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Although cortisone acetate is approved worldwide as corticosteroid substitution therapy in congenital adrenal hyperplasia (21-hydroxylase deficiency), its effectiveness is uncertain since its biologic activity depends on activation by 11β-hydroxysteroid dehydrogenase (11β-HSD). We sought to compare the effect of cortisone acetate with that of hydrocortisone. In 10 patients with congenital adrenal hyperplasia, cortisone acetate was replaced with hydrocortisone in substitution therapy. During this change, blood concentrations of 17-hydroxy-progesterone, adrenocorticotropin (ACTH), and requirements for each drug were monitored. Concentrations of 17-hydroxyprogesterone decreased (mean 10.1 vs. 48.6 ng/ml), as did those of ACTH. Cortisone acetate dose requirements averaged 33.9 mg/m(2), while hydrocortisone dose requirements averaged only 20.3 mg/m(2). In one of the patients resistant to cortisone acetate therapy, DNA sequences in the coding regions and promoter of the 11β-HSD gene were analyzed, detecting no genetic abnormalities. Cortisone acetate is inferior to hydrocortisone as substitution therapy in patients with congenital adrenal hyperplasia.