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INTRODUCTION

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  • Hyperfunction of the adrenal glands involves excess production of the adrenal hormones cortisol (resulting in Cushing syndrome) or aldosterone (resulting in hyperaldosteronism).

  • Adrenal gland hypofunction is associated with primary (Addison disease) or secondary adrenal insufficiency.

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CUSHING SYNDROME

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PATHOPHYSIOLOGY

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  • Cushing syndrome results from effects of supraphysiologic glucocorticoid levels originating from either exogenous administration or endogenous overproduction by the adrenal gland (adrenocorticotropic hormone [ACTH] dependent) or by abnormal adrenocortical tissues (ACTH independent).

  • ACTH-dependent Cushing syndrome (80% of all Cushing syndrome cases) is usually caused by overproduction of ACTH by the pituitary gland, causing adrenal hyperplasia. Pituitary adenomas account for about 85% of these cases (Cushing disease). Ectopic ACTH-secreting tumors and nonneoplastic corticotropin hypersecretion cause the remaining 20% of ACTH-dependent cases.

  • Ectopic ACTH syndrome refers to excessive ACTH production resulting from an endocrine or nonendocrine tumor, usually of the pancreas, thyroid, or lung (eg, small-cell lung cancer).

  • ACTH-independent Cushing syndrome is usually caused by adrenal adenomas and carcinomas.

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CLINICAL PRESENTATION

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  • The most common findings in Cushing syndrome are central obesity and facial rounding (90% of patients). Peripheral obesity and fat accumulation occur in 50% of patients. Fat accumulation in the dorsocervical area (buffalo hump) is nonspecific, but increased supraclavicular fat pads are more specific for Cushing syndrome. Patients are often described as having moon facies and a buffalo hump.

  • Other findings may include myopathy or muscular weakness, abdominal striae, hypertension, glucose intolerance, psychiatric changes, gonadal dysfunction, facial plethora (a reddish complexion), and amenorrhea and hirsutism in women.

  • Up to 60% of patients develop Cushing-induced osteoporosis; about 40% present with back pain, and 20% progress to spinal compression fractures.

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DIAGNOSIS

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  • Hypercortisolism can be established with one or more of the following tests: 24-hour urinary free cortisol (UFC), midnight plasma cortisol, late-night (11 PM) salivary cortisol, and/or low-dose dexamethasone suppression test (DST).

  • Other tests to determine etiology are plasma ACTH test; adrenal vein catheterization; metyrapone stimulation test; adrenal, chest, or abdominal computed tomography (CT); corticotropin-releasing hormone (CRH) stimulation test; inferior petrosal sinus sampling; and pituitary magnetic resonance imaging (MRI).

  • Adrenal nodules and masses are identified using high-resolution CT scanning or MRI.

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TREATMENT

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  • Goals of Treatment: Limit morbidity and mortality and return the patient to a normal functional state by removing the source of hypercortisolism while minimizing pituitary or adrenal deficiencies.

  • Treatment plans in Cushing syndrome based on etiology are included in Table 18–1.

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Table Graphic Jump Location
TABLE 18–1Treatment Options in Cushing Syndrome Based on Etiology

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