URINARY HORMONE STUDIES IN UNTREATED CONGENITAL ADRENAL HYPERPLASIA DURING AND AFTER PREGNANCY

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Abstract
A 22-year-old patient is described with congenital adrenal hyperplasia and spontaneous menstrual cycles who became pregnant prior to the institution of steroid therapy. During the latter part of gestation there was a progressive decrease in the daily urinary excretion of neutral 17-ketosteroids, pregnanediol and pregnanetriol. Excretion of the latter two compounds remained low until the sixteenth week, when spontaneous abortion occurred. Chromatographic separation of the urinary neutral 17-ketosteroids revealed a predominance of androsterone. Following the administration of corticotropin during pregnancy there was a pronounced increase in the urinary output of androsterone, a decrease in the output of etiocholanolone, and a rise in the ratio of androsterone to etiocholanolone from 1.3 to 5.8. These changes took place at a time when there was no significant urinary excretion of pregnanediol and pregnanetriol. The data indicate an alteration in either the production or metabolism of progesterone and 17[alpha]-hydroxyprogesterone, and suggest that the major source of the increased androsterone was an excess adrenal production of C19 steroids relative to C21 compounds. The presence of ovulatory menses in this patient demonstrates that the increased circulating androgens could not disrupt the normal pituitary-ovarian relationship despite their virilizing capabilities.

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