Abstract:

Objective - To determine the degree of hypophyseal deficiency in Kallmann's syndrome, and the effects of Gn-RH priming and HRT. Patients and Methods - Seven female patients with complete Kallmann's syndrome were subjected to dynamic tests (chlorpromazine, TRH and double Gn-RH provocation test) immediately after their first admission to the hospital. In five patients the diagnosis was established for the first time (unprimed patients), while in the other two cases the diagnosis has been established earlier and the patients were already receiving hormonal replacement therapy (HRT-primed patients). In the 5 unprimed patients, 100 μg Gn-RH s.c. were administered daily for 28-32 days and the double Gn-RH test was repeated immediately after. Results - The gonadotropic response of the unprimed patients in the administration of Gn-RH was insufficient, mainly in the second stimulation, with secretory dominance of FSH (ratio LH/FSH <1), while after the monthly Gn-RH priming, the gonadotropic response to Gn-RH had improved, with a considerable increase in the peak values of plasma FSH and LH after both stimulations, and the LH/FSH ratio was reversed to >1. In the two primed patients, the gonadotropic response to Gn-RH administration was better in both stimulations than that of the unprimed patients. Conclusions - Both the short-term Gn-RH and the long-term HRT priming improve the secretory promptitude of the hypophyseal cells for both gonadotropins, while after long-term Gn-RH priming the LH-secreting cells are capable of both release and synthesis of the hormone, as can be seen by the results of the second stimulation in the Gn-RH primed patients. Consequently, for women with Kallmann's syndrome who wish to become pregnant, ovulation induction and conception can be achieved sooner and with less cost if they are previously primed.