Journal of Clinical Endocrinology & Metabolism, Vol 43, 1128-1133, Copyright © 1976 by Endocrine Society

ARTICLES

Spontaneous hypercortisolism without Cushing's syndrome

AC Vingerhoeds, JH Thijssen and F Schwarz

A very high cortisol production rate (CPR) with elevated plasma ACTH was found in a hypertensive, hypokalemic, but otherwise healthy male patient. There were no symptoms or signs of Cushing's syndrome. The hypercortisolism appeared to be of the pituitary dependent type. During the follow-up of 36 months, no changes in outward appearance occurred, notwithstanding persistent hypercortisolism.The possibility of either Conn's syndrome or of an enzymatic defect in steroidogenesis could be ruled out. One of the three children (a healthy boy of 20 years) also showed hypertension and hypercortisolism. A possibly genetically determined hyposensitivity to the glucocorticoid action of cortisol is postulated.

This article has been cited by other articles:

				E. Charmandari, T. Kino, T. Ichijo, and G. P. Chrousos Generalized Glucocorticoid Resistance: Clinical Aspects, Molecular Mechanisms, and Implications of a Rare Genetic Disorder J. Clin. Endocrinol. Metab., May 1, 2008; 93(5): 1563 - 1572. [Abstract] [Full Text] [PDF]

				H. Russcher, P. Smit, E. F. C. van Rossum, E. L. T. van den Akker, A. O. Brinkmann, L. J. M. de Heide, F. H. de Jong, J. W. Koper, and S. W. J. Lamberts Strategies for the Characterization of Disorders in Cortisol Sensitivity J. Clin. Endocrinol. Metab., February 1, 2006; 91(2): 694 - 701. [Abstract] [Full Text] [PDF]

				E R. DE KLOET and R. DERIJK Signaling Pathways in Brain Involved in Predisposition and Pathogenesis of Stress-Related Disease: Genetic and Kinetic Factors Affecting the MR/GR Balance Ann. N.Y. Acad. Sci., December 1, 2004; 1032(1): 14 - 34. [Abstract] [Full Text] [PDF]

				I. C. Chikanza and D. L. Kozaci Corticosteroid resistance in rheumatoid arthritis: molecular and cellular perspectives Rheumatology, November 1, 2004; 43(11): 1337 - 1345. [Full Text] [PDF]

				E. CHARMANDARI, T. KINO, and G. P. CHROUSOS Familial/Sporadic Glucocorticoid Resistance: Clinical Phenotype and Molecular Mechanisms Ann. N.Y. Acad. Sci., June 1, 2004; 1024(1): 168 - 181. [Abstract] [Full Text] [PDF]

				E. Charmandari, T. Kino, E. Souvatzoglou, A. Vottero, N. Bhattacharyya, and G. P. Chrousos Natural Glucocorticoid Receptor Mutants Causing Generalized Glucocorticoid Resistance: Molecular Genotype, Genetic Transmission, and Clinical Phenotype J. Clin. Endocrinol. Metab., April 1, 2004; 89(4): 1939 - 1949. [Abstract] [Full Text] [PDF]

				E. F.C. van Rossum, J. W. Koper, N. A.T.M. Huizenga, A. G. Uitterlinden, J. A.M.J.L. Janssen, A. O. Brinkmann, D. E. Grobbee, F. H. de Jong, C. M. van Duyn, H. A.P. Pols, et al. A Polymorphism in the Glucocorticoid Receptor Gene, Which Decreases Sensitivity to Glucocorticoids In Vivo, Is Associated With Low Insulin and Cholesterol Levels Diabetes, October 1, 2002; 51(10): 3128 - 3134. [Abstract] [Full Text] [PDF]

				T. KINO, A. VOTTERO, E. CHARMANDARI, and G. P. CHROUSOS Familial/Sporadic Glucocorticoid Resistance Syndrome and Hypertension Ann. N.Y. Acad. Sci., September 1, 2002; 970(1): 101 - 111. [Abstract] [Full Text] [PDF]

				M. R. Yudt and J. A. Cidlowski The Glucocorticoid Receptor: Coding a Diversity of Proteins and Responses through a Single Gene Mol. Endocrinol., August 1, 2002; 16(8): 1719 - 1726. [Abstract] [Full Text] [PDF]

				I. C. CHIKANZA Mechanisms of Corticosteroid Resistance in Rheumatoid Arthritis: A Putative Role for the Corticosteroid Receptor {beta} Isoform Ann. N.Y. Acad. Sci., June 1, 2002; 966(1): 39 - 48. [Abstract] [Full Text] [PDF]

				W Shamim, M Yousufuddin, D P Francis, P Gualdiero, J W Honour, S D Anker, and A J S Coats Raised urinary glucocorticoid and adrenal androgen precursors in the urine of young hypertensive patients: possible evidence for partial glucocorticoid resistance Heart, August 1, 2001; 86(2): 139 - 144. [Abstract] [Full Text] [PDF]

				N. A. T. M. Huizenga, P. de Lange, J. W. Koper, W. W. de Herder, R. Abs, J. H. L. M. v. Kasteren, F. H. de Jong, and S. W. J. Lamberts Five Patients with Biochemical and/or Clinical Generalized Glucocorticoid Resistance without Alterations in the Glucocorticoid Receptor Gene J. Clin. Endocrinol. Metab., May 1, 2000; 85(5): 2076 - 2081. [Abstract] [Full Text]

				Adrenal Hyperandrogenism in Children J. Clin. Endocrinol. Metab., December 1, 1999; 84(12): 4431 - 4435. [Full Text]

				New Resistance to Several Steroids in Two Sisters J. Clin. Endocrinol. Metab., December 1, 1999; 84(12): 4454 - 4464. [Abstract] [Full Text]

				M. Panarelli, C. D. Holloway, R. Fraser, J. M. C. Connell, M. C. Ingram, N. H. Anderson, and C. J. Kenyon Glucocorticoid Receptor Polymorphism, Skin Vasoconstriction, and Other Metabolic Intermediate Phenotypes in Normal Human Subjects J. Clin. Endocrinol. Metab., June 1, 1998; 83(6): 1846 - 1852. [Abstract] [Full Text]

				N. A. T. M. Huizenga, J. W. Koper, P. de Lange, H. A. P. Pols, R. P. Stolk, H. Burger, D. E. Grobbee, A. O. Brinkmann, F. H. de Jong, and S. W. J. Lamberts A Polymorphism in the Glucocorticoid Receptor Gene May Be Associated with an Increased Sensitivity to Glucocorticoids in Vivo J. Clin. Endocrinol. Metab., January 1, 1998; 83(1): 144 - 151. [Abstract] [Full Text]

				B. R. Walker, R. Best, C. H.L. Shackleton, P. L. Padfield, and C. R.W. Edwards Increased Vasoconstrictor Sensitivity to Glucocorticoids in Essential Hypertension Hypertension, February 1, 1996; 27(2): 190 - 196. [Abstract] [Full Text]

				B. Greenstein Steroid Resistance: Implications for Lupus Lupus, June 1, 1994; 3(3): 143 - 143. [PDF]