Measurements of plasma 17-hydroxycorticosteroid concentration and urinary 17-ketosteroid excretion were made in children with congenital adrenal hyperplasia. These results have been compared with blood ACTH titers in the same children. Prior to cortisone therapy endogenous ACTH concentrations and 17-ketosteroid excretion were abnormally high whereas circulating 17-hydroxycorticosteroid concentrations were exceptionally low. During the period of cortisone therapy these values approached normal. Adrenal unresponsiveness to ACTH was evidenced in patients with congenital adrenal hyperplasia by their failure to produce elevations of circulating 17-hydroxycorticosteroid concentrations in response to ACTH injection.

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