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Evidence for Cortisol as the mineralocorticoid in the syndrome of apparent mineralocorticoid excess

Journal of Endocrinological Investigation volume 15, pages 471–474 (1992)Cite this article

Abstract

The hypothesis that Cortisol is the functioning mineralocorticoid in the syndrome of apparent mineralocorticoid excess was tested by suppressing its secretion with dexamethasone. The subjects were two siblings with the type 2 form of this syndrome in which the defect in the peripheral metabolism of Cortisol lies predominantly in ring A reduction but not in 11β-hydroxy dehydrogenation of Cortisol to cortisone. Low dosage dexamethasone improved the hypokalemia within several days and hypertension was corrected after 3 weeks of treatment. Mineralocorticoid manifestations remained in remission during 10 yr of therapy with the synthetic glucocorticoid during which normal growth and development were restored. The effectiveness of dexamethasone supports the hypothesis that Cortisol is the functioning mineralocorticoid in the AME syndrome.

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References

  1. Ulick S., Levine LS., Gunczler P., Zanconato G., Ramirez L.C., Rauh W., Rosier A., Bradlow L, New M.I. A syndrome of apparent mineralocorticoid excess associated with defects in the peripheral metabolism of Cortisol. J. Clin. Endocrinol. Metab. 49: 757, 1979.

    CAS  PubMed  Article  Google Scholar 

  2. Stewart P.M., Corrie J.E.T., Shackleton C.H.L., Edwards C.R.W. Syndrome of apparent mineralocorticoid excess. J. Clin. Invest. 82: 340, 1988.

    CAS  PubMed Central  PubMed  Article  Google Scholar 

  3. Ulick S., Tedde R., Mantero F. Pathogenesis of the type 2 variant of the syndrome of apparent mineralocorticoid excess. J. Clin. Endocrinol. Metab. 70: 200, 1990.

    CAS  PubMed  Article  Google Scholar 

  4. Edwards C.R.W., Stewart P.M., Burt D., Brett L, Mclntyre M.A., Sutanto W.S., De Kloet E.R., Monder C. Localization of 11β-hydroxysteroid dehydrogenase-tissue specific protector of the mineralocorticoid receptor. Lancet 2: 986, 1988.

    CAS  PubMed  Article  Google Scholar 

  5. Funder J.W., Pearce P.T., Smith R., Smith A.I. Mineralocorticoid action: target tissue specificity is enzyme, not receptor, mediated. Science 242: 583, 1988.

    CAS  PubMed  Article  Google Scholar 

  6. Arriza J.L., Weinberger C., Cerelli G., Glaser T.M., Handelin B.L., Housman D.E., Evans R.M. Cloning of human mineralocorticoid receptor complementary DNA: structural and functional kinship with the glucocorticoid receptor. Science 237: 268, 1987.

    CAS  PubMed  Article  Google Scholar 

  7. Ulick S., Tedde R., Wang J.Z. Defective ring A reduction of Cortisol as the major metabolic in the syndrome of apparent mineralocorticoid excess. J. Clin. Endocrinol. Metab. 74: 593, 1992.

    CAS  PubMed  Google Scholar 

  8. Oberfield S.E., Levine L.S., Carey R.M., Greig F., Ulick S., New M.I. Metabolic and blood pressure responses to hydrocortisone in the syndrome of apparent mineralocorticoid excess. J. Clin. Endocrinol. Metab. 56: 332, 1983.

    CAS  PubMed  Article  Google Scholar 

  9. Sann I., Revol A., Zachmann M., Legrand J.C., Bethenod M. Unusual low plasma renin hypertension in a child. J. Clin. Endocrinol. Metab. 43: 265, 1975.

    Article  Google Scholar 

  10. Fiselier T.J.W., Often B.J., Monnens L.A.H., Honour J.W., Van Munster P.J.J. Low-renin, low-aldosterone hypertension and abnormal Cortisol metabolism in a 19 month old child. Hormone Res. 16: 107, 1982.

    CAS  PubMed  Article  Google Scholar 

  11. Honour J.W., Dillon M.J., Levin M., Shah V. Fatal, low renin hypertension associated with a disturbance of Cortisol metabolism. Arch. Dis. Childhood 58: 1018, 1983.

    CAS  Article  Google Scholar 

  12. Harinck H.I.G., Van Brummelen P., Van Seters A.P., Moolenaar A.J. Apparent mineralocorticoid excess and deficient 11β-oxidation of Cortisol in a young female. Clin. Endocrinol. 21: 505, 1984.

    CAS  Article  Google Scholar 

  13. Tedde R., Sechi L.A., Marigliano A., Pala A., Scano L, Melis A. Low renin, low aldosterone hypertension: ten years of therapy with dexamethasone (Abstract). 12th Scientific Meet, of the Int. Soc. of Hypertension, 1988.

    Google Scholar 

  14. Mantero F., Tedde R., Scaroni C., Opocher G., Campus S. Dexamethasone-suppressible low renin-low aldosterone hypertension: report of 3 cases. In: Mantero F., Biglieri E.G., Funder J.W., Scoggins B.A., (eds.), The Adrenal Gland and Hypertension. Raven Press, New York, 1985, p. 337.

    Google Scholar 

  15. Ulick S., Chan CK., Rao K.N., Edassery J., Mantero F. A new form of the syndrome of apparent mineralo corticoid excess. J. Steroid. Biochem. 32: 209, 1989.

    CAS  PubMed  Article  Google Scholar 

  16. Downey M.K., New M.I. Apparent mineralocorticoid excess: a genetic form of fatal low-renin hypertension. Abstract. Amer. Soc. Hyper. New York, NY, May 1987.

    Google Scholar 

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Affiliations

  1. Clinica Medica, Università di Sassari Sassari, Italy

    R. Tedde, A. Pala & A. Melis

  2. Veterans Affairs Hospital, Bronx, New York, USA

    S. Ulick M.D.

Authors
  1. R. Tedde
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  2. A. Pala
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  3. A. Melis
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  4. S. Ulick M.D.
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Tedde, R., Pala, A., Melis, A. et al. Evidence for Cortisol as the mineralocorticoid in the syndrome of apparent mineralocorticoid excess. J Endocrinol Invest 15, 471–474 (1992). https://doi.org/10.1007/BF03348778

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  • DOI: https://doi.org/10.1007/BF03348778

Key-words

  • Apparent mineralocorticoid excess
  • Cortisol
  • dexamethasone
  • hypertension
  • mineralocorticoid receptor