THE URINARY EXCRETION OF THREE C-21 METHYL CORTICOSTEROIDS IN THE ADRENOGENITAL SYNDROME*†

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Abstract
Three of the more abundant C-21 methyl corticosteroids were measured in the urine of 11 patients (aged 2 weeks to 23 years) with the adrenogenital syndrome, and compared with the excretion in normal controls and 4 cases of virilizing adrenal adenomas. In all patients with the adrenogenital syndrome due to congenital adrenal hyperplasia, there was an elevation of urinary pregnanetriol (average, 0.29 mg. / Kg. / day), 11-ketopregnanetriol (0.44 mg. / Kg. / day) and 17-hydroxypregnanolone (0.44 mg. / Kg. / day). In the normal controls, the excretion of each steroid was 0.01 mg. / Kg. / day or less. No significant elevation of any of the 3 compounds was observed in the cases of adrenal adenoma. Urinary 11-ketopregnanetriol was highest in the youngest patients, all of whom were “salt-losers,” but not in an older child who was also a “salt-loser”; thus the disproportionate increase in this single compound may be related to age. The combined excretion of pregnanetriol and 17-OH-pregnanolone (products of 17-hydroxyprogesterone) was higher than that of 11-ketopregnanetriol in the older patients, whether or not they were “saltlosers.” A single case with hypertension failed to demonstrate similar increases in excretion of any of the 3 compounds. Otherwise there did not appear to be any definite relationship between the pattern of excretion and the type of disease. As expected, the urinary neutral 17-ketosteroids were elevated in all patients with the adrenogenital syndrome (0.90 mg. / Kg. / day) and were highest in the patients with tumors (9.64 mg. / Kg. / day).

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