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Journal of Clinical Endocrinology & Metabolism Vol. 62, No. 5 934-940
doi:10.1210/jcem-62-5-934
Copyright © 1986 by the Endocrine Society.
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Evidence for the Secretion of an Antimineralocorticoid in Congenital Adrenal Hyperplasia*

URSULA KUHNLE{dagger}, MARIANNE LAND and STANLEY ULICK

Children's Hospital, University of Munich D-8000 Munich 2, West Germany Veterans Administration Hospital Bronx, New York 10468 Department of Medicine, Mount Sinai School of Medicine New York, New York 10029

Address requests for reprints to: Dr.S.Ulick, Veterans Administration Hospital, Bronx, New York 10468.

Plasma extracts from patients with congenital adrenal hyperplasia were found to contain substances that competed with aldosterone for mineralocorticoid receptor-binding sites in a rat kidney cytosol system. In normal subjects and patients with other disorders, the mineralocorticoid receptor-binding activity in such extracts could be entirely accounted for by the sum of the contributions of the steroids known to bind to the mineralocorticoid receptor. The secretion of these binding substances in patients with the C-21 hydroxylation defect was ACTH dependent. While these substances could be either min-eralocorticoid agonists or antagonists, the latter is more likely. Production of mineralocorticoid antagonists would account for the compensatory hyperaldosteronism that occurs in the simple virilizing form, in which there is minimal impairment of aldosterone secretory reserve, and for the tendency to Addisonian crisis in patients with the salt-losing form, who have a more severe defect in aldosterone biosynthesis.

* This work was supported by the V.A. and NIH Grant AM-32257.

{dagger} Recipient of a Deutsche Forschungsgemeinshaft grant.

Received June 11, 1985.




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P. C. White and P. W. Speiser
Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency
Endocr. Rev., June 1, 2000; 21(3): 245 - 291.
[Abstract] [Full Text]




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