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Requirement of Mineralocorticoid in Congenital Adrenal Hyperplasia due to 11β-Hydroxylase Deficiency

Rambam Medical Center Haifa Israel
Carmel Hospital Haifa Israel
Kaplan Hospital Rehovot Israel and Faculty of Medicine
Technion-Israel Institute of Technology Hai Israel

Address requests for reprints to: Zeev Hochberg, M.D., Endocrine Laboratory, Rambam Medical Center, P.O.B. 9602, Haifa 31096, Israel

Marginal salt loss occurs in patients with congenital adrenal hyperplasia due to 11β-hydroxylase (11-OHase) deficiency treated with dexamethasone and is accompanied by increased PRA.The present study was undertaken to evaluate the effect of the stimulated renin-angiotensin systemon pituitary-adrenal suppression. Seven patients with 11-OHase deficiency were subjected to a series of treatments with dexameth-asone, cortisol, and combined cortisol and 9ªfluorohydrocorti-sone. The latter combination suppressed PRA and sodium excretion, and produced better control of the pituitary-adrenal axis, as measured by plasma ACTA and serum 11-deoxycortisol. We conclude that in childrenwith 11-OHase deficiency, PRA needs to be monitored, and when it is elevated, mineralocorticoid replacement is indicated.

Received September 9, 1985.

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