Androgen and Gonadotropin Dynamics in Testicular Feminization Syndrome

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Abstract
Four adult siblings with the complete form of testicular feminization (TFS) were studied before and after gonadectomy. The mean preoperative LH level of 34.8 ± 8.4 (se) mIU/ml was significantly higher (P < 0.05) than the levels observed in normal males. The mean FSH level was in the normal male range. The meanplasma testosterone level of 1.06 ± 0.13 μg/100 ml was significantly higher (P < 0.001) than male controls. Testosterone production rates were normal. However, the testosterone metabolic clearance rates per meter2 (392 ± 8.4 L/24 hr) were significantly lower than normal men (P < 0.02), and significantly higher than normal women (P < 0.001). The decreased clearance in spite of male testosterone production rates demonstrated that the usual relationship of testosterone production to its own clearance rate did not exist in these patients. The mean androstenedione production rate and plasma concentration were significantly higher than male and female controls. The drostenedione metabolic clearance rates were normal. Gonadectomy resulted in a significant reduction of plasma testosterone and androstene dione levels and a significant elevation of FSH and LH levels. These results demonstrate abnormal androgen metabolism and a disparity in negative gonadal feedback control of FSH LH in patients with TFS.

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