{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T10:03:37Z","timestamp":1790071417288,"version":"4.0.1"},"reference-count":19,"publisher":"The Endocrine Society","issue":"8","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2001,8,1]]},"abstract":"<jats:p>An extremely ill patient, with Cushing\u2019s syndrome caused by an ACTH-secreting pituitary macroadenoma, experienced complications of end-stage cardiomyopathy, profound psychosis, and multiple metabolic disturbances. Initially treated unsuccessfully by a combination of conventional surgical, medical, and radiotherapeutic approaches, he responded dramatically to high-dose long-term mifepristone therapy (up to 25 mg\/kg\u00b7d). Treatment efficacy was confirmed by the normalization of all biochemical glucocorticoid-sensitive measurements, as well as by the significant reversal of the patient\u2019s heart failure, the resolution of his psychotic depression, and the eventual unusual return of his adrenal axis to normal. His 18-month-long mifepristone treatment course was notable for development of severe hypokalemia that was attributed to excessive cortisol activation of the mineralocorticoid receptor, which responded to spironolactone administration. This case illustrates the efficacy of high-dose long-term treatment with mifepristone in refractory Cushing\u2019s syndrome. The case also demonstrates the potential need for concomitant mineralocorticoid receptor blockade in mifepristone-treated Cushing\u2019s disease, because cortisol levels may rise markedly, reflecting corticotroph disinhibition, to cause manifestations of mineralocorticoid excess.<\/jats:p>","DOI":"10.1210\/jcem.86.8.7740","type":"journal-article","created":{"date-parts":[[2014,1,8]],"date-time":"2014-01-08T17:07:29Z","timestamp":1389200849000},"page":"3568-3573","source":"Crossref","is-referenced-by-count":8,"title":["Successful Long-Term Treatment of Refractory Cushing\u2019s Disease with High-Dose Mifepristone (RU 486)"],"prefix":"10.1210","volume":"86","author":[{"given":"James W.","family":"Chu","sequence":"first","affiliation":[{"name":"Departments of Medicine (J.W.C., D.F.M., A.R.H., D.F.), Stanford, California 94305"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dwight F.","family":"Matthias","sequence":"additional","affiliation":[{"name":"Departments of Medicine (J.W.C., D.F.M., A.R.H., D.F.), Stanford, California 94305"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joseph","family":"Belanoff","sequence":"additional","affiliation":[{"name":"Psychiatry (J.B., A.S.), Stanford University School of Medicine, Stanford, California 94305"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alan","family":"Schatzberg","sequence":"additional","affiliation":[{"name":"Psychiatry (J.B., A.S.), Stanford University School of Medicine, Stanford, California 94305"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew R.","family":"Hoffman","sequence":"additional","affiliation":[{"name":"Departments of Medicine (J.W.C., D.F.M., A.R.H., D.F.), Stanford, California 94305"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Feldman","sequence":"additional","affiliation":[{"name":"Departments of Medicine (J.W.C., D.F.M., A.R.H., D.F.), Stanford, California 94305"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"80","reference":[{"key":"2020071617385338400_R1","first-page":"1848","article-title":"Diseases of the adrenal cortex","author":"Orth","year":"1998"},{"key":"2020071617385338400_R2","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1016\/S0889-8529(05)70064-5","article-title":"Medical therapy for Cushing\u2019s disease.","volume":"28","author":"Sonino","year":"1999","journal-title":"Endocrinol Metab Clin North Am"},{"key":"2020071617385338400_R3","doi-asserted-by":"crossref","first-page":"404","DOI":"10.1056\/NEJM199308053290607","article-title":"Mifepristone (RU 486)\u2014a modulator of progestin and glucocorticoid action.","volume":"329","author":"Spitz","year":"1993","journal-title":"N Engl J Med"},{"key":"2020071617385338400_R4","doi-asserted-by":"crossref","first-page":"191","DOI":"10.1016\/S0889-8529(05)70063-3","article-title":"Newer diagnostic techniques and problems in Cushing\u2019s disease.","volume":"28","author":"Findling","year":"1999","journal-title":"Endocrinol Metab Clin North Am"},{"key":"2020071617385338400_R5","doi-asserted-by":"crossref","first-page":"409","DOI":"10.1210\/edrv-7-4-409","article-title":"Ketoconazole and other imidazole derivatives as inhibitors of steroidogenesis.","volume":"7","author":"Feldman","year":"1986","journal-title":"Endocr Rev"},{"key":"2020071617385338400_R6"},{"key":"2020071617385338400_R7","doi-asserted-by":"crossref","first-page":"639","DOI":"10.1210\/jcem-63-3-639","article-title":"Pituitary-adrenal response to the antiglucocorticoid action of RU 486 in Cushing\u2019s syndrome.","volume":"63","author":"Bertagna","year":"1986","journal-title":"J Clin Endocrinol Metab"},{"key":"2020071617385338400_R8","doi-asserted-by":"crossref","first-page":"1040","DOI":"10.1001\/archinte.1983.00350050206041","article-title":"Cushing\u2019s disease in a patient with a\u2018 nonfunctioning\u2019 pituitary tumor. 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