Zheng J, Mao J, Xu H, et al. Pulsatile GnRH therapy may restore hypothalamus-pituitary-testis axis function in patients with congenital combined pituitary hormone deficiency: a prospective, self-controlled trial. J Clin Endocrinol Metab. 2017. OUP |?
Context: The effectiveness of pulsatile gonadotropin-releasing hormone (GnRH) therapy in congenital combined pituitary hormone deficiency (CCPHD) patients has not been investigated due to the limited number of patients as well as these patients’ presumed pituitary hypoplasia, poor gonadotrophic cell reserve, and impaired gonadotrophic response to GnRH.
Objective: To assess the pituitary response to pulsatile GnRH therapy in men with CCPHD.
Design: A prospective, self-controlled, 3 months clinical trial.
Settings: A University Endocrine Clinic.
Patients: Men with hypogonadotropic hypogonadism caused by CCPHD.
Intervention(s): Pulsatile GnRH was administered subcutaneously for three months.
Main Outcome Measures: Primary endpoints were total serum testosterone, testicular volume, and LH and FSH levels. Secondary endpoints included occurrence of spermatogenesis.
Results: A total of 40 male CCPHD patients completed the study. Of these, 60% (24/40) showed a good response to pulsatile GnRH treatment (response group), and their LH and FSH levels increased into the normal range and testosterone levels also increased to 8.67± 4.83 nmol/L at three months.
Of the patients in the response group, 33.3% (8/24) of them achieved spermatogenesis. The remaining 40% (16/40) of patients had a poor response to pulsatile GnRH treatment.
MRI did not reveal any correlation between pituitary response and pituitary height and/or integrity of the pituitary stalk.
Conclusions: This study suggests that gonadotrophs in CCPHD patients can exist and be functional— even with MRI evidence of pituitary hypoplasia or dysplasia. Pulsatile GnRH therapy restored pituitary-testis axis function in 60% of patients with CCPHD. These results may directly guide the clinical therapeutic choice.
Context: The effectiveness of pulsatile gonadotropin-releasing hormone (GnRH) therapy in congenital combined pituitary hormone deficiency (CCPHD) patients has not been investigated due to the limited number of patients as well as these patients’ presumed pituitary hypoplasia, poor gonadotrophic cell reserve, and impaired gonadotrophic response to GnRH.
Objective: To assess the pituitary response to pulsatile GnRH therapy in men with CCPHD.
Design: A prospective, self-controlled, 3 months clinical trial.
Settings: A University Endocrine Clinic.
Patients: Men with hypogonadotropic hypogonadism caused by CCPHD.
Intervention(s): Pulsatile GnRH was administered subcutaneously for three months.
Main Outcome Measures: Primary endpoints were total serum testosterone, testicular volume, and LH and FSH levels. Secondary endpoints included occurrence of spermatogenesis.
Results: A total of 40 male CCPHD patients completed the study. Of these, 60% (24/40) showed a good response to pulsatile GnRH treatment (response group), and their LH and FSH levels increased into the normal range and testosterone levels also increased to 8.67± 4.83 nmol/L at three months.
Of the patients in the response group, 33.3% (8/24) of them achieved spermatogenesis. The remaining 40% (16/40) of patients had a poor response to pulsatile GnRH treatment.
MRI did not reveal any correlation between pituitary response and pituitary height and/or integrity of the pituitary stalk.
Conclusions: This study suggests that gonadotrophs in CCPHD patients can exist and be functional— even with MRI evidence of pituitary hypoplasia or dysplasia. Pulsatile GnRH therapy restored pituitary-testis axis function in 60% of patients with CCPHD. These results may directly guide the clinical therapeutic choice.
