Objective: To evaluate the efficacy of pulsatile gonadotropin-releasing hormone (GnRH) therapy in male patients with pituitary stalk interruption syndrome (PSIS).
Methods: Fourteen male PSIS patients, who had received subcutaneous pulsatile GnRH therapy for at least four weeks at Peking Union Medical College Hospital from April 2014 to September 2015, were included in the study. Serum luteinizing hormone (LH), follicle-stimulating hormone (FSH), testosterone level, testicular volume and sperm in ejaculation were measured. After treatment for four weeks, subjects with serum LH≥1.24 U/L were classified as"good response group", and others were classified as"poor response group".
Results: All patients were born by breech delivery and presented with absent pubertal development. Laboratory findings showed combined pituitary hormone deficiency in all patients. Pituitary stalk interruption syndrome was diagnosed based on MRI findings. Totally 11 out of 14 patients showed good response to pulsatile GnRH therapy. Their LH level increased from 0.32 (0.15, 0.61) U/L to 4.34(2.52, 7.39)U/L(P<0.001), FSH from 0.84 (0.30, 1.50) U/L to 7.12 (4.53, 10.09) U/L(P<0.001); serum testosterone level from 0 (0, 0.45) nmol/L to 2.46 (0.56, 11.45) nmol/L (P=0.004) and testicular volume from 2.0 (2.0, 4.0) ml to 4.0 (3.0, 6.0) ml (P=0.005). Sperm was detected in seminal samples after treatment for 8 weeks in two patients and for 12 weeks in one patients. Three out of 14 patients showed poor response to pulsatile GnRH therapy.
Conclusion: Pulsatile GnRH therapy can effectively establish the pituitary-testicular axis function for most patients with pituitary stalk interruption syndrome, indicating enough reserve of gonadotrophs in the pituitary.