- ACTH-Independent Macronodular Adrenal Hyperplasia.
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Livia M Mermejo, Tania L Mazzuco, Solange Grunenwald, Maria Candida B V Fragoso, Isabelle Bourdeau, Andre Lacroix
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Endocrinol Metab. 2011;26(1):1-11. Published online March 1, 2011
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DOI: https://doi.org/10.3803/EnM.2011.26.1.1
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 Abstract
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- ACTH-independent macronodular adrenal hyperplasia (AIMAH) is an uncommon cause of Cushing's syndrome (CS). The pathophysiology of this disorder is heterogeneous in its molecular origin and also in its clinical presentation. AIMAH can present mainly as an incidental radiological finding with sub-clinical CS or rarely with overt CS. In a few familial cases reported with AIMAH, specific aberrant G-protein coupled receptors were expressed in the adrenals of all affected members, but sporadic cases are more common. The aberrant adrenal function of G-protein coupled receptors can lead to cell proliferation and abnormal regulation of steroidogenesis. Unilateral or bilateral adrenalectomy has been the most frequently used treatment for this adrenal disorder; alternatively, the identification of aberrant receptors using in vivo protocol of investigation can offer specific pharmacological approach to control abnormal steroidogenesis and possibly prevent AIMAH progression.
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Citations
Citations to this article as recorded by  - Cushing Syndrome Caused by ACTH-independent Macronodular Adrenal Hyperplasia
Yeon-Kyung Choi, In-Ryang Hwang, Jung-Bum Seo, Ji Yun Jeong, Jung-Guk Kim
The Korean Journal of Medicine.2017; 92(2): 190. CrossRef - Primary bilateral adrenal nodular disease with Cushing's syndrome: varying aetiology
Kush Dev Singh Jarial, Rama Walia, Uma Nahar, Anil Bhansali
BMJ Case Reports.2017; : bcr-2017-220154. CrossRef - Multiple aberrant hormone receptors in Cushing's syndrome
Nada El Ghorayeb, Isabelle Bourdeau, André Lacroix
European Journal of Endocrinology.2015; 173(4): M45. CrossRef
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