Primary generalized familial and sporadic glucocorticoid resistance (chrousos syndrome) and hypersensitivity

Evangelia Charmandari, Tomoshige Kino, George P. Chrousos

Research output: Chapter in Book/Report/Conference proceedingChapter

38 Citations (Scopus)

Abstract

Familial or sporadic primary generalized glucocorticoid resistance or Chrousos syndrome is a rare genetic condition characterized by generalized, partial, target-tissue insensitivity to glucocorticoids and a consequent hyperactivation of the hypothalamic-pituitary-adrenal (HPA) axis. Primary generalized glucocorticoid hypersensitivity (PGGH) represents the mirror image of the former, and is characterized by generalized, partial, target-tissue hypersensitivity to glucocorticoids, and compensatory hypoactivation of the HPA axis. The molecular basis of both conditions has been ascribed to mutations in the human glucocorticoid receptor (hGR) gene, which impair the molecular mechanisms of hGR action and alter tissue sensitivity to glucocorticoids. This review summarizes the pathophysiology, molecular mechanisms and clinical aspects of Chrousos syndrome and PGGH.

Original languageEnglish
Title of host publicationHormone Resistance and Hypersensitivity: From Genetics to Clinical Management
PublisherS. Karger AG
Pages67-85
Number of pages19
Volume24
ISBN (Electronic)9783318022681
ISBN (Print)9783318022674
DOIs
Publication statusPublished - 4 Feb 2013
Externally publishedYes

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Glucocorticoids
Hypersensitivity
Glucocorticoid Receptors
Tissue
Glucocorticoid Receptor Deficiency
Mirrors
Genes
Mutation

ASJC Scopus subject areas

  • Medicine(all)
  • Biochemistry, Genetics and Molecular Biology(all)

Cite this

Charmandari, E., Kino, T., & Chrousos, G. P. (2013). Primary generalized familial and sporadic glucocorticoid resistance (chrousos syndrome) and hypersensitivity. In Hormone Resistance and Hypersensitivity: From Genetics to Clinical Management (Vol. 24, pp. 67-85). S. Karger AG. https://doi.org/10.1159/000342505

Primary generalized familial and sporadic glucocorticoid resistance (chrousos syndrome) and hypersensitivity. / Charmandari, Evangelia; Kino, Tomoshige; Chrousos, George P.

Hormone Resistance and Hypersensitivity: From Genetics to Clinical Management. Vol. 24 S. Karger AG, 2013. p. 67-85.

Research output: Chapter in Book/Report/Conference proceedingChapter

Charmandari, E, Kino, T & Chrousos, GP 2013, Primary generalized familial and sporadic glucocorticoid resistance (chrousos syndrome) and hypersensitivity. in Hormone Resistance and Hypersensitivity: From Genetics to Clinical Management. vol. 24, S. Karger AG, pp. 67-85. https://doi.org/10.1159/000342505
Charmandari E, Kino T, Chrousos GP. Primary generalized familial and sporadic glucocorticoid resistance (chrousos syndrome) and hypersensitivity. In Hormone Resistance and Hypersensitivity: From Genetics to Clinical Management. Vol. 24. S. Karger AG. 2013. p. 67-85 https://doi.org/10.1159/000342505
Charmandari, Evangelia ; Kino, Tomoshige ; Chrousos, George P. / Primary generalized familial and sporadic glucocorticoid resistance (chrousos syndrome) and hypersensitivity. Hormone Resistance and Hypersensitivity: From Genetics to Clinical Management. Vol. 24 S. Karger AG, 2013. pp. 67-85
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