Cook DM. Acromegaly: a review of current medical therapy and new drugs on the horizon. Neurosurg Focus 2010;29(4):E15. Cite error: Invalid
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tag; name "Fleseriu" defined multiple times with different content - ↑ 5.0 5.1 5.2 5.3 5. Reid TJ, Post KD, Bruce JN, Kanibir MN, Reyes-Vidal CM, Freda PU. Features at diagnosis of 324 patients with acromegaly did not change from 1981 to 2006: acromegaly remains under-recognized and under-diagnosed. Clinical Endocrinology 2010;72:203-208. Cite error: Invalid
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tag; name "Reid" defined multiple times with different content - ↑ 6.00 6.01 6.02 6.03 6.04 6.05 6.06 6.07 6.08 6.09 6.10 6.11 6.Melmed S. Acromegaly pathogenesis and treatment. J. Clin. Invest 2009;119:3189-3202. Cite error: Invalid
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