Journal of Korean Endocrine Society 2002;17(4):603-609.
Published online August 1, 2002.
A Case of AVP Dependent Bilateral Macronodular Adrenal Hyperplasia.
Hyun Jin Kim, Se Hwa Kim, Yu Mie Rhee, Sung Eun Kim, Chul Woo Ahn, Bong Soo Cha, Kyung Rae Kim, Hyun Chul Lee, Kap Bum Huh, Sung Kil Lim
1Department of Internal Medicine, Yonsei University, College of Medicine, Seoul, Korea.
2Department of Pathology, Yonsei University, College of Medicine, Seoul, Korea.
Abstract
Adrenocorticotropin (ACTH) independent bilateral macronodular adrenal hyperplasia (AIMAH) is a rare form of Cushing's syndrome, in which unique endocrinological, clinical and histopathological features have been described. In AIMAH, cortisol secretion is autonomous and independent of ACTH, thus plasma ACTH levels are persistently suppressed. Various etiological mechanisms have been proposed to explain the development of AIMAH, the development of aberrant adrenal sensitivity to gastric inhibitory polypeptide (GIP), vasopressin, beta-adrenergic receptor agonists or the presence of circulating adrenal stimulating immunoglobulins have been suggested. We report on a 46-year-old female who had Cushing's syndrome, due to AIMAH, with a positive response to vasopressin.
Key Words: ACTH independent bilateral macronodular adrenal hyperplasia, Cushing's syndrome, AVP stimulation test


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