Abstract
The existence of a salt-excreting factor in CAH due to a 21-hydroxylase defect has been postulated for many years.However extensive investigations sofar were unable to isolate such a factor.Our demonstration of a salt-excreting factor in CAH utilized direct and indirect measurements of the mineralocorticoidactivity.The direct method was based on the ability of mineralocorticoid agonists and antagonists to displace aldosterone in a mineralocorticoid radio-receptor assay.The indirect method measured the separate contributions of the principal mineralocorticoid agonists:aldosterone, 11-deoxycorticosterone,corticosterone and cortisol as determined from their relative receptor affinity. In controls there was good agreement between these two measurements.However,when plasma extracts from patients with CAH were assayed, the direct method showed almost twice as much activity as the indirect method. This difference in mineralocorticoid receptor activity was observed in both the salt-wasting and the non-salt-wasting form and was suppressed by dexamethasone and restored by ACTH. We propose that this difference results from the presence of a mineralocorticoid antagonist which competes with the known agonists for the mineralocoid receptor. In salt-wasting CAH this antagonist may contribute to salt-wasting symptoms;in the simple-virilizing form this antagonist may cause hyperreninemia and hyperaldosteronism.
Article PDF
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Kuhnle, U., Dörr, H., Bidlingmaier, F. et al. EVIDENCE FOR A NATURALLY OCCURING MINERALOCORTICOIDANTAGONIST IN CONGENITAL ADRENAL HYPERPLASIA (CAH). Pediatr Res 18, 1208 (1984). https://doi.org/10.1203/00006450-198411000-00043
Issue Date:
DOI: https://doi.org/10.1203/00006450-198411000-00043