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== Definition/Description&nbsp;<br> ==
== Introduction ==
[[File:Acromegaly_classic_woman.gif|alt=|thumb|Acromegaly classic woman]]
Acromegaly is the result of excessive [[Growth Hormone|human growth hormone]] production in skeletally mature patients, most commonly from a pituitary adenoma. This excess of growth hormone in individuals whose epiphyses have not fused results in gigantism (excessively tall stature).  


Acromegaly is a rare systemic disease which affects the entire body.<ref name="Vance">1. Vance M. Acromegaly: a fascinating pituitary disorder. Neurosurg Focus 2010;29(4):1.</ref> It is characterized by a hypersecretion of growth hormone (GH) which the body is unable to regulate.<ref name="Cordero">2. Cordero RA, Barkan AL. Current diagnosis of acromegaly. Rev Endocr Metab Disord 2008;9:13-19.</ref>&nbsp;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).<ref name="Vance">1.</ref> <sup>,</sup><ref name="Merck">3.Merck Manual Home Edition. Acromegaly and gigantism: pituitary gland disorders. http://www.merckmanuals.com/home/print/sec13/ch162e.html (Accessed March 11 2011).</ref> 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.<ref name="Cordero">2.</ref><ref name="Fleseriu">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.</ref> 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.<ref name="Cordero">2.</ref><ref name="Merck">3.</ref>
== Epidemiology ==
 
Acromegaly is most commonly diagnosed in middle-aged adults and can result in extreme disfigurement, grave complicating conditions, and early death. It has an insidious onset, slow progression and may be difficult to diagnose in the early stages, only being diagnosed when the external features, especially those of the face, become noticeable.<ref name=":0">Radiopedia Acromegaly Available: https://radiopaedia.org/articles/acromegaly (accessed 18.8.2022)</ref>
== Prevalence  ==
*The&nbsp;prevalence of acromegaly is&nbsp;approximately 40-70 cases&nbsp;per million persons. However, new research suggests that the prevalence may be as high as 77 cases per million persons.<ref name="Fleseriu">Fleseriu M, Delashaw JB, Cook DM. [https://scholar.google.com/scholar_url?url=https://thejns.org/focus/view/journals/neurosurg-focus/29/4/2010.7.focus10154.xml&hl=en&sa=T&oi=gsb-ggp&ct=res&cd=0&d=6680632314369309009&ei=PZcEYqOpOIz2yATZiLfgDQ&scisig=AAGBfm0DhTHX0gLuxRivB0GjNm3hXRVaXw Acromegaly: a review of current medical therapy and new drugs on the horizon.] Neurosurg Focus 2010;29(4):E15.</ref><ref name="Cordero">Cordero RA, Barkan AL. [https://scholar.google.com/scholar_url?url=https://link.springer.com/article/10.1007/s11154-007-9060-2&hl=en&sa=T&oi=gsb&ct=res&cd=0&d=601527571386589898&ei=yZYEYu7NHoTyyASduqq4Dg&scisig=AAGBfm2r5CUlonGiSfRNPjXv70AydLm4vg Current diagnosis of acromegaly]. Rev Endocr Metab Disord 2008;9:13-19.</ref>
 
*The annual incidence of acromegaly is approximately is 3-4 new cases per million persons.<ref name="Fleseriu" />
*The&nbsp;prevalence of acromegaly is&nbsp;approximately 40-70 cases&nbsp;per million persons.<ref name="Cordero">2.</ref><ref name="Fleseriu">4.</ref>&nbsp;
*However, new research suggests that the prevalence may be as high as 77 cases per million persons.<ref name="Fleseriu">4.</ref>
*The annual incidence of acromegaly is approximately is 3-4 new cases per million persons.<ref name="Fleseriu">4.</ref>


== Characteristics/Clinical Presentation  ==
== Characteristics/Clinical Presentation  ==
 
[[File:Acromegaly_comorbidities.jpg|right|alt=|397x397px]]Acromegaly affects many of the body's systems, and various signs and symptoms develop over a period of several years.<ref name="Reid">Reid TJ, Post KD, Bruce JN, Kanibir MN, Reyes-Vidal CM, Freda PU. [https://scholar.google.com/scholar_url?url=https://onlinelibrary.wiley.com/doi/abs/10.1111/j.1365-2265.2009.03626.x&hl=en&sa=T&oi=gsb&ct=res&cd=0&d=8372557375255968456&ei=ZpcEYqGoNYK4ygTc_rBI&scisig=AAGBfm2_QlVQSREBGcpr3u1M_jorHuOz2A 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.</ref>&nbsp;They range from subtle changes to notable disfigurement.<ref name="Cordero" /> Common features are:<ref name="Cordero" /><ref name="Merck">Merck Manual Home Edition. [http://www.merckmanuals.com/home/print/sec13/ch162e.html Acromegaly and gigantism: pituitary gland disorders.] (last accessed 10/2/2022).</ref><ref name="Reid" /><ref name="Shlomo">Melmed S. [https://scholar.google.com/scholar_url?url=https://www.jci.org/articles/view/39375&hl=en&sa=T&oi=gsb-ggp&ct=res&cd=0&d=2814766874452575472&ei=jZcEYsO4CYTyyASduqq4Dg&scisig=AAGBfm1nsnx-c-2AkNiIjHF_9iW0gEJwHQ Acromegaly pathogenesis and treatment.] J. Clin. Invest 2009;119:3189-3202.</ref>:
Acromegaly affects many of the body's systems, and various signs and symptoms develop over a period of several years.<ref name="Reid">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.</ref>&nbsp;They range from subtle changes to notable disfigurement.<ref name="Cordero">2.</ref>
*Hyperhydrosis (increased perspiration) and acne.
 
*Increased skin thickness and darkening and thickening of body hair
Clinical Presentation<ref name="Cordero">2.</ref><ref name="Merck">3.</ref><ref name="Reid">5.</ref><ref name="Shlomo">6.Melmed S. Acromegaly pathogenesis and treatment. J. Clin. Invest 2009;119:3189-3202.</ref>:
*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, joint pain
*Hand and foot enlargement
*Skeletal overgrowth and thickening causing many areas to appear swollen, 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
*Deep and husky voice due to thickening of cartilage in the larynx
*Ventilatory dysfunction
*Ventilatory dysfunction
*Weight gain
*Weight gain
*Joint pain
*Sleep apnea
*Sleep apnea
*Acne
*Vision problems
*Vision problems


&nbsp;<br>
== Markers ==
 
Typically cases show elevated levels of growth hormone and [[Insulin Like Growth Factor-1 (IGF-1)|IGF-1]] (insulin-like growth factor 1)
== Associated Co-morbidities  ==
 
IGF-1, the target&nbsp;molecule of GH,&nbsp;enables many of the&nbsp;growth-promoting actions of GH; GH itself is also&nbsp;a regulator of mineral, lipid, and carbohydrate metabolism.<ref name="Shlomo">6.</ref> Therefore the elevated levels of&nbsp;GH and IGF-1&nbsp;which are characteristic of acromegaly excessive soft&nbsp;tissue growth, swelling of internal organs,&nbsp;and musculoskeletal, neurological, and metabolic comorbidities.<ref name="Shlomo">6.</ref>&nbsp;<br>
 
Cardiovascular:<ref name="Vance">1.</ref><ref name="Cordero">2.</ref><ref name="Reid">5.</ref><ref name="Shlomo">6.</ref>
 
*Hypertension
*Arrhythmias
*Valvulopathy
*Cardiomyopathy
*Hypertrophy (biventricular or asymmetric septal)
*Congestive heart failure
 
<br>
 
Pulmonary:<ref name="Vance">1.</ref><ref name="Cordero">2.</ref><ref name="Reid">5.</ref><ref name="Shlomo">6.</ref>
 
*Obstructive sleep apnea
*Macroglossia
*Upper airway obstruction
*Ventilatory dysfunction
*Upper airway obstruction
 
<br>
 
Metabolic:<ref name="Vance">1.</ref><ref name="Cordero">2.</ref><ref name="Shlomo">6.</ref>
 
*Insulin resistance
*Impaired glucose metabolism
*Diabetes mellitus
 
<br>
 
Visceral:<ref name="Vance">1.</ref><ref name="Shlomo">6.</ref>
 
*Organ enlargement
*Colon polyps
*Fluid retention
*Renal failure
 
<br>
 
Musculoskeletal:<ref name="Cordero">2.</ref>
 
*Arthropathy/osteoarthritis
*Carpal tunnel syndrome
*Osteopenia
 
== Medications  ==
 
*Somatostatin analogs- somatostatin inhibits endocrine cells, including GH-secreting&nbsp;cells of the pituitary gland.&nbsp; Somatostatin analogs (SSAs) mimic the GH-suppressing effects of the body's own somatostatin.<ref name="Fleseriu">4.</ref>&nbsp; SSAs are one&nbsp;of the most common medications prescribed for acromegaly.&nbsp; There&nbsp;are currently three SSAs approved in the US: short-acting octreotride, octreotride LAR, and Somatuline Depot.<ref name="Fleseriu">2.</ref>&nbsp;Most common adverse events are glucose intolerance, and gallbladder and sludge stones.<ref name="Fleseriu">4.</ref>
*Dopamine agonists- leads to GH suppression in a portion of acromegaly patients.&nbsp; Interestingly, this medication stimulates GH release in healthy patients.<ref name="Fleseriu">4.</ref>&nbsp; Advantages of this medication are relatively low cost, oral administration, and no hypopituitarism associated with medication.&nbsp; However,&nbsp; the medication is&nbsp;only effective at lowering GH and IGF-1 to safe levels in approximately 10% of patients, and potentially causes cardiac valvular damage.<ref name="Fleseriu">4.</ref>
*Growth hormone receptor agonists- blocks the GH signal for IGF-1 production.&nbsp; More effective for patients with higher levels of IGF-1, and demonstrated a more favorable influence on glycemic control.<ref name="Fleseriu">4.</ref>&nbsp;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:<br>
 
Oral Glucose Tolerance Test (OGTT)- glucose has a neuroendocrine suppressive signal that lowers GH.<ref name="Shlomo">6.</ref>&nbsp; In this test, 75 g of glucose is administered with GH measurements&nbsp;at different points over a period of 120 minutes.<ref name="Cordero">2.</ref>&nbsp;The failure to suppress GH secretion to less than 1 microgram/liter is currently the standard for diagnosis of acromegaly.<ref name="Shlomo">6.</ref>
 
Magnetic Resonance Imaging (MRI)- the preferred imaging method when diagnosing acromegaly.<ref name="Cordero">2.</ref>&nbsp; An MRI of the pituitary gland is taken in order to look for any abnormal growth.<ref name="Merck">3.</ref>&nbsp;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>&nbsp;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>&nbsp;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>


Radiographs- an x-ray of the skull can show bone thickening as well as enlargement of the nasal sinuses.<ref name="Merck">3.</ref>&nbsp;Thickening of the carpal phalanges can be seen in x-rays of the hands.<ref name="Merck">3.</ref>
== Pathology ==


Computed tomography (CT)- this imaging method can be used to identify abnormal growths in the pituitary gland.<ref name="Merck">3.</ref>&nbsp;  
* Approximately 95% of cases are the result of a pituitary adenoma. The remaining
* 5% of cases are the result of other tumors of the pancreas, lungs, or adrenal glands that release growth hormone.  
* A few number of cases result from the excessive use of exogenous growth hormone in athletes. &nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;


Lab Values:
== Treatment ==
The treatment of choice is resection of the secreting adenoma.


Growth Hormone (GH)- in individuals without acromegaly, GH values are typically 0.1-0.2 micrograms/liter.&nbsp; There are, however, 6-10 bursts of GH secretion where levels of the hormone are 5-30 micrograms/liter.<ref name="Cordero">2.</ref>&nbsp;This may overlap with the values seen in patients with acromegaly.<ref name="Cordero">2.</ref>&nbsp;Growth hormone concentrations of less than 2.5 ng/ml are associated with mortality rates similar to the normal population.<ref name="Fleseriu">4.</ref>&nbsp;Thus normalization of GH levels is currently defined as below 2.5 ng/ml.<ref name="Del Porto">7.</ref>
* Radiation therapy is also used in medical circumstances where other therapies have not been able to control tumor size, growth and production of excess growth hormone.  
* The severity of symptoms and comorbidities for acromegaly patients is relates directly to the level of elevated hormone as well as how long the patient was exposed to a high level of HGH.  
* Mortality rates are returned  to those of the general population if appropriate diagnosis and treatment are achieved to normalize serum growth hormone and IGF-1 levels.<ref name=":0" />


== Etiology/Causes  ==
== Physical Therapy Management  ==


*The majority (99%) of acromegaly cases are caused by the proliferation of somatotroph cells&nbsp;in the pituitary which leads to a pituitary adenoma.<ref name="Cordero">2.</ref><ref name="Shlomo">6.</ref>&nbsp;Somatotroph cells&nbsp;make up more than half of the hormone-secreting cells in the ptuitary.<ref name="Shlomo">6.</ref><br>
Acromegaly is a disease which affects several systems in the body.<ref name="Vance">Vance M. [https://scholar.google.com/scholar_url?url=https://thejns.org/focus/view/journals/neurosurg-focus/29/4/2010.10.focus.intro.xml&hl=en&sa=T&oi=gsb&ct=res&cd=0&d=696610041890456563&ei=l5YEYvzrKreN6rQP8ZCWsAI&scisig=AAGBfm0Etwqk3I7nqfm_WpoAhTCowHAKqQ Acromegaly]: a fascinating pituitary disorder. Neurosurg Focus 2010;29(4):1.</ref><ref name="Cordero" /><ref name="Shlomo" />&nbsp;Therapists may be called on to provide a program addressing the musculoskeletal and neurological manifestations of the disease. <ref name="Goodman">Goodman CC, Fuller KS. Pathology: implications for the physical therapist. 3rd ed. St.Louis: Saunders-Elsevier, 2009.</ref>  
*The pituitary tumor causes an increase in circulating levels of GH.<ref name="Cordero">2.</ref>&nbsp; GH mediates anabolic reactions by binding to growth hormone receptors, creating docking sites for cell signaling proteins, and initiating the&nbsp;synthesis of target genes like IGF-1.<ref name="Del Porto">7.</ref>&nbsp; This in turn increases the production of insulin-like growth factor 1 (IGF-1)<ref name="Cordero">2.</ref><ref name="Fleseriu">4.</ref><ref name="Del Porto">7.</ref>.&nbsp; IGF-1 causes metabolic changes and&nbsp;somatic growth,&nbsp;stimulating&nbsp;proliferation of cartilage, skeletal muscle and bone which ultimately lead to tissue enlargement.<ref name="Cordero">2.</ref><ref name="Del Porto">7.</ref>
*Enlargement of tissues and metabolic abnormalities cause the two to three-fold increase in mortality seen in acromegaly.<ref name="Vance">1.</ref><ref name="Cordero">2.</ref><ref name="Fleseriu">4.</ref><ref name="Shlomo">6.</ref><ref name="Del Porto">7.</ref>


== Systemic Involvement  ==
Preferred Practice Patterns:<ref name="Goodman" />


Cardiovascular&nbsp;Involvement
*4D: Impaired joint mobility, motor function, muscle performance, and range of motion associated with connective tissue dysfunction.<ref name="Goodman" />
*4E: Impaired joint mobility, motor function, muscle performance, and range of motion associated with localized inflammation.<ref name="Goodman" />
*4F: Impaired joint mobility, motor function, muscle performance, range of motion, and reflex integrity associated with spinal disorders.<ref name="Goodman" />
*5F: Impaired peripheral nerve integrity and muscle performance&nbsp;associated with peripheral nerve injury.<ref name="Goodman" />


*Acromegalic cordiomyopathy- common features include biventricular hypertrophy, thickening of cardiac walls, diastolic dysfunction, limited systolic function, and heart failure with signs of dilative cardiomyopathy.<ref name="Annamaria">8.Colao A, Ferone D, Marzullo P, Lombardi G. Systemic complications of acromegaly: epidemiology, pathogenesis, and management. Endocrine Reviews. 2004; 25(1):102-152</ref>
=== Screening ===
*Mitral and aortic valve dysfunction.<ref name="Annamaria">8.</ref>
*Individuals with acromegaly should be screened for changes in joint mobility, decreased exercise tolerance, and weakness.<ref name="Goodman" />  
*Arrhythmia-&nbsp;linked to a combination of factors, including increased left ventricle wall thickness as well as late action potentials in the heart.<ref name="Annamaria">8.</ref><ref name="Cordero">2.</ref>
*If an acromegalic client presents to physical&nbsp;therapy&nbsp;with unusual muscle weakness, they should be referred to a physician for a complete workup to look for neuropathies and inflammatory myopathies.&nbsp; This will help identify underlying causes which can be treated.<ref name="Goodman" />
*Hypertension- due in part to increased plasma volume.<ref name="Cordero">2.</ref><ref name="Annamaria">8.</ref>
*Atherosclerosis and endothelial dysfunction<ref name="Annamaria">8.</ref>


=== Orthopedic Intervention ===
*Arthritis of&nbsp;several joints is often&nbsp;present in patients with acromegaly.<ref name="Goodman" />&nbsp;In addition, carpal tunnel syndrome is seen in approximately 50% of patients with acromegaly, and there is a similar incidence of thoracic and lumbar pain in acromegalic patients.<ref name="Goodman" />
*X-ray studies have demonstrated large osteophytes in the anterior longitudinal ligament and increased intervertebral space.<ref name="Goodman" />
*A physical therapy program would work to promote muscle strength, mobility of the joints, and independence with functional activities.<ref name="Goodman" />&nbsp; Adaptive equipment for assistance with activities of daily living may be considered depending on the severity of the disease.<ref name="Goodman" />


 
=== Postoperative Intervention ===
 
*Exercise and ambulation are encouraged within the first 24 hours after removal of the pituitary tumor.<ref name="Goodman" />  
 
*Breathing exercises are indicated.&nbsp; However, coughing, sneezing, and blowing the nose are contraindicated.<ref name="Goodman" />  
Metabolic Involvement
*Monitor vital signs closely. Any change in vision, dropping pulse rate, or rising blood pressure could indicate an increase in intracranial pressure and should be reported immediately.<ref name="Goodman" />  
 
*The physical therapist should consult with the nursing staff to determine if blood glucose monitoring is needed during exercise.&nbsp; GH levels may drop rapidly after removal of the tumor and result in hypoglycemia.<ref name="Goodman" />
*Impaired glucose tolerance and diabetes mellitus- growth hormone counteracts the effects of insulin on glucose metabolism.<ref name="Annamaria">8.</ref>&nbsp;GH also regulates the response of tissues to insulin; as a result excessive GH may be a causative factor in insulin resistance.<ref name="Annamaria">8.</ref>
*Impaired lipid metabolism- acromegaly is correlated with lipid metabolism abnormalities.<ref name="Annamaria">8.</ref>Studies have found serum triglyceride levels to be higher in patients with acromegaly.<ref name="Annamaria">8.</ref>
 
 
 
Pulmonary Involvement
 
*Mortality associated with repiratory disorders increased in patients with acromegaly.<ref name="Annamaria">8.</ref>
*Increased incidence of sleep apnea.<ref name="Cordero">2.</ref><ref name="Annamaria">8.</ref>
*Barrel chest developed due to changes in morphology of vertebra and ribs.<ref name="Annamaria">8.</ref>&nbsp;Ribs become elongated and vertebral bodies become enlarged and elongated, which impair inspiration.<ref name="Annamaria">8.</ref>
 
== Medical Management (current best evidence)  ==
 
The goal of currrent medical management is to reduce the effects of the pituitary tumor by surgical resection, regulate the hypersecretion of GH and IGF-1 with medication, attempt to improve the numerous&nbsp;comorbidities associated with the disease, reduce the mortality risk, and maintain normal function of the pituitary gland.<ref name="Fleseriu">4.</ref>
 
<br>
 
Surgery
 
*Pituitary surgery is often the first choice in acromegaly management&nbsp;because it can reduce hypersecretion of GH and IGF-1 and reduce the effects of the tumor on surrounding brain structures.<ref name="Del Porto">7.</ref>
*The goal of <span id="fck_dom_range_temp_1301858332531_320"></span>surgical debulking is to resect as much of the tumor as possible while still maintaining normal function of the pituitary gland.<ref name="Fleseriu">4.</ref><ref name="Shlomo">6.</ref>
*Resection of adenomas in the pituitary gland is difficult due to the location of the pituitary gland and its proximity to important vascular and neural structures.<ref name="Shlomo">6.</ref>&nbsp;As a result, surgery is most successful with an experienced surgeon; numerous studies have demonstrated that the outcome of surgical treatment of acromegaly&nbsp;is correlated with the level of experience of the surgeon.<ref name="Del Porto">7.</ref>
 
Drug Therapy<ref name="Fleseriu">4.</ref>
 
*Somatostatin Analogs
*Dopamine Agonists
*Growth hormone receptor agonists
*The specific actions of these medications are described in the Medications section.
 
Radiation Therapy
 
*Involves the use of supervoltage irradiation.<ref name="Merck">3.</ref>
*Less traumatic than traditional surgery.<ref name="Merck">3.</ref>
*The radiation therapy is delivered in weekly doses spread over a period of six weeks.<ref name="Shlomo">6.</ref>
*Approximately 50% of patients with acromegaly experience biochemical remission after radiation therapy.<ref name="Shlomo">6.</ref>
*The radiation treatment may take years to have its full effect.<ref name="Merck">3.</ref>
 
== Physical Therapy Management (current best evidence)  ==
 
Acromegaly is a disease which affects several systems in the body.<ref name="Vance">1.</ref><ref name="Cordero">2.</ref><ref name="Shlomo">6.</ref>&nbsp;Therapists may be called on to provide a program addressing the musculoskeletal and neurological manifestations of the disease. <ref name="Goodman">9.Goodman CC, Fuller KS. Pathology: implications for the physical therapist. 3rd ed. St.Louis: Saunders-Elsevier, 2009.</ref>
 
Preferred Practice Patterns:<ref name="Goodman">9.</ref>
 
*4D: Impaired joint mobility, motor function, muscle performance, and range of motion associated with connective tissue dysfunction.<ref name="Goodman">9.</ref>
*4E: Impaired joint mobility, motor function, muscle performance, and range of motion associated with localized inflammation.<ref name="Goodman">9.</ref>
*4F: Impaired joint mobility, motor function, muscle performance, range of motion, and reflex integrity associated with spinal disorders.<ref name="Goodman">9.</ref>
*5F: Impaired peripheral nerve integrity and muscle performance&nbsp;associated with peripheral nerve injury.<ref name="Goodman">9.</ref>
 
<br>
 
Screening
 
*Individuals with acromegaly should be screened for changes in joint mobility, decreased exercise tolerance, and weakness.<ref name="Goodman">9.</ref>
*If an acromegalic client presents to physical&nbsp;therapy&nbsp;with unusual muscle weakness, they should be referred to a physician for a complete workup to look for neuropathies and inflammatory myopathies.&nbsp; This will help identify underlying causes which can be treated.<ref name="Goodman">9.</ref>
 
<br>
 
Orthopedic Intervention
 
*Arthritis of&nbsp;several joints is often&nbsp;present in patients with acromegaly.<ref name="Goodman">9.</ref>&nbsp;In addition, carpal tunnel syndrome is seen in approximately 50% of patients with acromegaly, and there is a similar incidence of thoracic and lumbar pain in acromegalic patients.<ref name="Goodman">9.</ref>
*X-ray studies have demonstrated large osteophytes in the anterior longitudinal ligament and increased intervertebral space.<ref name="Goodman">9.</ref>
*A physical therapy program would work to promote muscle strength, mobility of the joints, and independence with functional activities.<ref name="Goodman">9.</ref>&nbsp; Adaptive equipment for assistance with activities of daily living may be considered depending on the severity of the disease.<ref name="Goodman">9.</ref>
 
 
 
Postoperative Intervention
 
*Exercise and ambulation are encouraged within the first 24 hours after removal of the pituitary tumor.<ref name="Goodman">9.</ref>
*Breathing exercises are indicated.&nbsp; However, coughing, sneezing, and blowing the nose are contraindicated.<ref name="Goodman">9.</ref>
*Monitor vital signs closely. Any change in vision, dropping pulse rate, or rising blood pressure could indicate an increase in intracranial pressure and should be reported immediately.<ref name="Goodman">9.</ref>
*The physical therapist should consult with the nursing staff to determine if blood glucose monitoring is needed during exercise.&nbsp; GH levels may drop rapidly after removal of the tumor and result in hypoglycemia.<ref name="Goodman">9.</ref>
 
== Alternative/Holistic Management (current best evidence)  ==
 
add text here


== Differential Diagnosis  ==
== Differential Diagnosis  ==


Several of the comorbidities of acromegaly overlap common disorders; as a result, the acromegaly may be misdiagnosed.<ref name="Reid">5.</ref> The comorbidities which may lead to improper diagnosis include:
Several of the comorbidities of acromegaly overlap common disorders; as a result, the acromegaly may be misdiagnosed.<ref name="Reid" /> The comorbidities which may lead to improper diagnosis include:  
 
*Arthritis<ref name="Reid">5.</ref>
*Hypertension<ref name="Reid">5.</ref>
*Diabetes mellitus<ref name="Reid">5.</ref><ref name="Shlomo">6.</ref>
*Carpal tunnel syndrome<ref name="Reid">5.</ref>
*Respiratory dysfunction<ref name="Annamaria">8.</ref>
*Cardiac involvement<ref name="Annamaria">8.</ref>
*Failure to suppress growth hormone can also be seen in patients with diabetes, renal failure, hepatic failure, and patients with obesity or those on estrogen replacement.<ref name="Shlomo">6.</ref>&nbsp; This could potentially lead to misdiagnosis.
 
== Case Reports/ Case Studies  ==
 
add links to case studies here (case studies should be added on new pages using the [[Template:Case Study|case study template]])<br>Case Report 1
 
== Resources <br> ==
 
add appropriate resources here
 
== Recent Related Research (from Pubmed)  ==
 
add appropriate resources here
 
== Recent Related Research (from [http://www.ncbi.nlm.nih.gov/pubmed/ Pubmed])
 
&lt;divclass="researchbox"&gt;<rss>http://eutils.ncbi.nlm.nih.gov/entrez/eutils/erss.cgi?rss_guid=1z_pxc-9fhP4GP3q0wWQCYsnlVFM4agzcQ1rGpworXkUZUpgvo |charset=UTF-8|short|max=10</rss>&lt;/div&gt;
 
<br>


== References  ==
*Arthritis<ref name="Reid" />
*Hypertension<ref name="Reid" />
*Diabetes mellitus<ref name="Reid" /><ref name="Shlomo" />
*Carpal tunnel syndrome<ref name="Reid" />
*Respiratory dysfunction<ref name="Annamaria">Colao A, Ferone D, Marzullo P, Lombardi G. [https://scholar.google.com/scholar_url?url=https://academic.oup.com/edrv/article-abstract/25/1/102/2355270&hl=en&sa=T&oi=gsb&ct=res&cd=0&d=9931154446895740729&ei=XpgEYr6gEreN6rQP8ZCWsAI&scisig=AAGBfm2QtVHid0r3dF6a4OM199So8MPuwA Systemic complications of acromegaly: epidemiology, pathogenesis, and management]. Endocrine Reviews. 2004; 25(1):102-152</ref>
*Cardiac involvement<ref name="Annamaria" />
*Failure to suppress growth hormone can also be seen in patients with diabetes, renal failure, hepatic failure, and patients with obesity or those on estrogen replacement.<ref name="Shlomo" />&nbsp; This could potentially lead to misdiagnosis.


see [[Adding References|adding references tutorial]].
== References ==


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[[Category:Bellarmine_Student_Project]]
[[Category:Bellarmine_Student_Project]]

Latest revision as of 09:25, 19 August 2022

Original Editors - Alex Kent from Bellarmine University's Pathophysiology of Complex Patient Problems project.

Top Contributors - Alex Kent, Admin, Gayatri Jadav Upadhyay, Lucinda hampton, WikiSysop, Kim Jackson, Chelsea Mclene, Elaine Lonnemann, Wendy Walker and 127.0.0.1  

Introduction[edit | edit source]

Acromegaly classic woman

Acromegaly is the result of excessive human growth hormone production in skeletally mature patients, most commonly from a pituitary adenoma. This excess of growth hormone in individuals whose epiphyses have not fused results in gigantism (excessively tall stature).  

Epidemiology[edit | edit source]

Acromegaly is most commonly diagnosed in middle-aged adults and can result in extreme disfigurement, grave complicating conditions, and early death. It has an insidious onset, slow progression and may be difficult to diagnose in the early stages, only being diagnosed when the external features, especially those of the face, become noticeable.[1]

  • The prevalence of acromegaly is approximately 40-70 cases per million persons. However, new research suggests that the prevalence may be as high as 77 cases per million persons.[2][3]
  • The annual incidence of acromegaly is approximately is 3-4 new cases per million persons.[2]

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.[4] They range from subtle changes to notable disfigurement.[3] Common features are:[3][5][4][6]:

  • Hyperhydrosis (increased perspiration) and acne.
  • Increased skin thickness and 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, joint pain
  • Skeletal overgrowth and thickening causing many areas to appear swollen, hand and foot enlargement
  • Deep and husky voice due to thickening of cartilage in the larynx
  • Ventilatory dysfunction
  • Weight gain
  • Sleep apnea
  • Vision problems

Markers[edit | edit source]

Typically cases show elevated levels of growth hormone and IGF-1 (insulin-like growth factor 1)

Pathology[edit | edit source]

  • Approximately 95% of cases are the result of a pituitary adenoma. The remaining
  • 5% of cases are the result of other tumors of the pancreas, lungs, or adrenal glands that release growth hormone.
  • A few number of cases result from the excessive use of exogenous growth hormone in athletes.                        

Treatment[edit | edit source]

The treatment of choice is resection of the secreting adenoma.

  • Radiation therapy is also used in medical circumstances where other therapies have not been able to control tumor size, growth and production of excess growth hormone.
  • The severity of symptoms and comorbidities for acromegaly patients is relates directly to the level of elevated hormone as well as how long the patient was exposed to a high level of HGH.
  • Mortality rates are returned to those of the general population if appropriate diagnosis and treatment are achieved to normalize serum growth hormone and IGF-1 levels.[1]

Physical Therapy Management[edit | edit source]

Acromegaly is a disease which affects several systems in the body.[7][3][6] Therapists may be called on to provide a program addressing the musculoskeletal and neurological manifestations of the disease. [8]

Preferred Practice Patterns:[8]

  • 4D: Impaired joint mobility, motor function, muscle performance, and range of motion associated with connective tissue dysfunction.[8]
  • 4E: Impaired joint mobility, motor function, muscle performance, and range of motion associated with localized inflammation.[8]
  • 4F: Impaired joint mobility, motor function, muscle performance, range of motion, and reflex integrity associated with spinal disorders.[8]
  • 5F: Impaired peripheral nerve integrity and muscle performance associated with peripheral nerve injury.[8]

Screening[edit | edit source]

  • Individuals with acromegaly should be screened for changes in joint mobility, decreased exercise tolerance, and weakness.[8]
  • If an acromegalic client presents to physical therapy with unusual muscle weakness, they should be referred to a physician for a complete workup to look for neuropathies and inflammatory myopathies.  This will help identify underlying causes which can be treated.[8]

Orthopedic Intervention[edit | edit source]

  • Arthritis of several joints is often present in patients with acromegaly.[8] In addition, carpal tunnel syndrome is seen in approximately 50% of patients with acromegaly, and there is a similar incidence of thoracic and lumbar pain in acromegalic patients.[8]
  • X-ray studies have demonstrated large osteophytes in the anterior longitudinal ligament and increased intervertebral space.[8]
  • A physical therapy program would work to promote muscle strength, mobility of the joints, and independence with functional activities.[8]  Adaptive equipment for assistance with activities of daily living may be considered depending on the severity of the disease.[8]

Postoperative Intervention[edit | edit source]

  • Exercise and ambulation are encouraged within the first 24 hours after removal of the pituitary tumor.[8]
  • Breathing exercises are indicated.  However, coughing, sneezing, and blowing the nose are contraindicated.[8]
  • Monitor vital signs closely. Any change in vision, dropping pulse rate, or rising blood pressure could indicate an increase in intracranial pressure and should be reported immediately.[8]
  • The physical therapist should consult with the nursing staff to determine if blood glucose monitoring is needed during exercise.  GH levels may drop rapidly after removal of the tumor and result in hypoglycemia.[8]

Differential Diagnosis[edit | edit source]

Several of the comorbidities of acromegaly overlap common disorders; as a result, the acromegaly may be misdiagnosed.[4] The comorbidities which may lead to improper diagnosis include:

  • Arthritis[4]
  • Hypertension[4]
  • Diabetes mellitus[4][6]
  • Carpal tunnel syndrome[4]
  • Respiratory dysfunction[9]
  • Cardiac involvement[9]
  • Failure to suppress growth hormone can also be seen in patients with diabetes, renal failure, hepatic failure, and patients with obesity or those on estrogen replacement.[6]  This could potentially lead to misdiagnosis.

References[edit | edit source]

  1. ↑ 1.0 1.1 Radiopedia Acromegaly Available: https://radiopaedia.org/articles/acromegaly (accessed 18.8.2022)
  2. ↑ 2.0 2.1 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.
  3. ↑ 3.0 3.1 3.2 3.3 Cordero RA, Barkan AL. Current diagnosis of acromegaly. Rev Endocr Metab Disord 2008;9:13-19.
  4. ↑ 4.0 4.1 4.2 4.3 4.4 4.5 4.6 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.
  5. ↑ Merck Manual Home Edition. Acromegaly and gigantism: pituitary gland disorders. (last accessed 10/2/2022).
  6. ↑ 6.0 6.1 6.2 6.3 Melmed S. Acromegaly pathogenesis and treatment. J. Clin. Invest 2009;119:3189-3202.
  7. ↑ Vance M. Acromegaly: a fascinating pituitary disorder. Neurosurg Focus 2010;29(4):1.
  8. ↑ 8.00 8.01 8.02 8.03 8.04 8.05 8.06 8.07 8.08 8.09 8.10 8.11 8.12 8.13 8.14 8.15 8.16 Goodman CC, Fuller KS. Pathology: implications for the physical therapist. 3rd ed. St.Louis: Saunders-Elsevier, 2009.
  9. ↑ 9.0 9.1 Colao A, Ferone D, Marzullo P, Lombardi G. Systemic complications of acromegaly: epidemiology, pathogenesis, and management. Endocrine Reviews. 2004; 25(1):102-152
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