Basal and Human Chorionic Gonadotropin-Stimulated 17α-Hydroxyprogesterone and Testosterone Levels in Klinefelter's Syndrome

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Abstract
In nine patients with Klinefelter's syndrome, mean basal plasma levels of testosterone (302 ± 145 ng/100 ml) and its major precursor, 17α-hydroxyprogesterone (17-OHP; 86 ± 46 ng/100 ml), were significantly lower (P s = 0.87). Administration of hCG for 3 days raised plasma testosterone and 17-OHP levels in both groups. In the Klinefelter patients, the plasma 17-OHP rise exceeded the testosterone increment, leading to a statistically significant increase (0.48 ± 0.19) of the 17-OHP to testosterone ratio, whereas this ratio remained virtually unchanged in the control subjects (0.20 ± 0.06). Together, these findings indicate that in the basal state testicular steroidogenesis is globally attenuated, whereas short term hCG stimulation shows that the later steps in the biosynthesis of testosterone may be rate limiting in Klinefelter's syndrome.

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