Chrousos syndrome: a seminal report, a phylogenetic enigma and the clinical implications of glucocorticoid signalling changes.

European Journal of Clinical Investigation
Evangelia Charmandari, Tomoshige Kino

Abstract

Glucocorticoids regulate a broad spectrum of physiologic functions and play important roles in resting and stress homeostasis. Their actions are mediated by the nuclear glucocorticoid receptor (GR). Using a patient as a stimulus, we reviewed briefly the area of Primary Generalized Glucocorticoid Resistance in man and nonhuman primates. In man, Primary Generalized Glucocorticoid Resistance is a rare sporadic or familial syndrome characterized by target-tissue insensitivity to glucocorticoids and compensatory elevations in adrenocorticotropic hormone (ACTH), leading to increased secretion of cortisol and adrenal steroids with mineralocorticoid and/or androgenic activity, and causing hypermineralocorticoidism and hyperandrogenism without Cushing stigmata. The presentation, diagnosis and therapy of this condition are summarized. Many or, most likely, all New World primates have markedly elevated cortisol and ACTH, and resistance to dexamethasone suppression, without any pathology. These primates in fact have 'pan-steroid/sterol' resistance, including all five steroid hormones and 1,25-dihydroxy-vitamin D. In humans, the molecular basis of Primary Generalized Glucocorticoid Resistance has been mainly ascribed to recent mutations in ...Continue Reading

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Citations

Nov 1, 2012·Science Signaling·Evangelia CharmandariGeorge P Chrousos
Mar 31, 2011·World Journal of Gastroenterology : WJG·Sara De IudicibusGiuliana Decorti
Dec 7, 2013·World Journal of Gastroenterology : WJG·Sara De IudicibusGiuliana Decorti
Feb 8, 2014·Lancet·Evangelia CharmandariGeorge P Chrousos
Apr 15, 2014·Arquivos brasileiros de endocrinologia e metabologia·Manoel Carlos L A SouzaMargaret de Castro
Jul 15, 2018·European Journal of Endocrinology·Nicolas C Nicolaides, George P Chrousos
May 7, 2019·Frontiers in Psychiatry·Zoe PapadopoulouMaria Syrrou
Jun 1, 2011·European Journal of Clinical Investigation·Kursad UnluhizarciFahrettin Kelestimur
Jan 2, 2019·Clinical Diabetes and Endocrinology·Ved V GossainBhavini Bhavsar
Feb 27, 2015·European Journal of Clinical Investigation·Nicolas C Nicolaides, Evangelia Charmandari
Feb 12, 2020·Endocrine Practice : Official Journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists·Lifen MaTao Chen
Nov 7, 2015·European Journal of Clinical Investigation·Nicolas C NicolaidesEvangelia Charmandari

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