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ea0081p15 | Adrenal and Cardiovascular Endocrinology | ECE2022

Decreased steroidogenic enzymes activity in benign adrenocortical tumors is more pronounced in bilateral lesions as determined by steroid profiling in HPLC-MSMS during ACTH stimulation test

Fideline Bonnet , Barat Maxime , Vaczlavik Anna , Jouinot Anne , Bouys Lucas , Laguillier-Morizot Christelle , Zientek Corinne , See Catherine , Larger Etienne , Guignat Laurence , Groussin Lionel , Assie Guillaume , Guibourdenche Jean , Nicolis Ioannis , Menet Marie-Claude , Bertherat Jerome

Objective: Large response of steroids precursors, including 17-hydroxyprogesterone and 11-deoxycortisol, to ACTH has been described in adrenocortical tumors, suggesting the existence of intra-tumoral enzymatic deficiencies. This study aimed to compare steroidogenic enzymes activity in unilateral and bilateral benign tumors using serum steroid profiling in HPLC-MS/MS in basal state and after ACTH 1-24 stimulation.Design and Methods: A serum profile of sev...

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ea0081yi6 | Young Investigator Awards | ECE2022

Integrated genomics reveals the molecular classification of Primary Bilateral Macronodular Adrenal Hyperplasia (PBMAH), correlating with specific profiles of illegitimate receptors expression and identifies KDM1A as the genetic cause of food-dependent Cushing syndrome

Bouys Lucas , Violon Florian , Vaczlavik Anna , Gaetan Giannone , Jouinot Anne , Armignacco Roberta , Cavalcante Isadora Pontes , Berthon Annabel , Letouze Eric , Vaduva Patricia , Barat Maxime , Fideline Bonnet , Perlemoine Karine , Ribes Christopher , Sibony Mathilde , North Marie-Odile , Espiard Stephanie , Haissaguerre Magalie , TAUVERON Igor , Guignat Laurence , Groussin Lionel , Dousset Bertrand , Reincke Martin , Barisson Villares Fragoso Maria Candida , Stratakis Constantine A , Pasmant Eric , Libe Rossella , Assie Guillaume , Ragazzon Bruno , Bertherat Jerome

Introduction: In Primary Bilateral Macronodular Adrenal Hyperplasia (PBMAH), cortisol secretion may be consecutive to physiological stimuli, through the illegitimate expression of G-protein coupled receptors (GPCR) in adrenocortical cells. The most characterized is the overexpression of GIP receptor (GIPR) leading to food-dependent Cushing syndrome (FDCS) but it has not been associated with the consecutive inactivation of ARMC5 responsible for 25% of PBMAH. This work aimed to ...

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Endocrine Abstracts

Decreased steroidogenic enzymes activity in benign adrenocortical tumors is more pronounced in bilateral lesions as determined by steroid profiling in HPLC-MSMS during ACTH stimulation test

Integrated genomics reveals the molecular classification of Primary Bilateral Macronodular Adrenal Hyperplasia (PBMAH), correlating with specific profiles of illegitimate receptors expression and identifies KDM1A as the genetic cause of food-dependent Cushing syndrome

BiosciAbstracts