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DEFINITION Cushing’s disease is a condition in which the pituitary gland releases too much adrenocorticotropic hormone (ATCH). ACTH stimulates the production and release of cortisol, a stress hormone. Too much ACTH means too much cortisol. The pituitary gland is an organ of the endocrine system. It is the hyperactivity of the adrenal cortex that results in excessive secretion of glucocorticoids (cortisol), mineralocorticoids (aldosterone) and androgen (male sex hormone). Cortisol is normally released during stressful situations. It controls the body's use of carbohydrates, fats, and proteins and also helps reduce the immune system's response to swelling (inflammation). It may be caused by taking too much corticosteroid medications, such as prednisone and prednisolone. These drugs are used to treat conditions such as asthma and rheumatoid arthritis. This pathology was described by Harvey Cushing in 1932. The syndrome is also called Itsenko-Cushing syndrome, hyperadrenocorticism or hypercorticism) Cushing's syndrome is not confined to humans and is also a relatively common condition in domestic dogs and horses.
Harvey Williams Cushing, M.D. (April 8, 1869 - October 7, 1939) was an American neurosurgeon and a pioneer of brain surgery, and the first to describeCushing's syndrome. He is often called the "father of modern neurosurgery". Cushing's Syndrome was also the first autoimmune disease identified in humans The incidence of pituitary tumors may be relatively high, as much as one in five people. CAUSE Exogenous vs. endogenous Hormones that come from outside the body are called exogenous; hormones that come from within the body are called endogenous. The most common cause of Cushing's syndrome is exogenous administration of glucocorticoids prescribed by a health care practitioner to treat other diseases (called iatrogenic Cushing's syndrome). This can be an effect of steroid treatment of a variety of disorders such as asthma and rheumatoid arthritis, or in immunosuppression after an organ transplant. Administration of synthetic ACTH is also possible, but ACTH is less often prescribed due to cost and lesser utility. Although rare, Cushing's syndrome can also be due to the use of medroxyprogesterone. Endogenous Cushing's syndrome results from some derangement of the body's own system of secreting cortisol. Normally, ACTH is released from
PATHOPHYSIOLOGY Cortisol excess result in anti-inflammatory effect and excessive catabolism of protein and peripheral fat to support hepatic glucose production. In adrenal Cushing's. The mechanism may be ACTH. a benign pituitary adenoma secretes ACTH. in which excess cortisol is produced by the adrenal cortex or exogenously administered. tumors outside the normal pituitary-adrenal system can produce ACTH that affects the adrenal glands. . In pituitary Cushing's.secreting tumors. hyperplastic adrenal glands.dependent. also present in ectopic ACTH. This final etiology is called ectopic or paraneoplastic Cushing's syndrome and is seen in diseases like small cell lung cancer. or adrenal glands with nodular adrenal hyperplasia. excess cortisol is produced by adrenal gland tumors.independent. This is also known as Cushing's disease and is responsible for 70% of endogenous Cushing's syndrome.the pituitary gland when necessary to stimulate the release of cortisol from the adrenal glands. This suppresses the hypothalamicpituitary-adrenal axis. Finally. or ACTH. in which elevated plasma ACTH level stimulate the adrenal cortex to produce excess cortisol.
thighs • Risk for infection • Hyper glycemia • Glycosuria • Osteoporosis • Voice deepening • Hirsutism (female) • Menstrual irregularities • Thinning hair • Decreased libido Androgens • Fluid volume • Hypertension • Hypokalemia • Sodium imbalance • Muscle weakness . abdomen.SIGNS AND SYMPTOMS Glucocorticiod Mineralocorticoid • Mood change • Skinny arms and leg • Thin skin • Muscle weakness • Poor wound healing • Bruising • Ketonuria • Buffalo hump • Truncal obesity • Pink/purple stretch mark on the breast.
and depression . impaired sexual functioning. fatigue. irritability. and decreased activity level • Disturbed thought process related to mood swings. muscle wasting. impaired healing.NURSING DIAGNOSIS • Risk for injury related to weakness • Risk for infection related to altered protein metabolism and inflammatory response • Self-care deficit related to weakness. and thin and fragile skin • Disturbed body image related to altered physical appearance. and altered sleep patterns • Impaired skin integrity related to edema.
NURSING INTERVENTION • Decreasing Risk of injury -establishing a protective environment -Foods high in protein.if indicated. and the postoperative course -transspenoidal hypophysectomy • Encouraging Rest and Activity -Encourage moderate activity -help patient to plan or make schedule for his/her rest periods throughout the day • Promoting Skin Integrity -Meticulous skin care -use of adhesive tapes is avoided -encourage and assists patient to change position frequently . calcium and vitamin D are recommended • Decreasing Risk of Infection -patient should avoid unnecessary exposure to others w/ infection • Preparing the Patient for Surgery -patient is prepared for adrenalectomy.
. • Improving Thought Processes -Explanations to the patient and family members about the cause of emotional instability are important -Psychotic behavior may occur in few patients and should be reported.• Improving Body Image -provide discussion of the effect the changes that had on his/her self-concept and relationship with others.
Efficacy of these medical interventions can be assessed with serial measurements of 24-hour urinary free cortisol. ketoconazole. In ACTH-dependent Cushing syndrome. Thus. ideally. in conjunction with a specialist. • Ketoconazole is probably the most popular and effective of these agents for long-term use and usually is the agent of choice. Ketoconazole and aminoglutethimide act at several sites. have been used to cause medical adrenalectomy.MEDICAL MANAGEMENT • Agents that inhibit steroidogenesis. It acts on . These medications are used rarely and often are toxic at the doses required to reduce cortisol secretion. metyrapone. • Metyrapone and trilostane are agents that competitively inhibit a single steroidogenic enzyme. which counters the actions of these medications. such as mitotane. trilostane. Patients should be counseled on the signs and symptoms of adrenal insufficiency when starting these drugs. ACTH secretion continues to stimulate steroidogenesis. and etomidate. medical treatment should be initiated cautiously and. aminoglutethimide. • Patients receiving these medications may require glucocorticoid replacement to avoid adrenal insufficiency.
Therapy is begun at 1 g/d divided into 4 doses and increased to a maximum dose of 4. the dose may be maintained while another steroid enzyme inhibitor. gynecomastia. nausea. cholesterol side-chain cleavage.5 g/d. impotence. Ketoconazole is less effective in patients on H2 blockers or proton-pump inhibitors because gastric acidity is required for metabolism. the final step in cortisol synthesis. o A daily dose of 600-800 mg often decreases cortisol production. typically metyrapone. The drug is contraindicated during pregnancy.several of the P450 enzymes. irregular menses. and hirsutism. decreased libido. acne. sedation. is initiated. including hypertension. . Adverse effects of ketoconazole include headache. including the first step in cortisol synthesis. and elevated liver function tests. If this agent is ineffective at controlling hypercortisolism. Adverse effects are from increases in androgen and mineralocorticoid precursors. o o • Metyrapone blocks 11-beta-hydroxylase activity. and conversion of 11deoxycortisol to cortisol.
It can be used in addition to radiation therapy for treatment of Cushing disease and in combination with metyrapone or aminoglutethimide for treatment of ectopic ACTH secretion.• Aminoglutethimide is an anticonvulsant agent that blocks cholesterol side-chain cleavage to pregnenolone. a generalized pruritic rash. In rare cases. it may cause bone marrow suppression. and goiter. o o • Mitotane is an adrenolytic agent that acts by inhibiting 11-beta hydroxylase and cholesterol side-chain cleavage enzymes. and doses of 1-2 g daily are often used. o Adverse effects of aminoglutethimide include somnolence. Aminoglutethimide is typically initiated at 250 mg twice daily. it is used in treatment of adrenal cancer at doses of 2-4 g daily. headache. hypothyroidism. Its survival benefit is unclear. For this reason. It is a relatively weak adrenal enzyme inhibitor at doses that patients can tolerate. This drug also leads to mitochondrial destruction and necrosis of adrenocortical cells in the zona fasciculata and reticularis. Aminoglutethimide increases the metabolism of dexamethasone but not cortisol. .
and ataxia. it is relatively contraindicated in women interested in remaining fertile. arthralgias. therefore. and leukopenia. and its utility is limited by adverse gastrointestinal and neurologic effects. including nausea. Other adverse effects include rash. dizziness. diarrhea. mitotane is expensive. o o . It is taken up by adipose tissues and persists in the circulation long after discontinuation.o Unfortunately. It is a potential teratogen and can cause abortion.
Sometimes a tumor cannot be identified. . well-defined pituitary adenoma is called transsphenoidal adenomectomy. lighted tube with a camera). The incision is extended through the sphenoid sinus. radiation. This type of surgery permanently cures Cushing's syndrome in 60 to 70 percent of people. or medication to lower cortisol levels. The pituitary is located at the base of the brain. half of the pituitary gland may be removed (hemihypophysectomy) or 85 to 90 percent of the pituitary gland may be removed (subtotal hypophysectomy) to be certain that the tumor has been removed. It is possible to access this area through the gums above the upper front teeth or the nose. Surgery Surgical removal of a small. treatment may include surgery.SURGICAL MANAGEMENT When Cushing's syndrome results from an ACTH-producing tumor of the pituitary gland (Cushing's disease). Lifelong hormone replacement is often necessary after surgery. the surgeon makes an incision in one of these areas. in these cases. allowing the surgeon to see and remove the adenoma with an endoscope (a thin. Surgical removal of half or more of the pituitary gland can reduce pituitary function and interfere with ovulation (in women) and sperm production (in men). Using special instruments.
medications that lower adrenal cortisol production may be given while waiting for the effects of radiation. and aminoglutethimide. These medications include ketoconazole. Radiation of pituitary tumors reduces cortisol levels in about half of adults and most children with Cushing's disease.Radiation Radiation can be a useful treatment when pituitary tumors cannot be completely removed by surgery. . metyrapone. Because this cortisol-lowering effect takes time (3 to 12 months). Adrenalectomy Surgical removal of the adrenal glands (adrenalectomy) is a final measure that may be recommended if other treatments are not successful. Adrenalectomy stops excess cortisol production but requires that you begin lifelong daily glucocorticoid and mineralocorticoid replacement therapy.
. The drug is contraindicated during pregnancy. -suppression of glucocorticoid synthesis .It is usually taken once a day.MEDICATION Metyrapone (Metopirone) . gynecomastia. . nausea. dizziness.Metyrapone is used to test if your pituitary gland is sending the proper Ketoconazole(Nizoral) .probably the most popular and effective of these agents for long-term use and usually is the agent of choice.SIDE EFFECTS: Nausea. . . sedation. .Adverse effects of ketoconazole include headache.Metyrapone 30mg/kg. impotence. irregular menses. signals to your adrenal glands. maximum dose 3000 mg. upset stomach. headache.blocks cortisol synthesis by inhibiting steroid 11β-hydroxylase. and elevated liver function tests. is administered at midnight usually with a snack. or drowsiness may occur. decreased libido.
or loss of appetite may occur. drowsiness. headache. .works by killing or slowing the growth of adrenal gland cells and also reverses the side effects caused by too much hormone production.Take this medication by mouth with or without food.Aminoglutethimide is indicated in conjunction with other drugs for the suppression ofadrenal function in patients with Cushing's syndrome. loss of appetite. nausea. usually 4 times a day (every 6 hours) or as directed by your doctor. Aminoglutethimide(Cytadren) . . .SIDE EFFECTS: Drowsiness.Mitotane (LYSODREN®) .Take this medication by mouth with or without food.SIDE EFFECTS: Dizziness. diarrhea.headache. vomiting. or unusual weakness may occur. usually 3 or 4 times daily or as directed by your doctor. dizziness. . .Tablets 500 mg . nausea.
In the LDDST. A modified LDDST uses a onetime overnight dose. If the cortisol level is more than 50 nanomoles per liter (nmol/L). a person is given a low dose of dexamethasone. but in Cushing’s syndrome. Cortisol production is normally suppressed at night. a synthetic glucocorticoid.Cortisol and other glucocorticoids signal the pituitary to release less ACTH.DIAGNOSTIC TESTS • 24-hour urinary free cortisol level. by mouth every 6 hours for 2 days. this suppression doesn’t occur. Levels higher than 50 to 100 micrograms a day for an adult suggest Cushing’s syndrome. a person’s urine is collected several times over a 24-hour period and tested for cortisol. • Midnight plasma cortisol and late-night salivary cortisol measurements. • Low-dose dexamethasone suppression test (LDDST). Cushing’s syndrome is suspected. Urine is collected before dexamethasone is administered and several times on each day of the test. In this test. The midnight plasma cortisol test measures cortisol concentrations in the blood. so the normal . The test generally requires a 48-hour hospital stay to avoid falsely elevated cortisol levels due to stress.
an injection of CRH causes the pituitary to secrete ACTH. Pseudo-Cushing’s does not have the same long-term effects on health as Cushing’s syndrome and does not require treatment directed at the endocrine glands. • Dexamethasone-corticotropin-releasing hormone (CRH) test. Pretreatment with dexamethasone prevents CRH from causing an increase in cortisol in people with pseudo-Cushing’s. In the CRH stimulation test. The dexamethasone-CRH test rapidly distinguishes pseudo-Cushing’s from mild cases of Cushing’s.response after taking dexamethasone is a drop in blood and urine cortisol levels. This test combines the LDDST and a CRH stimulation test. have poorly controlled diabetes. Some people have high cortisol levels but do not develop the progressive effects of Cushing’s syndrome. If cortisol levels do not drop. Cushing’s syndrome is suspected. These people may have pseudo-Cushing’s syndrome. such as muscle weakness. and thinning of the skin. Elevations of cortisol during this test suggest Cushing’s syndrome. or are severely obese. fractures. . drink excess alcohol. a condition sometimes found in people who have depression or anxiety disorders.
Collagen fibre weakness.Central obesity. Hirsutism I .Hypercortisolism.Striae. Hypertension.Iatrogenic (Increased administration of corticosteroids) N .Noniatrogenic (Neoplasms) G . Hyperglycaemia.MNEMONIC C . Comedones (acne) U .Glucose intolerance. Cervical fat pads.Urinary free cortisol and glucose increase S . Suppressed immunity H . Growth retardation .
REFERENCES http://emedicine. 12th ed. et.pp1281-1286 .com/article/117365-treatment http://www.Lippincott Williams and Wilkins.al.uptodate.2010.com/contents/patient-information-cushings-syndrometreatment Suzzane C.medscape.Medical-Surgical Nursing. Smeltzer.
Cushing’s syndrome And Addison’s disease .
Submitted by: Arnel John Marcera Eloisa Dajes Johayrah Macadato Zoren Kier Sabellina Joy Cheryl Pabololot Rolin Theresa Sabio Lorry Calisagan Submitted to: Ms.RN . Dolly Banluta.