Cushing's syndrome(redirected from Pituitary pars intermedia dysfunction)
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- 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
Causes and symptoms
- 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
- 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).
24-hour free cortisol test
Dexamethasone suppression test
Corticotropin-releasing hormone (crh) stimulation test
Petrosal sinus sampling
Radiologic imaging tests
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 syndromeThe 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).
|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.
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.
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.
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.
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.
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.
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.
|Test||Normal Result||Abnormality With Condition||Explanation|
|Overnight dexamethasone suppression test: 1 mg given PO at 11 p.m.; plasma cortisol levels are measured at 8 a.m. the next morning||Plasma cortisol level: 5 μg/dL||Elevated above 5 μg/dL||Failure 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 hr||Urine cortisol: 20 μg/dL for 24 hr||Elevated above 20 μg/dL for 24 hr||Failure 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 explanation||Elevated 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
DiagnosisFluid volume excess related to abnormal retention of sodium and water
OutcomesFluid balance; Hydration; Nutrition management; Nutrition therapy; Knowledge: Treatment regime
InterventionsFluid and electrolyte management; Fluid monitoring; Medication administration
Planning and implementation
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.
|Medication or Drug Class||Dosage||Description||Rationale|
|Mitotane||2–16 g per day in divided doses PO||Antineoplastic||Inhibits activity of adrenal cortex; used to treat inoperable adrenocortical carcinomas and Cushing’s syndrome|
|Cyproheptadine||4 mg 2–3 times a day PO||Antihistamine; serotonin antagonist||Inhibits 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.
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.
- 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.