Vasopressin-Responsive ACTH-Independent Macronodular Adrenal Hyperplasia Causing Cushing's Syndrome
Abstract
ACTH-independent macronodular adrenal hyperplasia (AIMAH) is a rare cause of endogenous Cushing's syndrome (CS). The diagnosis is suggested by bilateral adrenal nodular enlargement on conventional imaging and the demonstration of ACTH-independent hypercortisolism. Cortisol secretion in AIMAH can be regulated by the aberrant adrenal expression of receptors for gastric inhibitory polypeptide, vasopressin, catecholamines, luteinizing hormone/human chorionic gonadotropin and serotonin. We report on a 47-year-old man with CS due to AIMAH. The cortisol levels were increased after vasopressin stimulation, suggesting aberrant expression of vaso-pressin receptor in the adrenal glands. Total adrenalectomy is considered a standard therapy for AIMAH.
Keywords: Aberrant receptor , AIMAH , Cushing's syndrome
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PII: S1016-3190(10)60010-9
doi:10.1016/S1016-3190(10)60010-9
© 2007 Buddhist Compassion Relief Tzu Chi Foundation. Published by Elsevier Inc. All rights reserved.
