Further Evidence of a Target Organ Defect in the Syndrome of Testicular Feminization1

Abstract

Two patients with the syndrome of testicular feminization had peripheral venous plasma and urinary testosterone levels similar to those of normal adult males. In one, testosterone was 38.5 μg/100 ml in gonadal venous plasma. The disappearance rates of testosterone-4-14C in plasma of both patients were like those of normal adult male and female controls. Urinary metabolites of labeled testosterone likewise did not differ from those of normals. Plasma estradiol was in the range for normal adult females, while urinary estrogens were in the overlapping ranges for males and females. There was no evidence of a significant peripheral conversion of testosterone to estrogens. Under metabolic ward conditions the urinary excretion of nitrogen, phosphorus and citric acid was measured in one patient and a control before and during treatment with testosterone propionate. There was no change in the excretion of these compounds when the patient was given up to 200 mg every other day. On a smaller dosage, however, the control responded promptly with decreases in the excretion of all 3 substances. These findings provide further evidence that this syndrome is the result of a defect in the target organ response to testosterone.