Biologically active luteinizing hormone (LH) in Klinefelter's syndrome: response to gonadotropin-releasing hormone (GnRH) and effects of testosterone undecanoate.

Basal and gonadotropin-releasing hormone (GnRH)-stimulated levels of biologically active and immunoreactive LH (bLH and iLH) were measured in six patients with Klinefelter's syndrome (KS) (mean age 24.7 years). In the same patients the diurnal rhythm of serum testosterone (T) was investigated (morning values vs. evening values). The results were compared with those obtained in ten normal young men (mean age 29.3 years). Moreover, in one patient with KS we studied the effects of testosterone undecanoate (TU) administration on bLH and iLH basal levels. A sensitive "in vitro" bioassay, based on T production by mechanically dispersed mouse Leydig cells, was employed to assess LH bioactivity. Levels of iLH and T were determined by a double antibody radio-immunoassay technique. Mean basal levels of bLH and iLH were significantly higher (p less than 0.001) in the Klinefelter patients than in normal men, whereas the mean bioactivity to immunoreactivity (b/i) ratio of LH was similar in the two groups. The mean morning T concentration was significantly higher in normal men (p less than 0.001) than in the Klinefelter group. The diurnal T rhythm was lost in the patients with KS. In the Klinefelter patients the relative maximum response of bLH to GnRH (bLH delta%) was significantly lower (p less than 0.02) than in the control men. In addition, the b/i ratio of GnRH-stimulated Lh decreased significantly (p less than 0.05) from basal values in the Klinefelter patients, whereas it remained unchanged in the control group. In the patient with KS treated with androgen replacement therapy, TU decreased iLH serum levels more than bLH concentrations, thereby increasing the b/i ratio of basally secreted LH.