In a patient with acromegaly, which assessment finding will the nurse expect to find?
Continuing Education ActivityAcromegaly is a disorder caused by excessive growth hormone production from the anterior pituitary gland, resulting in excessive growth of body tissues and other metabolic dysfunctions. Adult patients with acromegaly have the characteristic facial features of a large lower jaw, prominent forehead, and large hands and feet. This occurs after the growth plates are fused, distinguishing acromegaly from gigantism, which occurs before the fusion of growth plates. This activity reviews the evaluation and management of acromegaly and highlights the interprofessional team's role in providing care to patients affected by this condition. Show
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Access free multiple choice questions on this topic. IntroductionAcromegaly is a rare disorder caused by excessive growth hormone production (GH), most commonly from an adenoma of the anterior pituitary gland. The resulting production of insulin-like growth factor 1 (IGF-1) causes the characteristic overgrowth of certain tissues resulting in coarsening of facial features, enlarging hands and feet, as well as effects on multiple systems throughout the body, including cardiovascular, rheumatologic, neurologic, pulmonary, neoplastic, and metabolic. Appropriate screening and early diagnosis are important to hinder associated morbidity and mortality related to the disease by treating early and the involvement of a multidisciplinary team.[1][2][3][4] EtiologyThe causes of acromegaly can be divided into primary GH excess, ectopic or iatrogenic GH excess, and excess growth hormone-releasing hormone (GHRH). Acromegaly is most commonly caused by a somatotroph GH-secreting adenoma of the anterior pituitary gland. The most commonly associated mutation involves activating the alpha subunit of the guanine nucleotide stimulatory protein gene.[5] Other causes of primary GH excess include pituitary adenomas that secrete multiple hormones and GH-cell carcinomas. Important familial syndromes to be aware of that are associated with acromegaly include Multiple endocrine neoplasia type 1, familial acromegaly, McCune-Albright syndrome, and Carney complex. GH excess can also be ectopic and produced by other tumors such as lymphoma and pancreatic-islet cell tumors. GH excess can also be iatrogenic, resulting from excessive GH administration. Rarer causes of acromegaly are related to GHRH excess. These can be further divided into central and peripheral causes. Central causes include hypothalamic hamartomas, choristoma, and ganglioneuroma. Peripheral causes include secretion of GHRH by bronchial carcinoid tumors, small cell lung cancer, adrenal adenoma, and even production by some medullary thyroid cancer or pheochromocytoma has been described.[6] EpidemiologyAcromegaly has a worldwide prevalence of about 4,600 per million population, with about 116.9 new cases per million per year. The mean age of diagnosis is 40 for men and 45 for women.[7][8] Acromegaly usually presents in the 3rd decade of life. History and PhysicalAcromegaly is usually a slow-progressing disorder, with onset usually in the third or fourth decade of life. The presenting complaints can include the following:
Signs upon physical examination can include the following: General Examination
Visual Field
Neck
Integumentary
Breast
Cardiovascular
Chest
Limbs
EvaluationAcromegaly screening is appropriate in patients presenting with the typical phenotypic features but can also be considered in those patients with multiple disorders commonly associated with acromegaly, such as sleep apnea, hypertension, uncontrolled type 2 diabetes, debilitating arthropathy, and carpal tunnel syndrome. However, screening is not recommended in patients with isolated sleep apnea, type 2 diabetes, or hypertension because of the relative rarity of the syndrome.[11] Screening is biochemical. IGF-1 is used as it does not vary with sleep patterns, exercise, or throughout the day like GH does. Increased IGF-1 level confirms GH excess, and imaging should be done next to localize the source. If the IGF-1 is normal, acromegaly can essentially be ruled out at this point. If the test is equivocal, a GH suppression test should be performed. 75g of glucose is administered to the patient orally, and GH levels are measured before and 2 hours after the glucose load. The diagnosis of acromegaly is confirmed if GH concentration is >1ng/mL after the glucose load.[12] Other dynamic tests exist but are seldom necessary. They can be done if acromegaly is suspected, but the IGF-1 and GH suppression test with oral glucose tolerance test (OGTT) are both normal. Thyrotropin-releasing hormone (TRH) 500 mg can be given intravenously, and in about half of acromegalic patients, the GH level would increase by 50% within 30 minutes. This effect is not observed in the general population.[13] The next step in evaluating these patients is obtaining imaging of the brain as a somatotroph adenoma is the most common cause of acromegaly. The imaging modality of choice is a pituitary MRI. Adenomas have a hypointense signal on T2-weighted images of the MRI.[14] If a mass is seen, proceed to treatment options detailed below. If imaging does not identify a mass, the adenoma may be too small to be visualized or indicate an alternate GH excess source. At this point, alternative imaging should be done, including CT of the chest and abdomen or DOTATE PET scan. This should be done in conjunction with serum measurement of GHRH, and elevated levels, usually > 300ng/mL, are suggestive of extra-pituitary sources. Other tests to be done include evaluating other pituitary hormones. Adenomas can co-secrete more than one hormone and sometimes multiple hormones. Also, depending on the size of the adenoma, compression of the normal pituitary gland can result in deficiencies of other hormonal cell lines.[15] Prolactin can be elevated either due to stalk compression from the pituitary adenoma or as a result of a co-secreting adenoma. One should also check ACTH, early morning cortisol, free T4, FSH, LH with either testosterone or estradiol as appropriate. As a result of common co-morbidities, a hemoglobin A1c should be measured for the screening of diabetes, and a lipid panel as these patients commonly have elevated triglyceride levels and low HDL. If they have symptoms suggestive of sleep apnea, they should be referred for a sleep study. Any concerning heart failure symptoms on physical examination should prompt referral to a cardiologist and an electrocardiogram and echocardiogram. Any visual concerns or adenomas close to or compressing the optic chiasm should be referred to an ophthalmologist for formal visual field testing. Depending on the guidelines followed, a colonoscopy should be done at diagnosis (or age 40).[15] Treatment / ManagementThe goals of acromegaly treatment include controlling biochemical parameters (GH and IGF-1 levels) and associated signs and symptoms, the local mass effect of the tumor, managing comorbidities, and improving mortality.[15] Surgical Therapy Surgery is the treatment of choice for all microadenomas as well as macroadenomas, causing a mass effect. Debulking of macroadenomas without mass effect can also be done and has been described as a modality to allow for better response to medical treatment even if a surgical cure is not likely. The best predictors of surgical cure include smaller tumor size, lower levels of GH/IGF-1, and absence of invasion of surrounding structures such as the cavernous sinus. Certain studies have also shown that neoadjuvant medical treatment with octreotide before surgery results in higher remission rates; however, larger studies are needed to ascertain whether this should be routinely done or which patient may benefit from this approach. In general, this type of surgery should be performed at a center with an experienced pituitary neurosurgeon who performs at least 50 cases per year.[16] Surgical Techniques
Post-surgical Follow-up and Management Immediately post-surgery, urine output monitoring and sodium levels are vital. Patients may either develop the syndrome of inappropriate antidiuretic hormone secretion (SIADH) or, conversely, diabetes insipidus and necessitate desmopressin administration. The adrenal function should be monitored and treated appropriately immediately post-operatively. Those patients with comorbid sleep apnea should not use their CPAP device post-operatively for a while to reduce the risk of pneumocephalus and infections as a result of the high nasal pressures. Thyroid and gonadal axes can undergo testing 6 to 12 weeks after surgery. Studies have shown that immediate day 1 post-operative measurement of GH can be done to assess remission. However, the remaining normal pituitary gland may produce excess GH as a response to surgical stress. An OGTT can be done 1 week post-operatively. A GH value of <0.4ng/mL is used as defining disease control. Serology of GH and IGF-1 can be measured by 3 to 6 months post-operatively as it can take this long for IGF-1 levels to normalize. Remission is then defined when normal IGF-1 levels are seen, and GH after OGTT is measured at <1ng/mL (although some recommend the use of <0.4ng/mL). If remission is confirmed, serology should be repeated at least annually, as relapse has been known to occur in some patients even as long as 10 or more years later. Post-operative imaging should be done a minimum of 3 months after surgery as the fat and gel foam packing can take that long to be resorbed.[16] If residual disease is noted, the patient may need further treatment with repeat surgery if possible and appropriate, medical management, or radiotherapy. Pathology specimens are helpful for further management and prognostication as, for example, when the tumor is densely granulated, this may predict response to octreotide.[18] Also, staining should be done for Ki67 and p21 as these levels have also shown to have prognostic value.[19] Also, if the adenoma stains positive for prolactin, this could predict response to dopamine agonists. Medical Therapy This is considered for patients who do not desire surgery, are too high risk for surgery, not a surgical candidate as the tumor may be unresectable, and in those with recurrent disease after initial surgical management who do not qualify for repeat surgery. As described above, there may be a role for neoadjuvant medical therapy before surgery as well. Medical Agents [20] [21] [22]
Combination medical therapy has also been described. Combining cabergoline with a somatostatin analog has been seen to be effective in a subset of patients, even in the absence of hyperprolactinemia. Another common combination is that of a somatostatin analog with Pegvisomant. A study demonstrated that by adding Pegvisomant to a somatostatin analog in patients seemingly resistant to the somatostatin analog, IGF-1 levels normalized in 95% of patients. Importantly, if this combination is used, one needs to monitor hepatic function as the liver derangement is more common with the combination of drugs than with Pegvisomant alone.[31] A combination of a low-dose somatostatin analog with weekly pegvisomant is not an on-label or approved regimen for acromegaly; however, a study has shown promise for this novel dosing regimen to be effective in biochemical control in addition to being cost-effective in those requiring combination therapy for control of their acromegaly.[32] Radiotherapy Radiotherapy is considered in those patients in whom medical management is not effective in controlling disease, recurrence after surgery, and again the failure of medical therapy. The patients treated with radiotherapy need to be closely monitored for hypopituitarism.
Management of Acromegaly in Pregnancy [15] GH secretion varies during normal pregnancy. GH-secreting tumors have estrogen receptors, particularly those who co-secrete prolactin. The concern is whether the pregnant state would increase the size of the tumor; however, in some studies, it has been found that tumor size does not change significantly during pregnancy in most women. But, given that the risk is still present, women need to be monitored closely with serial visual field monitoring. In a pregnant patient with acromegaly due to a microadenoma, the clinician should discontinue medical management, and these patients can just be closely monitored. The same applies to those with macroadenomas not affecting the optic chiasm with very close monitoring of visual fields. If a woman were to develop worsening symptoms of any kind related to acromegaly, medical management could be reinstituted to help alleviate those symptoms (no biochemical monitoring due to alterations of IGF-1 and GH during pregnancy). Bromocriptine has been used in pregnant women with acromegaly to manage the signs and symptoms without causing adverse fetal harm. However, cabergoline has not been extensively studied in pregnancy. Somatostatin analogs can cross the placenta and potentially decrease uterine blood flow, but longer use of octreotide does not seem to adversely affect the pregnancy or fetal development. Pegvisomant is not recommended as it has not been studied in pregnancy, and its use in pregnancy is limited to case reports. Should visual complaints arise, an MRI is a necessity to determine whether medical management or surgery is necessary at that point. Differential Diagnosis
PrognosisAcromegaly is associated with high mortality rates, chiefly due to malignancies, cardiovascular and respiratory disorders.[38] Individuals with acromegaly have 1.2 to 3.3 times the mortality rate compared to the general population as per the standardized mortality index. However, it has also been postulated that post-operative GH levels correlate the best with overall survival. Thus, if GH/IGF-1 levels are controlled, the life expectancy becomes the same as that of age-matched controls.[39] Factors associated with a worse prognosis include high GH/IGF-1 levels, cardiomyopathy, and hypertension.[40] Morbidity related to the condition often remains despite normalizing GH/IGF-1 levels, but treatment can ameliorate the severity and partially improves the quality of life.[41] Multiple factors influence the prognosis in acromegaly. Patient factors such as age, biochemical factors such as how high GH/IGF-1 levels are at diagnosis, tumor factors including tumor granularity, receptor expression (SSTR2, SSTR5, and D2), markers such as Ki67 and p21, specific mutations, how the tumor behaves in terms of its size and invasion and the T2 intensity of the tumor on MRI. Using this information, a classification of acromegaly into three subtypes has been proposed, guiding prognosis, predicting treatment responsiveness, and thus patient outcomes.[42]
ComplicationsMusculoskeletal Complications [10] Both GH and IGF-1 stimulate the production of periosteal bone formation. IGF-1 creates somatic growth by binding to insulin-like growth factor-1 Receptor (IGF-1R), which is relatively ubiquitous. IGF-1R is a receptor tyrosine kinase that brings about phosphorylation and activation of several intracellular signaling pathways, one of which is the AKT pathway activation that results in somatic cell growth and proliferation. In the craniofacial region, this manifests as characteristic features such as prognathism, teeth separation, jaw thickening, frontal bossing as well as nasal bone hypertrophy. The changes noted in the extremities are due to a combination of increased soft tissue, cartilage, and bone overgrowth, and deformity. Cartilage hypertrophy results in the widening of joint spaces and widening of the phalanges occur due to thickening of the base of the bone and the diaphysis. The spine is also affected by cartilage and bone overgrowth, the phenotype of which is dorsal kyphosis and lumbar hyperlordosis. Deformity due to overgrowth of the costochondral joints can cause splaying of the ribs. Bone mass can give differing results in these patients because acromegaly is associated with other endocrinopathies that may contribute to a low bone mineral density. An example of this would be hypogonadism. Peripheral arthralgias and myalgias are also common. Skin Manifestations The major changes seen with the skin are a result of hyperhidrosis, and patients may complain of excessive sweating and general oiliness of their skin. Additional findings may include skin tags. Skin is also generally thickened as a result of glycosaminoglycan deposition and connective tissue overgrowth. Neurologic Complication Carpal tunnel remains the most common neuropathy. Interestingly, the mechanism is thought to be edema of the median nerve and not external compression of the nerve from soft tissue and bone overgrowth. Also, cerebral aneurysms have been reported with increased frequency in patients with acromegaly, often found incidentally on imaging during an evaluation, but they can also present as cerebral hemorrhage if they should rupture. Cardiovascular Complications Hypertension is frequent in these patients. The pathophysiology seems multifactorial. Primarily, there is an increased plasma volume and perhaps an element of endothelial dysfunction as well. Secondary contributors to the development of hypertension include sleep apnea and insulin resistance. Myocardial biventricular hypertrophy can also occur as a direct result of GH on the myocardium, leading to diastolic dysfunction. Histologically there is necrosis of myocardial cells, interstitial fibrosis, and infiltration of lymphocytes. Initially, this presents as a hyperkinetic heart, then evolving to biventricular hypertrophy and eventually diastolic and then systolic dysfunction.[43] Arrhythmias can also occur but are seldom symptomatic. They occur due to the combination of fibrotic changes, cardiac remodeling, and associated cardiomyopathy. Valvular abnormalities can also occur with increased frequency in acromegalic patients. It has been postulated that this may, in part, be related to some fibrotic changes. Metabolic Complications Both insulin resistance and diabetes occur in acromegaly. This is because GH in excess causes insulin resistance at the level of the liver and peripheral tissues. There is then an increase in beta-cell function as compensation to attempt the maintain euglycemia. Physiologically, GH stimulates the hydrolysis of triglycerides into free fatty acids and glycerol. The combination of chronic insulin resistance and lipotoxicity eventually results in beta-cell dysfunction and eventual apoptosis with subsequent development of diabetes.[44] Respiratory Complications The most common respiratory complication is sleep apnea. The majority of acromegalic patients with sleep apnea have the obstructive kind due to craniofacial anatomic changes such as macroglossia, soft tissue overgrowth of the palate and uvula, and alterations of the jaw. However, one-third of patients do have central sleep apnea. Other abnormalities that can occur again are due to anatomic changes related to the ribcage, thus altering ventilatory mechanics. Neoplastic Complications The risk of colon cancer in acromegaly remains a very controversial topic. A large meta-analysis evaluating the presence of colorectal malignancy in acromegaly was published in 2008. It included around 700 patients with acromegaly compared to around 1500 controls. They found that the odds ratios of developing colon adenomas and colon cancer in patients with acromegaly were 2.5 and 4.3, respectively.[45] There remains no true consensus among different guidelines concerning colonoscopy surveillance. Some guidelines recommend screening at the time of diagnosis, and others starting at age 40. Repeat colonoscopies depend on the findings of the initial colonoscopy.[46] Thyroid nodules can also occur. Sometimes these patients can develop a multinodular goiter and become clinically thyrotoxic. Deterrence and Patient EducationAcromegaly is a multisystem disorder. Patients must be aware that even with treatment and biochemical normalization of GH and IGF-1 levels, comorbidities associated with the disorder may improve but not always be reversible. Long-term follow-up is important with an endocrinologist as well as relevant specialists. The disease can relapse even after years of remission, making follow-up vital. Quality of life in these patients is often low related to the chronicity of the disease, burden of treatment, and the multitude of associated comorbidities. It becomes the physician's responsibility to educate the patient on disease expectations and treatment options to optimize the patient's quality of life.[47] Patients should also thoroughly understand that if they receive a pituitary adenoma surgical intervention, they should be aware of the risk of post-operative complications such as panhypopituitarism or central diabetes insipidus, and they should alert their physician if they develop any new or concerning symptoms such as polyuria. Patients should also be aware of the potential need for additional treatment modalities should surgery not result in a cure. Pearls and Other IssuesMinoxidil use has been associated with a condition characterized by facial features of acromegaly but with normal growth hormone and IGF-1 blood levels. This condition is known as pseudoacromegaly.[37] Enhancing Healthcare Team OutcomesAcromegaly is not a common disorder, but it is associated with very high morbidity and mortality rates when it presents. Because the presentation of acromegaly is systemic, an interprofessional team approach is necessary. In particular, on top of an endocrinologist, other specialists like a cardiologist, oncologist, neurologist, and pulmonologist should be involved as the disorder is associated with malignancies, adverse cardiac, neurologic, and pulmonary events. The nurse practitioner should ensure that the patients are not showing signs of hypopituitarism after treatment and during follow-up appointments and report to the clinician as concerns arise. Levels of GH and IGF need to be monitored for life. Because of the risk of cancer, these patients should receive a referral to the appropriate specialist promptly. Patients need to be educated about the importance of follow-up because the morbidity from cardiac and respiratory complications is very high. Because of joint dysfunction, early physical therapy is recommended. Many of these individuals are not gainfully employed, and thus social work should have involvement in managing their financial status. Due to the challenges of managing medical therapy, a pharmacist should perform medication reconciliation and evaluate for toxicity. Any concerns should be reported to the clinical team. [Level 5] The mortality rates of acromegaly patients are three times the general population, with most dying from respiratory and cardiac complications. These patients also develop several types of tumors, including prostatic hypertrophy, uterine myomas, and skin tags. The overall outcome depends on whether the cause of acromegaly is treatable. Even after surgical removal of a pituitary tumor, some patients may need treatment due to residual disease. The quality of life in these patients is poor.[48][49][50] Thus, it is also important not to subject these patients to unnecessary procedures when medical treatment will suffice. The goal is not to harm the patient. Review QuestionsFigureIllustration of physical attributes of face and hands. Acromegaly, gigantism. Contributed by Chelsea Rowe References1.Drewes AM, Arlien-Søborg MC, Lunde Jørgensen JO, Jensen MP. [Acromegaly and symptoms of the motor apparatus]. Ugeskr Laeger. 2018 Nov 12;180(46) [PubMed: 30417820] 2.Pazarlı AC, Köseoğlu Hİ, Kutlutürk F, Gökçe E. Association of Acromegaly and Central Sleep Apnea Syndrome. Turk Thorac J. 2019 Apr;20(2):157-159. [PMC free article: PMC6453632] [PubMed: 30407161] 3.Postma MR, Wolters TLC, van den Berg G, van Herwaarden AE, Muller Kobold AC, Sluiter WJ, Wagenmakers MA, van den Bergh ACM, Wolffenbuttel BHR, Hermus ARMM, Netea-Maier RT, van Beek AP. Postoperative use of somatostatin analogs and mortality in patients with acromegaly. 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The role of colonoscopic screening in acromegaly revisited: review of current literature and practice guidelines. Pituitary. 2015 Aug;18(4):568-74. [PubMed: 25052731] 47.Adelman DT, Liebert KJ, Nachtigall LB, Lamerson M, Bakker B. Acromegaly: the disease, its impact on patients, and managing the burden of long-term treatment. Int J Gen Med. 2013;6:31-8. [PMC free article: PMC3555549] [PubMed: 23359786] 48.Buchfelder M, van der Lely AJ, Biller BMK, Webb SM, Brue T, Strasburger CJ, Ghigo E, Camacho-Hubner C, Pan K, Lavenberg J, Jönsson P, Hey-Hadavi JH. Long-term treatment with pegvisomant: observations from 2090 acromegaly patients in ACROSTUDY. Eur J Endocrinol. 2018 Dec 01;179(6):419-427. [PubMed: 30325178] 49.He W, Yan L, Wang M, Li Q, He M, Ma Z, Ye Z, Zhang Q, Zhang Y, Qiao N, Lu Y, Ye H, Lu B, Shou X, Zhao Y, Li Y, Li S, Zhang Z, Shen M, Wang Y. Surgical outcomes and predictors of glucose metabolism alterations for growth hormone-secreting pituitary adenomas: a hospital-based study of 151 cases. Endocrine. 2019 Jan;63(1):27-35. [PubMed: 30238327] 50.Rick J, Jahangiri A, Flanigan PM, Chandra A, Kunwar S, Blevins L, Aghi MK. Growth hormone and prolactin-staining tumors causing acromegaly: a retrospective review of clinical presentations and surgical outcomes. J Neurosurg. 2018 Sep 14;131(1):147-153. [PubMed: 30215558] Which symptom would the nurse assess in a patient with acromegaly?Symptoms of acromegaly include an enlarged face and hands. Changes to the face may cause the brow bone and lower jaw to protrude, and the nose and lips to get larger. Acromegaly is a hormonal disorder that develops when your pituitary gland produces too much growth hormone during adulthood.
What is the most common presenting symptom in patients with acromegaly?The most frequent manifestations were morphologic (enlarged hands, enlarged feet), facial modifications (frontal bump, enlargement of the nose), snoring syndrome, and asthenia. The manifestations that were more common in women than men were headache, carpal or cubital tunnel syndrome, constipation, and thyroid nodules.
How do you detect acromegaly?After you've fasted overnight, your doctor will take a blood sample to measure the IGF-1 level in your blood. An elevated IGF-1 level suggests acromegaly. Growth hormone suppression test. This is the best method for confirming an acromegaly diagnosis.
What is acromegaly and its symptoms?Acromegaly is a rare condition where the body produces too much growth hormone, causing body tissues and bones to grow more quickly. Over time, this leads to abnormally large hands and feet, and a wide range of other symptoms. Acromegaly is usually diagnosed in adults aged 30 to 50, but it can affect people of any age.
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