Tzu Chi Medical Journal
Volume 19, Issue 3 , Pages 164-168, September 2007

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

  • Yi-Yin Huang

      Affiliations

    • Division of Endocrinology and Metabolism, Department of Internal Medicine, Buddhist Tzu Chi General Hospital, Taichung Branch, Taichung, Taiwan
    • ,
  • Hsin-Dean Chen

      Affiliations

    • Division of Endocrinology and Metabolism, Department of Internal Medicine, Buddhist Tzu Chi General Hospital and Tzu Chi University, Hualien, Taiwan
    • ,
  • Chen-Chung Fu

      Affiliations

    • Division of Endocrinology and Metabolism, Department of Internal Medicine, Buddhist Tzu Chi General Hospital and Tzu Chi University, Hualien, Taiwan
    • ,
  • Jer-Chuan Li

      Affiliations

    • Division of Endocrinology and Metabolism, Department of Internal Medicine, Buddhist Tzu Chi General Hospital and Tzu Chi University, Hualien, Taiwan
    • ,
  • Du-An Wu

      Affiliations

    • Division of Endocrinology and Metabolism, Department of Internal Medicine, Buddhist Tzu Chi General Hospital and Tzu Chi University, Hualien, Taiwan
    • Corresponding Author InformationCorresponding author. Division of Endocrinology and Metabolism, Department of Internal Medicine, Buddhist Tzu Chi General Hospital, 707, Section 3, Chung Yang Road, Hualien, Taiwan

Received 13 December 2006; received in revised form 2 January 2007; accepted 13 March 2007.

Article Outline

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

Tzu Chi Medical Journal
Volume 19, Issue 3 , Pages 164-168, September 2007

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