Endocrinology and Metabolism

Case Reports

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; J Korean Endocr Soc. 2002;17(4):603-609. Published online August 1, 2002

Abstract PDF: 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.

A Case of Bilateral Macronodular Adrenal Hyperplasia with Cushing's Syndrome Treated by Unilateral Adrenalectomy.: Si Hoon Lee, Kwang Eun Lee, Eun Seok Kang, Sang Su Chung, Dae Jung Kim, Yun Mi Jin, Bong Soo Cha, Sung Kil Lim, Hyun Chul Lee, Kap Bum Huh; J Korean Endocr Soc. 2002;17(4):596-602. Published online August 1, 2002

Abstract PDF: In order to differentiate the causes of Cushing's syndrome; whether it is pituitary or adrenal-dependent, a high dose dexamethasone suppression test is usually performed but this does not always correlated with the imaging diagnosis. We report a case of bilateral macronodular adrenal hyperplasia, which was thought to be independent to adrenocorticotrophin (ACTH) stimulation. At first we thought it was pituitary-dependent Cushing's syndrome, due to its suppression by high dose dexamethasone. However, we found no abnormal findings on the brain sella magnetic resonance image (MRI). A significant finding, however, we found bilateral adrenal masses on the abdominal computed tomography (CT). We performed percutaneous selective adrenal venous sampling (PSAVS), and confirmed hypercortisolism of the left adrenal mass only. Therefore, we decided to remove the left adrenal gland to preserve the residual function of the right adrenal gland. After the left adrenalectomy, the patient became normotensive, and their buffalo hump disappeared, and her 24 hour urinary free cortisol level returned to normal.

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