Cushing's syndrome

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Cushing's Syndrome

 

Definition

Cushing's syndrome is a relatively rare endocrine (hormonal) disorder resulting from excessive exposure to the hormone cortisol. The disorder, which leads to a variety of symptoms and physical abnormalities, is most commonly caused by taking medications containing the hormone over a long period of time. A more rare form of the disorder occurs when the body itself produces an excessive amount of cortisol.

Description

The adrenals are two glands, each of which is perched on the upper part of the two kidneys. The outer part of the gland is known as the cortex; the inner part is known as the medulla. Each of these parts of the adrenal gland is responsible for producing different types of hormones. Regulation of hormone production and release from the adrenal cortex involves the pituitary gland, a small gland located at the base of the brain. After the hypothalamus (the part of the brain containing secretions important to metabolic activities) sends "releasing hormones" to the pituitary gland, the pituitary secretes a hormone called adrenocorticotropic hormone (ACTH). The ACTH then travels through the bloodstream to the adrenal cortex, where it encourages the production and release of cortisol (sometimes called the "stress" hormone) and other adrenocortical hormones.
Cortisol, a very potent glucocorticoid—a group of adrenocortical hormones that protects the body from stress and affect protein and carbohydrate metabolism—is involved in regulating the functioning of nearly every type of organ and tissue in the body, and is considered to be one of the few hormones absolutely necessary for life. Cortisol is involved in:
  • complex processing and utilization of many nutrients, including sugars (carbohydrates), fats, and proteins
  • normal functioning of the circulatory system and the heart
  • functioning of muscles
  • normal kidney function
  • production of blood cells
  • normal processes involved in maintaining the skeletal system
  • proper functioning of the brain and nerves
  • normal responses of the immune system
Cushing's syndrome, also called hypercortisolism, has an adverse effect on all of the processes described above. The syndrome occurs in approximately 10 to 15 out of every one million people per year, usually striking adults between the ages of 20 and 50.

Causes and symptoms

The most common cause of Cushing's syndrome is the long-term use of glucocorticoid hormones in medications. Medications such as prednisone are used in a number of inflammatory conditions. Such conditions include rheumatoid arthritis, asthma, vasculitis, lupus, and a variety of other autoimmune disorders in which the body's immune cells accidentally attack some part of the body itself. In these disorders, the glucocorticoids are used to dampen the immune response, thereby decreasing damage to the body.
Cushing's syndrome can also be caused by three different categories of disease:
  • a pituitary tumor producing abnormally large quantities of ACTH
  • the abnormal production of ACTH by some source other than the pituitary
  • a tumor within the adrenal gland overproducing cortisol
Although it is rare, about two-thirds of endogenous (occurring within the body rather than from a source outside the body, like a medication) Cushing's syndrome which is caused by excessive secretion of ACTH by a pituitary tumor, usually an adenoma (noncancerous tumor). The pituitary tumor causes increased growth of the adrenal cortex (hyperplasia) and increased cortisol production. Cushing's disease affects women more often than men.
Tumors in locations other than the pituitary can also produce ACTH. This is called ectopic ACTH syndrome ("ectopic" refers to something existing out of its normal place). Tumors in the lung account for more than half of all cases of ectopic ACTH syndrome. Other types of tumors that may produce ACTH include tumors of the thymus, the pancreas, the thyroid, and the adrenal gland. Nearly all adrenal gland tumors are benign (noncancerous), although in rare instances a tumor may actually be cancerous.
Symptoms of cortisol excess (resulting from medication or from the body's excess production of the hormone) include:
  • weight gain
  • an abnormal accumulation of fatty pads in the face (creating the distinctive "moon face" of Cushing's syndrome); in the trunk (termed "truncal obesity"); and over the upper back and the back of the neck (giving the individual what has been called a "buffalo hump")
  • purple and pink stretch marks across the abdomen and flanks
  • high blood pressure
  • weak, thinning bones (osteoporosis)
  • weak muscles
  • low energy
  • thin, fragile skin, with a tendency toward both bruising and slow healing
  • abnormalities in the processing of sugars (glucose), with occasional development of actual diabetes
  • kidney stones
  • increased risk of infections
  • emotional disturbances, including mood swings, depression, irritability, confusion, or even a complete break with reality (psychosis)
  • irregular menstrual periods in women
  • decreased sex drive in men and difficulty maintaining an erection
  • abormal hair growth in women (in a male pattern, such as in the beard and mustache area), as well as loss of hair from the head (receding hair line).

Diagnosis

Diagnosing Cushing's syndrome can be complex. Diagnosis must not only identify the cortisol excess, but also locate its source. Many of the symptoms listed above can be attributed to numerous other diseases. Although a number of these symptoms seen together would certainly suggest Cushing's syndrome, the symptoms are still not specific to Cushing's syndrome. Following a review of the patient's medical history, physical examination, and routine blood tests, a series of more sophisticated tests is available to achieve a diagnosis.

24-hour free cortisol test

This is the most specific diagnostic test for identifying Cushing's syndrome. It involves measuring the amount of cortisol present in the urine over a 24-hour period. When excess cortisol is present in the bloodstream, it is processed by the kidneys and removed as waste in the urine. This 24-hour free cortisol test requires that an individual collect exactly 24-hours' worth of urine in a single container. The urine is then analyzed in a laboratory to determine the quantity of cortisol present. This technique can also be paired with the administration of dexamethasone, which in a normal individual would cause urine cortisol to be very low. Once a diagnosis has been made using the 24-hour free cortisol test, other tests are used to find the exact location of the abnormality causing excess cortisol production.

Dexamethasone suppression test

This test is useful in distinguishing individuals with excess ACTH production due to a pituitary adenoma from those with ectopic ACTH-producing tumors. Patients are given dexamethasone (a synthetic glucocorticoid) orally every six hours for four days. Low doses of dexamethasone are given during the first two days; for the last two days, higher doses are administered. Before dexamethasone is administered, as well as on each day of the test, 24-hour urine collections are obtained.
Because cortisol and other glucocorticoids signal the pituitary to decrease ACTH, the normal response after taking dexamethasone is a drop in blood and urine cortisol levels. Thus, the cortisol response to dexamethasone differs depending on whether the cause of Cushing's syndrome is a pituitary adenoma or an ectopic ACTH-producing tumor.
However, the dexamethasone suppression test may produce false-positive results in patients with conditions such as depression, alcohol abuse, high estrogen levels, acute illness, and stress. On the other hand, drugs such as phenytoin and phenobarbital may produce false-negative results. Thus, patients are usually advised to stop taking these drugs at least one week prior to the test.

Corticotropin-releasing hormone (crh) stimulation test

The CRH stimulation test is given to help distinguish between patients with pituitary adenomas and those with either ectopic ACTH syndrome or cortisol-secreting adrenal tumors. In this test, patients are given an injection of CRH, the corticotropin-releasing hormone that causes the pituitary to secrete ACTH. In patients with pituitary adenomas, blood levels of ACTH and cortisol usually rise. However, in patients with ectopic ACTH syndrome, this rise is rarely seen. In patients with cortisol-secreting adrenal tumors, this rise almost never occurs.

Petrosal sinus sampling

Although this test is not always necessary, it may be used to distinguish between a pituitary adenoma and an ectopic source of ACTH. Petrosal sinus sampling involves drawing blood directly from veins that drain the pituitary. This test, which is usually performed with local anesthesia and mild sedation, requires inserting tiny, flexible tubes (catheters) through a vein in the upper thigh or groin area. The catheters are then threaded up slowly until they reach veins in an area of the skull known as the petrosal sinuses. X rays are typically used to confirm the correct position of the catheters. Often CRH is also given during the test to increase the accuracy of results.
When blood tested from the petrosal sinuses reveals a higher ACTH level than blood drawn from a vein in the forearm, the likely diagnosis is a pituitary adenoma. When the two samples show similar levels of ACTH, the diagnosis indicates ectopic ACTH syndrome.

Radiologic imaging tests

Imaging tests such as computed tomography scans (CT) and magnetic resonance imaging (MRI) are only used to look at the pituitary and adrenal glands after a firm diagnosis has already been made. The presence of a pituitary or adrenal tumor does not necessarily guarantee that it is the source of increased ACTH production. Many healthy people with no symptoms or disease whatsoever have noncancerous tumors in the pituitary and adrenal glands. Thus, CT and MRI is often used to image the pituitary and adrenal glands in preparation for surgery.

Treatment

The choice of a specific treatment depends on the type of problem causing the cortisol excess. Pituitary and adrenal adenomas are usually removed surgically. Malignant adrenal tumors always require surgical removal.
Treatment of ectopic ACTH syndrome also involves removing all of the cancerous cells that are producing ACTH. This may be done through surgery, chemotherapy (using combinations of cancer-killing drugs), or radiation therapy (using x rays to kill cancer cells), depending on the type of cancer and how far it has spread. Radiation therapy may also be used on the pituitary (with or without surgery) for patients who cannot undergo surgery, or for patients whose surgery did not successfully decrease pituitary release of ACTH.
There are a number of drugs that are effective in decreasing adrenal production of cortisol. These medications include mitotane, ketoconazole, metyrapone, trilostane, aminoglutethimide, and mifepristone. These drugs are sometimes given prior to surgery in an effort to reverse the problems brought on by cortisol excess. However, the drugs may also need to be administered after surgery (sometimes along with radiation treatments) in patients who continue to have excess pituitary production of ACTH.
Because pituitary surgery can cause ACTH levels to drop too low, some patients require short-term treatment with a cortisol-like medication after surgery. Patients who need adrenal surgery may also require glucocorticoid replacement. If the entire adrenal gland has been removed, the patient must take oral glucocorticoids for the rest of his or her life.

Prognosis

Prognosis depends on the source of the problem. When pituitary adenomas are identified as the source of increased ACTH leading to cortisol excess, about 80% of patients are cured by surgery. When cortisol excess is due to some other form of cancer, the prognosis depends on the type of cancer and the extent of its spread.

Key terms

Adenoma — A type of noncancerous (benign) tumor that often involves the overgrowth of certain cells of the type normally found within glands.
Adrenocorticotropic hormone (ACTH) — A pituitary hormone that stimulates the cortex of the adrenal glands to produce adrenal cortical hormones.
Cortisol — A hormone secreted by the cortex of the adrenal gland. Cortisol regulates the function of nearly every organ and tissue in the body.
Ectopic — In an abnormal position.
Endocrine — Pertaining to a gland that secretes directly into the bloodstream.
Gland — A collection of cells whose function is to release certain chemicals (hormones) that are important to the functioning of other, sometimes distantly located, organs or body systems.
Glucocorticoids — General class of adrenal cortical hormones that are mainly active in protecting against stress and in protein and carbohydrate metabolism.
Hormone — A chemical produced in one part of the body that travels to another part of the body in order to exert its effect.
Hypothalamus — the part of the brain containing secretions important to metabolic activities.
Pituitary — A gland located at the base of the brain, the pituitary produces a number of hormones, including hormones that regulate growth and reproductive function.

Resources

Books

Williams, Gordon H., and Robert G. Dluhy. "Hyperfunction of the Adrenal Cortex." In Harrison's Principles of Internal Medicine, edited by Anthony S. Fauci, et al. New York: McGraw-Hill, 1998.

Periodicals

Boscaro, Marco, Luisa Barzon, and Nicoletta Sonino. "The Diagnosis of Cushing's Syndrome: Atypical Presentations and Laboratory Shortcomings." Archives of Internal Medicine 160 (2000): 3045-53.
Boscaro, Marco, Luisa Barzon, Francesco Fallo, and Nicoletta Sonino. "Cushing's Syndrome." Lancet 357 (2001): 783-91.
Kirk, Lawrence F., Robert B. Hash, Harold P. Katner, and Tom Jones. "Cushing's Disease: Clinical Manifestations and Diagnostic Evaluation." American Family Physician 62, no. 5 (September 1, 2001): 1119-27.
Newell-Price J., and A. Grossman. "Diagnosis and Management of Cushing's Syndrome." Lancet 353 (1999): 2087-88.

Cushing's syndrome

 [koosh´ingz]
a group of symptoms produced by an excess of free circulating cortisol from the adrenal cortex. This may be the result of: (1) excessive secretion of adrenocorticotropic hormone (ACTH) from the pituitary gland, which may actually result from faulty release of corticotropin-releasing factor from the hypothalamus; (2) tumor of the adrenal cortex, causing hypersecretion of the glucocorticoids; (3) ectopic production of ACTH by extrapituitary tumors, most commonly lung carcinoma, medullary thyroid carcinoma, and thymoma; and (4) iatrogenic Cushing's syndrome resulting from overzealous administration of exogenous glucocorticoids.

Diagnosis of the disorder is established by laboratory findings of a continuous elevation of plasma cortisol. The symptoms and signs are a result of the action of this hormone and include fatty swellings in the interscapular area (buffalo hump) and in the facial area (moon face), distention of the abdomen, ecchymoses following even minor trauma, impotence, amenorrhea, high blood pressure, osteoporosis, and general weakness due to excessive protein catabolism and loss of muscle mass. There also can be hirsutism in females and streaked purple markings (atrophic striae) in the abdominal area as a result of collections of body fat. Patients who have a familial predisposition to diabetes mellitus frequently develop insulin-dependent diabetes mellitus as a result of the anti-insulin, diabetogenic properties of cortisol.

Treatment of Cushing's syndrome is becoming more effective as new modes of therapy become available. Pituitary Cushing's syndrome can be treated by surgical excision of the neoplasm using microsurgical techniques. Radiation with cobalt also is helpful in some cases. Drug therapy using adrenocorticolytic agents may be used as an adjunct to surgery and radiation or as an alternative when these modes of therapy are not feasible.
Cushing's syndrome. From Damjanov, 1996.

Cushing's syndrome

Etymology: Harvey W. Cushing
a metabolic disorder resulting from the chronic and excessive production of cortisol by the adrenal cortex or by the administration of glucocorticoids in large doses for several weeks or longer. When occurring spontaneously, the syndrome represents a failure in the body's ability to regulate the secretion of cortisol or adrenocorticotropic hormone (ACTH). (Normally cortisol is produced only in response to ACTH, and ACTH is not secreted in the presence of high levels of cortisol.) The most common cause of the syndrome is a pituitary tumor that increases secretion of ACTH. Also called hyperadrenocorticism. See also Addison's disease, Cushing's disease, Nelson's syndrome.
observations Characteristically the patient with Cushing's syndrome has a decreased glucose tolerance; central obesity; round "moon" face; supraclavicular fat pads; an overhanging, striae-covered pad of fat on the chest and abdomen; buffalo hump; scant menstrual periods or decreased testosterone levels; muscular atrophy; edema; hypokalemia; and some degree of emotional change. The skin may be abnormally pigmented and fragile; minor infections may become systemic and long-lasting. Children with the disorder may stop growing. Hypertension, kidney stones, and psychosis may also occur.
interventions The objective of all treatment is decreased cortisol secretion. The source of the excess ACTH is discovered by a series of tests that challenge the function of the adrenal and pituitary glands. If the excess ACTH is caused by an adenoma of the anterior pituitary, irradiation or surgical excision of the tumor corrects the condition. If the condition is the result of medication, decreasing or changing the dosage may alleviate the symptoms.
nursing considerations In caring for children the nurse normally observes height and weight, because rapidly developing obesity and failure to grow are suggestive of Cushing's syndrome. Some medications used for some forms of the condition may cause nausea and anorexia, somnolence, and lethargy. The patient is informed of this possibility. Nursing care of the hospitalized patient with Cushing's syndrome is similar to that of patients with Addison's disease, Cushing's disease, and other endocrinological disorders. Weight and electrolyte and fluid balance are monitored, an adequate balanced diet is urged, and emotional changes are observed with a goal of maintaining emotional equilibrium.
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Cushing's syndrome

Cushing's syndrome

The bodily changes caused by excessive secretion of corticosteroid hormones often as a result of a pituitary or adrenal tumour. A person with Cushing's syndrome is over-weight and has fat deposits on the back of the neck and shoulders. The face is ‘moon-shaped’ and there are often purplish streaks (striae) on the abdomen. Women often show male-pattern hairiness or baldness. There is weakness from wasted muscles, high blood pressure (HYPERTENSION), OSTEOPOROSIS, and often mental disturbances. (Harvey Williams Cushing, 1869–1939, American neurosurgeon).

Cushing’s Syndrome

DRG Category:644
Mean LOS:4.9 days
Description:MEDICAL: Endocrine Disorders With CC

Cushing’s syndrome is defined as the clinical effects of increased glucocorticoid hormone. It can be characterized by an excess production of glucocorticoids (primarily cortisol) by the cortex of the adrenal gland, but it is most commonly due to therapy with glucocorticoid drugs. Cortisol is an essential hormone for many body functions, including maintaining normal electrical excitation of the heart, blood glucose level, nerve cell conduction, and adequate circulatory volume, and for metabolizing proteins, fats, and carbohydrates.

Overproduction of glucocorticoids leads to a host of multisystem disorders in metabolism, water balance, wound healing, and response to infection. Complications affect almost every system of the body. Increased calcium resorption from bones may lead to osteoporosis and bone fractures. A blunted immune response causes a high risk for infection as well as poor wound healing. Cushing’s syndrome may also mask life-threatening infections. Gastrointestinal (GI) irritation may lead to peptic ulcers, and both insulin resistance and glucose intolerance can cause hyperglycemia.

Causes

The causes are divided into three categories: iatrogenic, primary, and secondary. Iatrogenic causes are a result of excessive cortisol levels from chronic therapy with glucocorticoids. Approximately 25% of cases have a primary cause of excessive cortisol production from adrenal neoplasms, such as adenomas or carcinomas. The tumors usually affect only one adrenal gland, and about 50% are malignant. Even with appropriate treatment, most patients with adrenal carcinoma die within 3 years because of metastases to the liver and lung. Secondary Cushing’s syndrome is more common than primary, and bilateral adrenal hyperplasia is the most common secondary condition. It is caused by excessive production of adrenocorticotropic hormone (ACTH) from the anterior pituitary gland.

Genetic considerations

Several genetic diseases can result in Cushing’s syndrome. These include multiple endocrine neoplasia type I (MEN I), McCune-Albright syndrome, Li-Fraumeni syndrome, Beckwith-Wiedemann syndrome, and Carney’s complex.

Gender, ethnic/racial, and life span considerations

While 90% of cases of Cushing’s syndrome occur in adults, in infants it usually results from adrenal carcinoma. Primary disease is often linked to a familial autoimmune disorder and is seen in children and young adults of both genders. Secondary disease is more common than primary disease in children older than 6 or 7 and, as in adults, is usually the result of overproduction of ACTH. In adults, secondary Cushing’s syndrome that results from pituitary disease is most common in females aged 30 to 50 years. Secondary Cushing’s syndrome that results from increased ACTH secretion is more common in males, possibly because of the higher incidence of bronchogenic carcinoma caused by smoking. There are no known racial and ethnic considerations.

Global health considerations

Incidence of adrenal tumors is greater in some regions of the world, such as South America and Japan, but it is unclear whether these differences are caused by environmental or genetic factors.

Assessment

History

Ask if the patient has had recent changes in memory, attention span, or behavior. Discuss the patient’s sleep-wake pattern and evaluate the patient for sleep disturbances. Family members may comment on the patient’s changed affect, short-term memory, emotional instability, and ability to concentrate.

Other signs include weakness, fatigue, back pain, general discomfort, difficulty completing activities of daily living, and changes in the urinary output. Be sure to question the patient about weight gain and changes in body proportions between the shoulders. Patients may also notice changes in their appetite and thirst. Other changes in appearance include hirsutism, oily skin, acne, purple striae, and poor wound healing. Women may have noted changes in menstruation, and both men and women may note changes in libido and in their feelings about themselves.

Physical examination

Most common symptoms include muscle weaknesses, visual disturbances, and weight gain. Changes in fat metabolism lead to generalized obesity, a round (“moon”) face, a hump in the interscapular area, and truncal obesity. Hyperpigmentation of skin and mucous membranes may be present as a result of increases in ACTH. Because of alterations in protein metabolism, loss of collagen support in the skin leaves the skin more fragile and easily bruised. Both males and females experience changes in secondary sexual characteristics and body hair distribution, along with an increase in acne. Some patients have peripheral edema from water and sodium retention. Muscle wasting, especially in extremities, leads to difficulty in getting up and down from a sitting position, difficulty in climbing stairs, or generalized weakness and fatigue. Note if the extremities are thin with atrophied muscles.

Auscultate the patient’s blood pressure; most patients are hypertensive because of increased circulating volume or increased sensitivity of the arterioles to circulating catecholamines. Neck vein distention may be present.

Psychosocial

A diagnosis of Cushing’s syndrome can be devastating. Determine the patient’s response to the disease and the effect the disease has had on the patient’s sexuality, body image, and relationships with others.

Diagnostic highlights

Abnormal blood chemistries are common, including hypokalemia, hypochloremia, and metabolic alkalosis because of increased excretion of potassium and chloride. Random cortical tests are not useful for diagnosis because of the wide range of normal values.

TestNormal ResultAbnormality With ConditionExplanation
Overnight dexamethasone suppression test: 1 mg given PO at 11 p.m.; plasma cortisol levels are measured at 8 a.m. the next morningPlasma cortisol level: 5 μg/dLElevated above 5 μg/dLFailure to suppress normal cortisol response is diagnostic of Cushing’s syndrome; abnormal results indicate need for low-dose dexamathasone suppression test
Low-dose dexamathasone suppression test; 0.5 mg dexamethasone given PO q 6 hr for 48 hrUrine cortisol: 20 μg/dL for 24 hrElevated above 20 μg/dL for 24 hrFailure to suppress normal cortisol response is diagnostic of Cushing’s syndrome; testing should not be done during severe illness or depression, which may lead to false-positive results; phenytoid therapy alters dexamethasone metabolism and may lead to false results
Adrenocorticotropic hormone level< 80 pg/mL in a.m. < 50 pg/mL in p.m.Results vary; see explanationElevated with high cortisol levels if ACTH-producing tumor; decreased with high cortisol levels if adrenal adenoma or carcinoma is present

Other Tests: Complete blood count, computed tomography scan, ultrasound, and angiogram

Primary nursing diagnosis

Diagnosis

Fluid volume excess related to abnormal retention of sodium and water

Outcomes

Fluid balance; Hydration; Nutrition management; Nutrition therapy; Knowledge: Treatment regime

Interventions

Fluid and electrolyte management; Fluid monitoring; Medication administration

Planning and implementation

Collaborative

The main focus is to find the primary cause of the cortisol excess and remove it if possible. In the case of iatrogenic Cushing’s syndrome, care is focused on alleviating as many of the signs and symptoms as possible when the therapy cannot be discontinued. If the patient has primary Cushing’s syndrome from an adrenal tumor, the tumor is removed surgically. Even if the tumor is unilateral, the patient is treated for adrenal insufficiency after the surgery because the high levels of cortisol from the tumor may have caused the unaffected adrenal gland to atrophy. Patients with adrenal carcinoma are treated postoperatively with mitotane to treat metastases. Throughout the patient’s recovery, fluid, electrolyte, and nutritional assessment and balance are essential.

For secondary Cushing’s syndrome from a pituitary tumor, the preferred option is a transsphenoidal adenectomy, a procedure that explores the pituitary gland to find microadenomas. It is successful in 20% to 70% of patients. A second option is the transsphenoidal hypophysectomy, a procedure that removes the entire pituitary gland and leads to a cure in 100% of the patients. It is usually used for more invasive tumors and requires lifelong hormone replacement (glucocorticoids, thyroid hormone, gonadal steroids, and antidiuretic hormone). In a newer procedure, surgeons use endoscopic surgery, operating through a fiberoptic device that has been inserted through an incision in the lining of the nose. This procedure takes less time and causes fewer complications than transsphenoidal hypophysectomy. A third alternative is bilateral total adrenalectomy, which cures the signs and symptoms of excess cortisol but does not decrease ACTH secretion. The patient requires lifelong replacement therapy with glucocorticoids and mineralocorticoids. If the patient has secondary Cushing’s syndrome because of ectopic production of ACTH from a nonendocrine tumor, the first concern is to remove the source of the ectopic secretion of ACTH. If this is not possible, mitotane (see Pharmacologic Highlights) decreases cortisol production in the adrenal gland but may cause damage to the gland and is used with caution. Radiation therapy is used when the patient either has no defined tumor or needs an adjunct to tumor removal.

postoperative.
Patients with pituitary surgery need careful management of airway, breathing, and circulation. In the first postoperative hours, serial neurological examinations are important to identify the risk for increased intracranial pressure from edema. The incision is generally performed through the upper gum line; ask the surgeon about the procedure for oral hygiene. Nasal packing is removed after 2 days in most patients. At that time, observe for rhinorrhea and ask the patient to report a “runny” nose. Teach the patient to avoid coughing, sneezing, or blowing the nose immediately after surgery.

Pharmacologic highlights

Medication or Drug ClassDosageDescriptionRationale
Mitotane2–16 g per day in divided doses POAntineoplasticInhibits activity of adrenal cortex; used to treat inoperable adrenocortical carcinomas and Cushing’s syndrome
Cyproheptadine4 mg 2–3 times a day POAntihistamine; serotonin antagonistInhibits the release of ACTH from pituitary gland; drug is considered last resort and often causes no improvement

Other Drugs: Aminoglutethimide inhibits cholesterol synthesis, and metyrapone partially inhibits adrenal cortex steroid synthesis. These drugs may be used in conjunction with surgery or radiation if the tumor is not completely resectable or if complete remission is not expected.

Independent

An important goal is to limit the risk of infection for the patient. Note, document, and report any signs of skin or pulmonary infection. Restrict visitors with upper respiratory infections. Unless contraindicated after surgery, encourage the patient to cough and deep breathe, turn in bed at least every 2 hours, and use good oral hygiene. Focus on helping the patient deal with changes in body image, sexuality, and self-esteem. Let the patient know that many of the body changes are reversible with treatment; this information allows the patient to focus on setting goals. Include the patient’s partner in all education. Patient and family teaching occurs throughout the patient’s hospitalization and after discharge. Provide information about patient care and activity restrictions. Explanations of all diagnostic tests and their findings, as well as the treatment plan, are important. The patient and family often require time to consider treatment options. As time progresses, the patient and family need information about the disease process and lifelong management with medication and diet changes.

Evidence-Based Practice and Health Policy

Dekkers, O.M., Horvath-Puho, E., Jorgensen, J.O., Cannegieter, S.C., Ehrenstein, V., Vandenbroucke, J.P., …Sorensen, H.T. (2013). Multisystem morbidity and mortality in Cushing’s syndrome: A cohort study. The Journal of Endocrinology and Metabolism, 98(6), 2277–2284.

  • Investigators reviewed data from a nationwide cohort study among 34,643 adults and documented 343 cases of Cushing’s syndrome, which they found were significantly associated with increased morbidity and mortality risk.
  • Patients with Cushing’s syndrome were 2.6 times more likely to develop venous thromboembolism (95% CI, 1.5 to 4.7), 3.7 times more likely to experience a myocardial infarction (95% CI, 2.4 to 5.5), twice as likely to have a stroke (95% CI, 1.3 to 3.2), and 4.9 times more likely to develop an infection (95% CI, 3.7 to 6.4) compared to patients without Cushing’s syndrome.
  • Patients with Cushing’s syndrome were also more likely than the comparison population to have comorbidities including cancer (7.6% versus 2.8%), diabetes (13.1% versus 1.4%), and hypertension (25.4% versus 2.6%).
  • Compared to the population cohort, the risk of mortality among patients with Cushing’s syndrome was increased by 5.2 times (95% CI, 2.7 to 9.7) in the first year, but decreased to 2.1 times (95% CI, 1.7 to 2.7) during long-term follow-up.

Documentation guidelines

  • Physical response: Vital signs, neurological assessment, cardiopulmonary assessment, wound healing, signs of infection (fever, wound drainage, productive cough), important laboratory deviations (serious electrolyte imbalances, alterations in glucose levels)
  • Nutrition: Daily weights, appetite, food tolerance, food preference, response to diet teaching, calorie count if indicated
  • Emotional response: Concerns over body image, self-concept, mood, affect

Discharge and home healthcare guidelines

Describe the pathophysiology of the disease. Identify factors that aggravate the disease (stress, changes in diet, injury) as well as the signs and symptoms. Explore complications of the disease and ask whom to notify if they occur. Describe the treatment plan and expected effects as well as possible complications. Describe all medications, including the name, dosage, action, side effects, route, and importance of lifelong dosing if indicated.

Cushing's syndrome

a group of signs produced by an excess of free circulating cortisol from the adrenal cortex. Causes are: (1) excessive secretion of adrenocorticotropic hormone (ACTH) from the pituitary gland, which may actually result from faulty release of corticotropin-releasing factor (CRF) from the hypothalamus or a functional tumor, usually corticotroph adenoma; (2) tumor of the adrenal cortex, causing hypersecretion of the glucocorticoids; (3) iatrogenic Cushing's syndrome resulting from excessive or prolonged administration of exogenous glucocorticoids; and in humans (4) ectopic production of ACTH by extrapituitary tumors. Most commonly seen in dogs (canine Cushing's syndrome; CCS), infrequently in aged horses and rarely in other species. See also hyperadrenocorticism.
Affected dogs show polyuria, polydipsia, polyphagia, muscle weakness and atrophy, pendulous abdomen, hair loss and an increased susceptibility to infection, particularly of the skin and urinary tract.
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Canine Cushing's syndrome.

pseudo Cushing's syndrome
see growth hormone-responsive dermatosis.