Urinary Steroids in Post-Natal Adrenal Hyperplasia With Virilism

ABSTRACTS The urinary steroids have been analyzed in a 12-year-old girl with adrenal hyperplasia in whom virilization started at age 6. Androsterone and etiocholanolone levels were elevated and increased normally in response to adrenocorticotrophin (ACTH). The excretion of 11β-hydroxy-androsterone was high and of 11β-hydroxy and 11-ketoetiocholanolone low. She excreted 16 mg of pregnanetriol daily before ACTH, and 48 mg with ACTH. The excretion of tetrahydrocortisol and tetrahydrocortisone was normal and increased with ACTH. This steroid pattern was compared with that of 3 patients with adrenal hyperplasia and Cushing's syndrome. These patients excreted only slightly increased amounts of pregnanetriol and large amounts of all of the 11-oxyketosteroids. The occurrence of post-natal virilizing adrenal hyperplasia has been reviewed and the hypothesis offered that there is a congenital partial block of 21-hydroxylation that becomes clinically apparent only some years later.