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Endocrinol Metab. 2011 Mar;26(1):1-11. English. Review. https://doi.org/10.3803/EnM.2011.26.1.1
Mermejo LM , Mazzuco TL , Grunenwald S , Fragoso MC , Bourdeau I , Lacroix A .
Division of Endocrinology, Department of Medicine, Centre de Recherche du Centre Hospitalier de l'Universite de Montreal (CRCHUM), Universite de Montreal, Quebec, H2W 1T8, Canada. andre.lacroix@umontreal.ca
Abstract

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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