Cushing's disease is defined as hypercortisolism due to chronic overproduction of corticotrophin by a corti-cotroph adenoma. Cortisol's lack of suppressibility during the administration of low doses of dexamethasone but suppressibility during high-dose dexamethasone is the key diagnostic finding in 99% of the patients with Cushing's disease. This contrasts with the lack of gluco-corticoid suppressibility typically found in patients with corticotrophin-independent hypercortisolism (Cushing's syndrome). A judicious selection of the available tests may be necessary to obtain an accurate diagnosis in patients with Cushing's syndrome.
1. During the period of withdrawal from extended glucocorticoid therapy
(A) Prompt recovery of the hypothalamic-pituitary-adrenal axis results in restoration of endogenous corticotrophin release.
(B) The patient may be eager to further reduce the dose of glucocorticoid.
(C) The physician should rapidly reduce glucocor-ticoid therapy to physiological doses.
(D) Patients should not require an increment in steroid therapy during increased stress (e.g., severe infection).
(E) The appearance of fever and malaise attributed to steroid withdrawal may be difficult to distinguish from reactivation of rheumatic disease.
2. Which one of the following enzymes is required for cortisol biosynthesis?
(D) 11-p-hydroxysteroid dehydrogenase-2
3. The primary goal of glucocorticoid treatment in rheumatic arthritis is
(A) Suppression of inflammation and improvement in functional capacity
(B) Eradication of all symptoms
(C) Reversal of the degenerative process
(D) Development of a sense of well-being in the patient
(E) Prevention of suppression of the hypothalamic-pituitary-adrenal axis
4. The addition of a fluoride group on ring C of cortisol to give 9-a-fluorocortisol
(A) Will shorten its half-life
(B) Will increase both glucocorticoid and miner-alocorticoid activity
(C) Shares an advantage over cortisol in that sodium retention is not as marked at equipotent inflammatory doses
(D) Will not cause suppression of the hypothalamic-pituitary-adrenal axis when applied topically.
(E) Provides a steroid widely used in the treatment of rheumatoid arthritis
(A) Is adequate replacement therapy in an adrena-lectomized patient
(B) Has a half-life equivalent to that of cortisol
(C) Produces salt retention in therapeutic doses
(D) Possesses most of the undesirable side effects of cortisol
(E) Has antiinflammatory potency equivalent to that of cortisol
6. Which answer is most appropriate for the action of ketoconazole?
(A) It has a single major action that is confined to the adrenal cortex.
(B) It provides long term treatment for Cushing's disease.
(C) It has an action on the adrenal cortex that is irreversible.
(D) Its action may be associated with liver dysfunction.
(E) It preferentially blocks cortisol synthesis as opposed to testosterone production.
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