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ARTICLE |

Primary Therapy for Cushing's Disease With Metyrapone FREE

Gabriel Dickstein, MD; Michal Lahav, PhD; Zila Shen-Orr, MS; Yehuda Edoute, MD; David Barzilai, MD
[+] Author Affiliations

Reprint requests to the Endocrine Institute, Haifa Medical Center (Rothschild), PO Box 4940, 31048 Haifa, Israel (Dr Dickstein).


JAMA. 1986;255(9):1167-1169. doi:10.1001/jama.1986.03370090089027
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A 13-year-old boy was diagnosed as suffering from pituitary-dependent Cushing's syndrome. He was treated with 2.0 g of metyrapone daily as the sole treatment for four years. All clinical and biochemical stigmata of Cushing's disease disappeared within a few months. The patient grew 23.0 cm in four years and regained normal health. No significant side effects of metyrapone were noticed. Administering the medication at 2 PM and 8 PM allowed higher cortisol levels in the morning and noon hours than in the evening and night, approximating the normal diurnal variation in cortisol production. We conclude that metyrapone may be considered the sole treatment in patients with Cushing's disease.

(JAMA 1986;255:1167-1169)

REFERENCES

Orth DN:  The old and the new in Cushing's syndrome . N Engl J Med 1984;;310:649-651.
Liddle GW, Island D, Lance EM, et al:  Alterations of adrenal steroid patterns in man resulting from treatment with a chemical inhibitor of 11 β-hydroxylation . J Clin Endocrinol Metabol 1958;;18:906-917.
Jeffcoate WJ, Rees LH, Tomlin S, et al:  Metyrapone in long-term management of Cushing's disease . Br Med J 1977;;2:215-217.
Orth DN:  Results of treatment in Cushing's syndrome , in Binder C, Hall P (eds): Cushing's Syndrome: Diagnosis and Treatment . London, Heinemann Medical Books Ltd, 1972;, p 148.
Beardwell CG, Adamson AR, Shalet SM:  Prolonged remission in florid Cushing's syndrome following metyrapone treatment . Clin Endocrinol 1981;;14:485-497.
Orth DN:  Metyrapone is useful only as adjunctive therapy in Cushing's disease . Ann Intern Med 1978;;89:128-130.
Spark RF:  Simplified assessment of pituitary-adrenal reserve: Measurement of serum 11-deoxycortisol and cortisol after metyrapone . Ann Intern Med 1971;;75:717-723.
Pavlatos FC, Smilo RP, Forsham PH:  A rapid screening test for Cushing's syndrome . JAMA 1965;;193:720-723.
Liddle GW:  Tests of pituitary adrenal suppressibility in the diagnosis of Cushing's syndrome . J Clin Endocrinol Metabol 1960;;20:1539-1560.
Greulich WW, Pyle SI: Radiographic Atlas of Skeletal Development of the Hand and Wrist , ed 2. Stanford (Calif) University Press, 1959;.
Caballiera AL, Fishman M, Jacobin JD:  Dual sites of inhibition by metyrapone of human adrenal steroidogenesis: Correlation of in vivo and in vitro studies . J Clin Endocrinol Metabol 1976;;42:687-695.
Salassa RM, Laws ER Jr, Carpenter PC, et al:  Transsphenoidal removal of pituitary microadenoma in Cushing's disease . Mayo Clin Proc 1978;;53:24-28.
Hardy J:  Cushing's disease: Fifty years later . Can J Neurol Sci 1982;;9:375-380.
Styne DM, Grumbach MM, Kaplan SL, et al:  Treatment of Cushing's disease in childhood and adolescence by transsphenoidal microadenectomy . N Engl J Med 1984;;310:889-893.
Burch W:  A survey of results with transsphenoidal surgery in Cushing's disease . N Engl J Med 1983;;308:103-104.
Daughaday WH:  Cushing's disease and basophilic microadenomas . N Engl J Med 1984;; 310:919-920.

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Orth DN:  The old and the new in Cushing's syndrome . N Engl J Med 1984;;310:649-651.
Liddle GW, Island D, Lance EM, et al:  Alterations of adrenal steroid patterns in man resulting from treatment with a chemical inhibitor of 11 β-hydroxylation . J Clin Endocrinol Metabol 1958;;18:906-917.
Jeffcoate WJ, Rees LH, Tomlin S, et al:  Metyrapone in long-term management of Cushing's disease . Br Med J 1977;;2:215-217.
Orth DN:  Results of treatment in Cushing's syndrome , in Binder C, Hall P (eds): Cushing's Syndrome: Diagnosis and Treatment . London, Heinemann Medical Books Ltd, 1972;, p 148.
Beardwell CG, Adamson AR, Shalet SM:  Prolonged remission in florid Cushing's syndrome following metyrapone treatment . Clin Endocrinol 1981;;14:485-497.
Orth DN:  Metyrapone is useful only as adjunctive therapy in Cushing's disease . Ann Intern Med 1978;;89:128-130.
Spark RF:  Simplified assessment of pituitary-adrenal reserve: Measurement of serum 11-deoxycortisol and cortisol after metyrapone . Ann Intern Med 1971;;75:717-723.
Pavlatos FC, Smilo RP, Forsham PH:  A rapid screening test for Cushing's syndrome . JAMA 1965;;193:720-723.
Liddle GW:  Tests of pituitary adrenal suppressibility in the diagnosis of Cushing's syndrome . J Clin Endocrinol Metabol 1960;;20:1539-1560.
Greulich WW, Pyle SI: Radiographic Atlas of Skeletal Development of the Hand and Wrist , ed 2. Stanford (Calif) University Press, 1959;.
Caballiera AL, Fishman M, Jacobin JD:  Dual sites of inhibition by metyrapone of human adrenal steroidogenesis: Correlation of in vivo and in vitro studies . J Clin Endocrinol Metabol 1976;;42:687-695.
Salassa RM, Laws ER Jr, Carpenter PC, et al:  Transsphenoidal removal of pituitary microadenoma in Cushing's disease . Mayo Clin Proc 1978;;53:24-28.
Hardy J:  Cushing's disease: Fifty years later . Can J Neurol Sci 1982;;9:375-380.
Styne DM, Grumbach MM, Kaplan SL, et al:  Treatment of Cushing's disease in childhood and adolescence by transsphenoidal microadenectomy . N Engl J Med 1984;;310:889-893.
Burch W:  A survey of results with transsphenoidal surgery in Cushing's disease . N Engl J Med 1983;;308:103-104.
Daughaday WH:  Cushing's disease and basophilic microadenomas . N Engl J Med 1984;; 310:919-920.
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