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Glucocorticoid Resistance

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Book cover Cushing's Syndrome

Part of the book series: Contemporary Endocrinology ((COE))

Summary

The syndrome of generalized glucocorticoid (GC) resistance is a familial disorder that is characterized by reduced cortisol effects, due to a GC receptor (GR) defect. This is compensated by increased activity of the hypothalamic-pituitary-adrenal (HPA)-axis, yielding elevated serum concentrations of cortisol, which does not result in the classical Cushingoid features related to cortisol overproduction. However, as a result of the increased levels of serum ACTH patients present with signs of adrenal overproduction of mineralocorticoids (hypertension and hypokalemic alkalosis) and, in females, of androgens (hirsutism, male pattern of baldness, menstrual irregularities). In some cases the underlying molecular basis has been elucidated, such as mutations in the gene coding for the GR, but in other patients the cause of GC resistance has not been revealed yet. We discuss the pathophysiology and provide tools for diagnosis and treatment of this rare hereditary syndrome.

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Authors and Affiliations

  1. Department of Internal Medicine, Erasmus MC University Medical Center, ’s-Gravendijkwal 230, 3015 CE, Rotterdam, The Netherlands

    Elisabeth F. C. van Rossum MD/PhD & Steven W. J. Lamberts MD/PhD

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  1. Elisabeth F. C. van Rossum MD/PhD
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  1. , Division of Endocrinology and Metabolism, Hospital das Clínicas, University of Sao, Sao Paulo, 05403-000, Brazil

    Marcello D. Bronstein

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van Rossum, E.F.C., Lamberts, S.W.J. (2010). Glucocorticoid Resistance. In: Bronstein, M. (eds) Cushing's Syndrome. Contemporary Endocrinology. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-60327-449-4_19

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