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Previous Volume 343:1362-1368 November 9, 2000 Number 19 Next

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ABSTRACT

Background Glucocorticoids are essential for the normal development and functioning of the adrenal medulla. Whether adrenomedullary structure and function are normal in patients with congenital adrenal hyperplasia is not known.

Methods We measured plasma and urinary catecholamines and plasma metanephrines in 38 children with congenital adrenal hyperplasia due to 21-hydroxylase deficiency (25 children with the salt-wasting form and 13 with the simple virilizing form), 39 age-matched normal subjects, and 20 patients who had undergone bilateral adrenalectomy. Adrenal specimens obtained from three other patients with 21-hydroxylase deficiency who had undergone bilateral adrenalectomy and specimens obtained at autopsy from eight other patients were examined histologically.

Results Plasma epinephrine and metanephrine concentrations and urinary epinephrine excretion were 40 to 80 percent lower in the patients with congenital adrenal hyperplasia than in the normal subjects (P< 0.05), and the values were lowest in the patients with the most severe deficits in cortisol production. Urinary epinephrine excretion and plasma epinephrine concentrations were at or below the limit of detection of the assay in 8 (21 percent) of the patients with congenital adrenal hyperplasia and in 19 (95 percent) of the patients who had undergone adrenalectomy. In the group of patients with congenital adrenal hyperplasia, plasma epinephrine and metanephrine concentrations and urinary epinephrine excretion were approximately 50 percent lower in those who had been hospitalized for adrenal crises than in those who had not. In three patients with congenital adrenal hyperplasia who had undergone bilateral adrenalectomy, the formation of the adrenal medulla was incomplete, and electron-microscopical studies revealed a depletion of secretory vesicles in chromaffin cells.

Conclusions Congenital adrenal hyperplasia compromises both the development and the functioning of the adrenomedullary system.

Source Information

From the Warren Grant Magnuson Clinical Center, National Institutes of Health, Bethesda, Md. (D.P.M.); the Pediatric and Reproductive Endocrinology Branch, National Institute of Child Health and Human Development, Bethesda, Md. (D.P.M., G.P.C., M.W., M.F.K., S.R.B.); the Clinical Neurocardiology Section, National Institute of Neurological Disorders and Stroke, Bethesda, Md. (G.E.); the University of Virginia Health System, Charlottesville (A.D.R.); and the Department of Pediatrics, University of North Carolina School of Medicine, Chapel Hill (J.J.V.W.).

Address reprint requests to Dr. Merke at the National Institutes of Health, Bldg. 10, Rm. 13S260, 10 Center Dr., MSC 1932, Bethesda, MD 20892-1932.

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