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The Korean Journal of Medicine 2005;69(3): 867-872.
증례 : 바소프레신 의존성 거대결절성 부신피질 증식증 1예
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A case of ACTH-independent bilateral macronodular adrenal hyperplasia with response to stimulation of vasopressin
Jae Pil Han, Hyun Cheol Koo, Ick Keun Kim, In Il Park, Hyun Hee Choi, Seong Jin Lee, Moon Gi Choi
Department of Medicine, Sungkyunkwan University School of Medicine
Macronodular adrernal hyperplasia (MAH) is an extremely rare cause of ACTH-independent Cushing syndrome, in which cortisol secretion is autonomous and plasma ACTH level is persistently suppressed. The pathogenesis of MAH is not clear but recent studies suggest that cortisol secretion may be mediated by expression of ectopic or aberrant receptors for gastric inhibitory polypeptide, vasopressin, β-adrenergic agonist or serotonin. The conventional treatment is bilateral adrenalectomy followed by concomittent glucocorticoid and mineralocorticoid replacement therapy. We report a fifty-seven year-old man presenting with generalized weakness, weight gain and hematochezia. The endocrinological and radiological findings revealed ACTH-independent MAH, in which plasma cortisol level was abruptly elevated by administration of vasopressin 10 IU. Because he had severe dilated cardiomyopathy, we only prescribed high dose ketoconazole (1,200 mg/day) which suppresses biosynthesis and secretion of cortisol, and thereafter twenty-four hours urinary free cortisol level was dramatically decreased. In summary, these results strongly suggest that aberrant expression of vasopressin receptor may be associated with the pathophysiology of MAH.(Korean J Med 69:S867-S872, 2005)
Keywords: Cushing syndrome, Adrenal hyperplasia, Vasopressin
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Cushing Syndrome Caused by ACTH-independent Macronodular Adrenal Hyperplasia  2017 April;92(2)
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