PMID: 18209860Jan 23, 2008Paper

Cushing's syndrome secondary to ACTH-independent macronodular adrenal hyperplasia

Arquivos brasileiros de endocrinologia e metabologia
Marcia Helena Soares Costa, A Lacroix

Abstract

ACTH-Independent macronodular adrenal hyperplasia (AIMAH) is a rare cause of endogenous Cushing's syndrome (CS), in which clinical features usually become apparent only after several decades of life. This form of adrenal hyperplasia typically produces excess cortisol with overt or subclinical CS, but concurrent secretion of mineralocorticoids or sexual steroids can also occur. The diagnosis is suspected by bilateral adrenal nodules larger than 1 cm on incidental imaging studies or following the demonstration of ACTH-independent hormonal hypersecretion. The pathophysiology of this entity is heterogeneous and has been intensely explored in recent years. Several G-protein coupled receptors aberrantly expressed in the adrenal cortex have been implicated in the regulation of steroidogenesis and in the initial cell proliferation in AIMAH. Several familial cases of AIMAH have been recently described with the same pattern of aberrant hormone receptors in all affected members of the family. It is probable that additional somatic genetic events related to cell cycle regulation, adhesion and transcription factors occur in addition over time in the various nodules; other mechanisms, as Gsp or ACTH receptor mutations and paracrine adrenal h...Continue Reading

References

Oct 1, 1992·The New England Journal of Medicine·A LacroixR Taillefer
Apr 1, 1989·The Journal of Clinical Endocrinology and Metabolism·C D MalchoffD N Orth
Feb 1, 1988·Radiology·J L DoppmanD L Loriaux
Jun 1, 1971·The Journal of Clinical Investigation·I Schorr, R L Ney
Jan 1, 1984·The Journal of Clinical Endocrinology and Metabolism·A G SmalsP W Kloppenborg
Mar 8, 1996·The Journal of Biological Chemistry·L J SliekerT W Stephens
Apr 1, 1996·Clinical Endocrinology·S MinamiI Wakabayashi
Jun 1, 1996·The Journal of Clinical Endocrinology and Metabolism·J E PabonC V Rao
Nov 14, 1997·The New England Journal of Medicine·A LacroixP Hamet
Jul 2, 1998·The New England Journal of Medicine·H S WillenbergS R Bornstein
Aug 7, 1998·Hormone and Metabolic Research = Hormon- Und Stoffwechselforschung = Hormones Et Métabolisme·C A Stratakis, L S Kirschner
May 20, 1999·Hormone and Metabolic Research = Hormon- Und Stoffwechselforschung = Hormones Et Métabolisme·A GlasowW A Scherbaum
Nov 24, 1999·The New England Journal of Medicine·A LacroixJ M Boutin
Apr 12, 2002·Endocrine Reviews·Mario AscoliDeborah L Segaloff
Oct 10, 2002·Experimental and Clinical Endocrinology & Diabetes : Official Journal, German Society of Endocrinology [and] German Diabetes Association·C NiesM Rothmund
Dec 6, 2002·The Journal of Clinical Endocrinology and Metabolism·Tomoatsu MuneKeigo Yasuda
Jan 10, 2003·The Journal of Clinical Endocrinology and Metabolism·Mark O GoodarziAndre J Van Herle
May 3, 2003·The Journal of Clinical Endocrinology and Metabolism·Maria Candida Barisson Villares FragosoBerenice Bilharinho Mendonca
Jul 5, 2003·The Journal of Clinical Endocrinology and Metabolism·Herve LefebvreJean-Marc Kuhn
Oct 15, 2003·The Journal of Clinical Endocrinology and Metabolism·Massimo MannelliAlessandro Peri
Feb 7, 2004·The Journal of Clinical Endocrinology and Metabolism·M A AbdallahCh V Rao
Mar 18, 2004·Radiographics : a Review Publication of the Radiological Society of North America, Inc·Andrea G RockallRodney H Reznek
Jul 28, 2004·The Journal of Steroid Biochemistry and Molecular Biology·S R AntoniniA Lacroix

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Citations

Jul 7, 2009·Arquivos brasileiros de endocrinologia e metabologia·Marcia Helena Soares CostaMaria Candida Barisson Villares Fragoso
Jun 6, 2009·Current Opinion in Endocrinology, Diabetes, and Obesity·Ty B Carroll, James W Findling
Apr 25, 2009·Current Opinion in Endocrinology, Diabetes, and Obesity·Tânia Longo MazzucoAndré Lacroix
Oct 27, 2020·Journal of Family Medicine and Primary Care·Yashendu SardaSanyam Katyal
Jan 26, 2010·Trends in Endocrinology and Metabolism : TEM·Guillaume AssiéEric Clauser

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