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The 21-Hydroxylase Activity in the Glomerulosa and Fasciculata of the Adrenal Cortex in Congenital Adrenal Hyperplasia^*

URSULA KUHNLE, DIANE CHOW, ROBERT RAPAPORT, SONGJA PANG, LENORE S. LEVINE and MARIA I. NEW

Division of Pediatric Endocrinology, Department of Pediatrics, The New York Hospital-Cornell Medical Center New York, New York 10021

Address requests for reprints to: Dr. Maria I. New, Department of Pediatrics, New York Hospital-Cornell Medical Center, 525 East 68th Street,New York, New York 10021.

In the two clinical syndromes of congenital adrenal hyperplasia due to a 21-hydroxylation defect of adrenal steroidogenesis, the simple virilizing and the salt-wasting forms, the 21-hydroxylase activity was studied considering the zona fasciculata and the zona glomerulosa of the adrenalcortex as two separate glands under different regulation. To test this hypothesis, we stimulated adrenal steroidogenesis by ACTH infusion or dietary sodium restriction in eight patients with congenital adrenal hyperplasia (four patients with the simple virilizing form and four with the salt-wasting form of congenital adrenal hyperplasia) and in six normal children. Both the 17-hydroxy and 17-deoxy pathways of adrenocortical steroid biosynthesis were examined by measuring serum concentrations of 17-hydroxyprogesterone, cortisol, progesterone, deoxycorticosterone, corticosterone, and aldosteroneand the excretion of free deoxycorticosterone, 18-hydroxydeoxycorticosterone, corticosterone, 18-hydroxycorticosterone, cortisol, and aldosterone. We considered the steroids 18-hydroxycorticosterone and aldosterone to be primarily of zona glomerulosa origin. These studies indicated that the zona fasciculata of both the salt-wasting and the simple virilizing forms is defective in 21-hydroxylation of 17-hydroxy and 17-deoxy steroids. The zona glomerulosa demonstrated deficient 21-hydroxylation only in the salt-wasting form, whereas in the simple virilizing form, the glomerulosa was spared this defect. (JClin Endocrinol Metab 52: 534, 1981)

^* This work was supported in part by the USPHS (NIH Grants HD-00072 and HL-17749) and a grant (RR-47) from the General Clinical Research Centers Program of the Division of Research Resources, NIH.

Recipient of a Deutsche Forschungsgemeinschaft grant.

Received July 21, 1980.

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