Journal of Endocrinological Investigation

, Volume 18, Issue 7, pp 512–517 | Cite as

Glucocorticoid-remediable aldosteronism

  • Gordon H. Williams
  • R. G. Dluhy
Views Facts, perspectives and opinions on selected topics


Congenital Adrenal Hyperplasia Mineralocorticoid Primary Aldosteronism Chimeric Gene Aldosterone Secretion 
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  1. 1.
    Sutherland D.J., Ruse J.L., Laidlaw J.C. Hypertension, increased aldosterone secretion and low plasma renin activity relieved by dexamethasone. Can. Med. Assoc. J. 95: 1109, 1966.PubMedCentralPubMedGoogle Scholar
  2. 2.
    New M.I., Peterson R.E., et al. A new form of congenital adrenal hyperplasia. J. Clin. Endocrin. Metab. 27: 300, 1967.CrossRefGoogle Scholar
  3. 3.
    Fallo F., Mantero F. Dexamethasone-suppressible hyperaldosteronism. In: Biglieri E.G., Melby J.C. (Eds.), Endocrine Hypertension: Comprehensive Endocrinology, vol 57. Raven Press, New York 1990, p. 8797.Google Scholar
  4. 4.
    Williams G.H., Dluhy R.G. Diseases of the adrenal cortex. In: Isselbacher K. et al (Eds.). Harrison’s Principles of Internal Medicine, ed. 13. MacGraw-Hill Book Company, New York, 1994, p. 1953.Google Scholar
  5. 5.
    Rich G.M., Ulick S., Cook S., et al. Glucocorticoid-remediable aldosteronism in a large kindred: Clinical spectrum and diagnosis using a characteristics biochemical phenotype. Ann. Int. Med. 116: 813, 1992.PubMedCrossRefGoogle Scholar
  6. 6.
    Fallo F., Sonino N., Armanini D., et al. A new family with dexamethasone-suppressibie hyperaldosteronism: Aldosterone unresponsiveness to angiotensin II. Clin. Endocrinol. 22: 777, 1985.CrossRefGoogle Scholar
  7. 7.
    Grim C., Weinberger M., et al. Familial dexamethasone-suppressible normokaiemic hyperaldosteronism. Pediatrics 65: 597, 1980.PubMedGoogle Scholar
  8. 8.
    Chu M.D., Ulick S.J., et al. Isolation and identification of 18-hydroxycortisol from the urine of patients with primary aldosteronism. J. Biol. Chem. 257: 2218, 1982.PubMedGoogle Scholar
  9. 9.
    Ganguly A., Weinberg M., Guthrie G.P., et al. Adrenal steroid response to ACTH in glucocorticoid-suppressible hyperaldosteronism. Hypertension 6: 563, 1984.PubMedCrossRefGoogle Scholar
  10. 10.
    Giebink G.S., Gotlin R.W., Biglieri E.G., et al. A kindred with familial glucocorticoid-suppressible hyperaldosteronism. J. Clin. Endocrin. Metab. 36: 715, 1973.CrossRefGoogle Scholar
  11. 11.
    New M.I., Siegal E.J., Peterson R.E.: Dexamethasone-suppressible hyperaldosteronism. J Clin Endocrin Metab 37: 93–100, 1973CrossRefGoogle Scholar
  12. 12.
    Ganguly A., Grim C., Weinberger M. Anomalous postural response in glucocorticoid-suppressible hyperaldosteronism. N Engl J Med 305: 991–993, 1981PubMedCrossRefGoogle Scholar
  13. 13.
    Oberfield S.E., Levine L.S., Stoner E., et al. Adrenal glomerulosa function in patients with dexamethasone-suppressible hyperaldosteronism. J. Clin. Endocrinol. Metab. 53: 158, 1981.PubMedCrossRefGoogle Scholar
  14. 14.
    Woodland E., Tunny T.J., Hamlet S.M., et al. Hypertension corrected and aldosterone responsiveness to renin-angiotensin restored by long-term dexamethasone in glucocorticoid-suppressible hyperaldosteronism. Clin. Exp. Pharmacol. Physiol. 12: 245, 1985.PubMedCrossRefGoogle Scholar
  15. 15.
    Spence C.D., Coghlan J.P., Denton D.A., et al. Blood pressure and metabolic effects of 18-oxo-cortisol in sheep. J. Steroid. Biochem. 28: 441, 1987.PubMedCrossRefGoogle Scholar
  16. 16.
    Gomez-Sanchez C., Gomez-Sanchez E.P., Smith J.S. Receptor binding and biological activity of 18-oxocortisol. Endocrinology 116: 6, 1985.PubMedCrossRefGoogle Scholar
  17. 17.
    Chua S.C., Szabo P., Vitek A., et al. Cloning of cDNA encoding steroid 11-hydroxylase (P-450c11). Proc Natl Acad Sci USA 84: 7193, 1987.PubMedCentralPubMedCrossRefGoogle Scholar
  18. 18.
    Kawamoto T., Mitsuuchi Y., Ohnishi T. et al: Cloning and expression of a cDNA for human cytochrome P-450aldo as related to primary aldosteronism. Biochem. Biophys. Res. Commun. 173: 309, 1990.CrossRefGoogle Scholar
  19. 19.
    Mornet E., Dupont B., Vitek A., et al. Characterization of two genes encoding human steroid 11 hydroxylase (P-450c11). J. Biol.Chem. 264: 961, 1989.Google Scholar
  20. 20.
    Ogishima T., Shibata H., Shimada H., et al. Aldosterone synthase cytochrome P-450 expressed in the adrenals of patients with primary aldosteronism. J Biol. Chem. 266: 731, 1991.Google Scholar
  21. 21.
    Lifton R.P., Dluhy R.G., Powers M., et al. A chimeric 11-hydroxylase/aldosterone synthase gene causes glucocorticoid-remediable aldosteronism and human hypertension. Nature 355: 262, 1992.PubMedCrossRefGoogle Scholar
  22. 22.
    Lifton R.P., Dluhy R.G., Powers M., et al. Hereditary hypertension caused by chimaeric gene duplications and ectopic expression of aldosterone synthase. Nature Genet. 2: 66, 1992.PubMedCrossRefGoogle Scholar
  23. 23.
    Pascoe L., Curnow K.M., Slutsker L., et al. Glucocorticoid-suppressible hyperaldosteronism results from hybrid genes created by unequal crossovers between CYP11B1 and CYP11B2. Proc. Natl. Acad. Sci. USA 89: 8327, 1992.PubMedCentralPubMedCrossRefGoogle Scholar
  24. 24.
    Stockigt J.R., Scoggins B.A., et al. Long-term evolution of glucocorticoid-suppressible hyperaldosteronism. J. Clin. Endocrin. Metab. 64: 22, 1987.CrossRefGoogle Scholar

Copyright information

© Italian Society of Endocrinology (SIE) 1995

Authors and Affiliations

  • Gordon H. Williams
    • 1
  • R. G. Dluhy
    • 1
  1. 1.Harvard Medical SchoolBrigham and Women’s HospitalBoston

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