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Vasopressin-Responsive ACTH-Independent Macronodular Adrenal Hyperplasia Causing Cushing's Syndrome

Yi-Yin Huang a, Hsin-Dean Chen b, Chen-Chung Fu b, Jer-Chuan Li b, Du-An Wu b

aDivision of Endocrinology and Metabolism, Department of Internal Medicine, Buddhist Tzu Chi General Hospital, Taichung Branch, Taichung, Taiwan
bDivision of Endocrinology and Metabolism, Department of Internal Medicine, Buddhist Tzu Chi General Hospital and Tzu Chi University, Hualien, Taiwan

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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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