URINARY EXCRETION OF ADRENAL HORMONES IN GONADAL DYSGENESIS AND ITS RESPONSE TO CORTICOTROPHIN AND GONADOTROPHIN STIMULATION

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Abstract
The functional capacity of the adrenals was studied in eight adult patients with gonadal dysgenesis, ranging in age from 16-42 years, by measuring the 24-hour urinary excretion of 17-hydroxycorticosteroids (17-OHCS) and of 17-ketosteroids (17-KS) with the patient at rest and also by estimating its response to stimulation with corticotrophin and chorionic gonadotrophin. In six patients the clinical diagnosis of the syndrome was confirmed by laparotomy, when no gonads were found in five patients and a white fibrous band replacing the gonads was found in the other. The remaining, two patients of the series both had male sex-chromatin. The resting excretion of both had male sex-chromatin. The resting excretion of both 17-OHCS and 17-KS was within normal limits in all eight patients. After corticotrophin stimulation (25 IU infused over 8 hours) an increased 17-OHCS excretion was found in all patients but the increase was less constant for the 17-KS. These results are identical with those expected from normal subjects and were considered to indicate a normal adrenal secretory capacity in the present cases. Stimulation by chorionic gonadotrophin (HCG test) was performed in four patients using various doses of the hormone, namely 1500 IU for 5 days, 5000 IU for 2 or 5 days, or 10 000 IU for 2 days. Three of these patients had no gonads and the fourth had male sex-chromatin. The HCG test failed to elicit an increased excretion of either 17-OHCS or 17-KS in these patients. This absence of response is regarded as evidence that HCG does not stimulate adrenal tissue'' to secrete the precursors of these hormones. The findings of the present study are discussed in the light of previous publications.

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