›› 2012, Vol. 32 ›› Issue (3): 366-.doi: 10.3969/j.issn.1674-8115.2012.03.028

• 综述 • 上一篇    下一篇


朱丽颖, 宁 光   

  1. 上海交通大学 医学院附属瑞金医院内分泌代谢病科, 上海 200025
  • 出版日期:2012-03-28 发布日期:2012-03-28
  • 通讯作者: 宁 光, 电子信箱: guangning@medmail.com.cn。
  • 作者简介:朱丽颖(1984—), 女, 硕士生;电子信箱: tianshi_baba@163.com。

Research progress of adrenocorticotropic hormone-independent macronodular adrenal hyperplasia

ZHU Li-ying, NING Guang   

  1. Department of Endocrine and Metabolic Diseases, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai 200025, China
  • Online:2012-03-28 Published:2012-03-28



关键词: 促肾上腺皮质激素非依赖性肾上腺大结节样增生, 库欣综合征, 异常受体, 肾上腺切除


Adrenocorticotropic hormoneindependent macronodular adrenal hyperplasia (AIMAH) is a rare adrenal pathological lesion. AIMAH presents clinically as overt or subclinical Cushing's syndrome with typical radiological findings, occasionally with other diseases. What is rarer is the familial type. The mechanism of AIMAH may be the aberrant expression of several hormones and cytokines receptors in adrenal, which leads to the oversecretion of glucocorticoid, and the molecular mechanism involves genic mutation or deficiency of the receptors. Although presently adrenalectomy is the main treatment, with further research on pathophysiology,more and more effective medication will be used. The research progress of clinical features, mechanism, and treatment of AIMAH is reviewed in this paper.

Key words: adrenocorticotropic hormone-independent macronodular adrenal hyperplasia, Cushing´s syndrome, aberrant receptor, adrenalectomy