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Metyrapone Is Useful Only as Adjunctive Therapy in Cushing's Disease

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Division of Endocrinology, Vanderbilt University School of Medicine; Nashville, Tennessee

Ann Intern Med. 1978;89(1):128-130. doi:10.7326/0003-4819-89-1-128
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A recent paper in the British Medical Journal (1) has recommended the use of metyrapone (Metopirone®; 2-methyl-1,2-bis(3-pyridyl)-1-propanone) for long-term treatment of patients with pituitary ACTH-dependent Cushing's syndrome (bilateral adrenal hyperplasia). Metyrapone inhibits the final step in adrenal steroid biosynthesis—the conversion of biologically inactive 11-deoxycortisol to cortisol (2). Metyrapone and aminoglutethimide (Elipten®; α-ethyl-α-p-aminophenylglutarimide), an agent that blocks the first step in steroid biosynthesis (3), have long been used to reduce hypercortisolism associated with adrenocortical tumors or ectopic ACTH syndrome (4-6); the only novelty in the British report lies in the use of such agents in Cushing's disease. What is the pathophysiology


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