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KEY CONCEPTS

KEY CONCEPTS

  • Image not available. Glucocorticoid secretion from the adrenal cortex is stimulated by adrenocorticotropic hormone (ACTH) or corticotropin that is released from the anterior pituitary in response to the hypothalamic-mediated release of corticotropin-releasing hormone (CRH).

  • Image not available. To ensure the proper treatment of Cushing syndrome, diagnostic procedures should (1) establish the presence of hypercortisolism and (2) discover the underlying etiology of the disease.

  • Image not available. The rationale for treating Cushing syndrome is to reduce the morbidity and mortality resulting from disorders such as diabetes mellitus, cardiovascular disease, and electrolyte abnormalities.

  • Image not available. The treatment of choice for both ACTH-dependent and ACTH-independent Cushing syndrome is surgery. Pharmacologic agents are reserved for adjunctive therapy, refractory cases, or inoperable disease.

  • Image not available. Pharmacologic agents that may be used to manage the patient with Cushing syndrome include steroidogenesis inhibitors, adrenolytic agents, neuromodulators of ACTH release, and glucocorticoid-receptor blocking agents.

  • Image not available. Spironolactone, a competitive aldosterone-receptor antagonist, is the drug of choice in bilateral adrenal hyperplasia (BAH)–dependent hyperaldosteronism.

  • Image not available. Addison's disease (primary adrenal insufficiency) is a state of deficiency in cortisol, aldosterone, and various androgens due to the loss of function in all regions of the adrenal cortex.

  • Image not available. Secondary adrenal insufficiency usually results from exogenous steroid use, leading to hypothalamic–pituitary–adrenal (HPA)–axis suppression followed by a decrease in ACTH release, and low levels of androgens and cortisol.

  • Image not available. Virilism results from the excessive secretion of androgens from the adrenal gland and often manifests as hirsutism in females.

PATIENT CARE PROCESS

Patient Care Process for Cushing Syndrome

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Collect

  • Patient characteristics (eg, age, sex, pregnant)

  • Patient medical history (personal and family)

  • Social history (eg, tobacco/ethanol use)

  • Iatrogenic causes of Cushing syndrome: current medications including corticosteroids (all routes, past and present), medroxyprogesterone acetate, megestrol acetate, gamma-hydroxybutryic acid, CYP3A4 inhibitors and inducers

  • Objective data

    • Blood pressure, heart rate, body mass index

    • Test for hypercortisolism: 24-hour UFC, midnight plasma cortisol, late-night salivary cortisol, or low-dose dexamethasone suppression test

    • Follow-up diagnostic tests to differentiate etiologies (see Fig. 93-4)

Assess

  • Presence of Cushing syndrome complications:

    • Metabolic: impaired glucose metabolism, dyslipidemia

    • Cardiovascular: hypertension, vascular damage, thrombosis, hypokalemia

    • Immunologic: bacterial, fungal, and viral infections; rebound autoimmunity of Musculoskeletal: osteopenia/osteoporosis, myopathy

    • Neuropsychiatric: depression, anxiety, bipolar disorder

    • Reproductive: decreased libido, hypogonadism (men), menstrual irregularity (women)

    • Dermatologic: hirsutism, alopecia, hyperhidrosis

  • Physical exam: Peripheral obesity, fat accumulation (Buffalo Hump), rounded face (moon face), striae, ecchymosis, hyperpigmentation, acanthosis nigricans, acne, and thin skin

  • Current medications that may contribute to or worsen Cushing syndrome.

  • Results of follow-up diagnostic testing for etiology (see Fig. 93-4, and Tables 93-2 and 93-3)

  • Ability/willingness to pursue surgical/chemotherapeutic (if indicated) versus medical management

Plan*

  • Ensure proper administration of necessary corticosteroid therapy; discontinue unnecessary corticosteroid therapy, with taper if HPA axis integrity is suspect

  • Nondrug options for endogenous Cushing syndrome, depending on etiology: surgery, chemotherapy, irradiation, postoperative steroid replacement (see Table 93-4)

  • Steroid replacement regimens postoperatively for patients with adrenal adenomas (see Table 93-7)

  • Medical management when surgery is not possible or against patient wishes (see Tables 93-5 and 93-6 for ...

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