The predictive value of double Gn-RH provocation test in unprimed Gn-RH-primed and steroid-primed female patients with Kallmann's syndrome

Objective-To determine the degree of hypophyseal deficiency in Kallmann's s yndrome, and the effects of Gn-RH priming and HRT. Patients and Methods-Sev en female patients with complete Kallmann's syndrome were subjected to dyna mic tests (chlorpromazine, TRH and double Gn-RH provocation test) immediate ly after their first admission to the hospital. In five patients the diagno sis was established for the first time (unprimed patients), while in the ot her two cases the diagnosis has been established earlier and the patients w ere already receiving hormonal replacement therapy (HRT-primed patients). I n the 5 unprimed patients, 100 mu g Gn-RH s.c. were administered daily for 28-32 days and the double Gn-RH test was repeated immediately after. Result s-The gonadotropic response of the unprimed patients in the administration of Cn-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 incre ase in the peak values of plasma FSH and LH after both stimulations, and th e LH/FSH ratio was reversed to >1. In the two primed patients, the gonadotr opic response to Gn-RH administration was better in both stimulations than that of the unprimed patients. Conclusions-Both the short-term Gn-RH and th e long-term HRT priming improve the secretory promptitude of the hypophysea l 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, a s 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 beco me pregnant, ovulation induction and conception can be achieved sooner and with less cost if they are previously primed.