KMID : 0356620110260010001
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Journal of Korean Society of Endocrinology 2011 Volume.26 No. 1 p.1 ~ p.11
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ACTH-Independent Macronodular Adrenal Hyperplasia
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Mermejo Livia M.
Mazzuco Tania L. Grunenwald Solange Fragoso Maria Candida B. V. Bourdeau Isabelle
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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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KEYWORD
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ACTH-independent macronodular adrenal hyperplasia (AIMAH), Aberrant adrenal G-protein coupled receptors, Cushing¡¯s syndrome, Familial forms
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