Context: We previously observed that continuous subcutaneous infusion (CSI) of gonadotropins in infants with congenital hypogonadotropic hypogonadism (CHH) can mimic the hormonal pattern of minipuberty.
Objective: Describe the peripubertal outcome in gonadotropins CSI treated patients.
Patients: A retrospective study was realised in Bicetre Hospital between 2004 and 2022. Follow-up data and long-term observations were available for 7 boys with HH, aged 12,9 years [12,6-13,3]; 4/7 with combined pituitary hormone deficiencies (CPHD) and ectopic neurohypophysis ; 3/7 with isolated HH (IHH). 5/7 patients have cryptorchidism; Patients were treated at the mean age of 5.8 months [0.25-11] with rhLH and rhFSH CSI at a daily rate of 50 and 75-150 UI, respectively. As a retrospective control cohort, we provide data of eleven untreated CHH boys (age : 14,6 years [13–17.4 years], 6/11 with combined pituitary hormone deficiencies (CPHD) and ectopic neurohypophysis ; 5/11 with IHH.
Results: In response to early gonadotropin CSI, serum testosterone and inhibine B levels increased from 0,02 ng/ml to 1,32 ng/ml [1,12-3,36] and from 90 [5-145] to 328 [185-578] pg/ml, respectively. Testicular volume (TV) increased from 0.28 ml [0.2-0.4] to 0.75 ml[0.4-0.9]. Orchidopexy was realized in 2/5 patients. Penis reached normal dimensions. In long-term follow-up, FSH and LH values were very low in the two groups. Inhibine B values available in 7/7 boys (35 pg/ml [19-134]), significantly differ from the controls (6 pg/ml [5-34])(P=0,04)) ; TV was also significatively better in the CSI treated group (0,78ml [0,5-3]) vs untreated group (0,3ml [0,13-2,6] (P=0,012).
Conclusion: We describe the first long-term follow-up data in CHH boys treated with gonadotropin CSI. These results suggest that the effects of the gonadotropin infusion seem persistant at the beginning of the puberty. Follow-up should be continued until the end of puberty to assess spermatogenesis in these patients.
15 Sep 2022 - 17 Sep 2022