scholarly journals Predictors of reproductive and non-reproductive outcomes of gonadotropin mediated pubertal induction in male patients with congenital hypogonadotropic hypogonadism (CHH)

2021 ◽  
Author(s):  
B. Cangiano ◽  
G. Goggi ◽  
S. Federici ◽  
C. Bresesti ◽  
L. Cotellessa ◽  
...  
Keyword(s):  
Genetic Background ◽  
Semen Analysis ◽  
Hypogonadotropic Hypogonadism ◽  
Bone Age ◽  
Reproductive Outcomes ◽  
Targeted Next Generation Sequencing ◽  
Congenital Hypogonadotropic Hypogonadism ◽  
Gonadotropin Stimulation ◽  
Complete Form ◽  
Male Patients

Abstract Purpose To investigate predictors of testicular response and non-reproductive outcomes (height, body proportions) after gonadotropin-induced puberty in congenital hypogonadotropic hypogonadism (CHH). Design A retrospective analysis of the puberty induction in CHH male patients, undergoing an off-label administration of combined gonadotropin (FSH and hCG). Methods Clinical and hormonal evaluations before and during gonadotropin stimulation in 19 CHH patients genotyped by Targeted Next Generation Sequencing for CHH genes; 16 patients underwent also semen analysis after gonadotropins. Results A lesser increase in testicular volume after 24 months of induction was significantly associated with: (I) cryptorchidism; (II) a positive genetic background; (III) a complete form of CHH. We found no significant correlation with the cumulative dose of hCG administered in 24 months. We found no association with the results of semen analyses, probably due to the low numerosity. Measures of body disproportion (eunuchoid habitus and difference between adult and target height: deltaSDSth), were significantly related to the: (I) age at the beginning of puberty induction; (II) duration of growth during the induction; (III) initial bone age. The duration of growth during induction was associated with previous testosterone priming and to partial forms of CHH. Conclusions This study shows that a strong genetic background and cryptorchidism, as indicators of a complete GnRH deficiency since intrauterine life, are negative predictors of testicular response to gonadotropin stimulation in CHH. Body disproportion is associated with a delay in treatment and duration of growth during the induction, which is apparently inversely related to previous androgenization.

scholarly journals MON-273 Retrospective Analysis of Gonadotropin-Mediated Pubertal Induction in Male Patients with Congenital Hypogonadotropic Hypogonadism (CHH)

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Biagio Cangiano ◽  
Giovanni Goggi ◽  
Fabiana Guizzardi ◽  
Valeria Vezzoli ◽  
Paolo Duminuco ◽  
...  
Keyword(s):  
Retrospective Analysis ◽  
Multiple Regression ◽  
Semen Analysis ◽  
Final Height ◽  
Pubertal Development ◽  
Hypogonadotropic Hypogonadism ◽  
Bone Age ◽  
Testicular Development ◽  
Testosterone Replacement Therapy ◽  
Male Patients

Abstract CHH is a rare disease characterized by a failure to enter (complete forms) or to complete (partial forms) pubertal development. It has a strong genetic background and it needs a treatment to allow the puberty to complete. In male this goal could be achieved either by the classic testosterone replacement therapy or by the exogenous gonadotropins (Gn) administration which allows both the endogenous testosterone production and the testicular development. So far, only few studies have explored this latter therapeutic approach in inducing the CHH pubertal development and no internationally recognized protocols are available. Aim of this retrospective analysis is to (i) investigate clinical and biochemical predictors of testicular response to Gn-induced puberty in CHH; (ii) study the non-reproductive outcomes of this treatment (height, body proportions) and their determinants. A total of 19 CHH male patients, undergoing two years of Gn-mediated (FSH and hCG) puberty induction started between the ages of 14 and 23 years, were retrospectively evaluated. For each patient clinical history, physical examination, hormonal evaluation, and genetic analysis using Targeted Next Generation Sequencing for CHH genes was performed; 8 patients accepted to perform a semen analysis (SA) at the end of their treatment.Mann Whitney test and multiple regression analysis showed testicular volume after 24 months of Gn-mediated pubertal induction, to be significantly associated with: (i) the presence of cryptorchidism; (ii) the presence of a completely definable genetic cause for the disease; (iii) the presence of a complete CHH form. No significant association was found with the cumulative dose of hCG administered in 24 months. The statistical analyses regarding SA could not find the same associations. Multiple regression analyses investigating the eunuchoid habitus and a measure of the difference of subject’s final height from his target (deltaSDSth), showed a significant association with: (i) age at the beginning of the induction; (ii) the duration of growth during induction; (iii) and (for deltaSDSth) bone age before the induction. Duration of growth during induction resulted to be associated with previous testosterone priming and with partial CHH.In summary, our study confirms cryptorchidism and complete genetic forms of CHH as negative predictors of testicular response probably because they usually affect early phases of life with a complete GnRH deficiency. We also found that the eunuchoid habitus and deltaSDSth are associated not only with delayed treatment, but also with the duration of stature growth during the induction, apparently related to earlier androgenization.

Gonadotropins for testicular descent in cryptorchid congenital hypogonadotropic hypogonadism males beyond infancy

2021 ◽  
Vol 0 (0) ◽  
Author(s):  
Shreya Sharma ◽  
Ravikumar Shah ◽  
Virendra Patil ◽  
Anurag R. Lila ◽  
Vijaya Sarathi ◽  
...  
Keyword(s):  
Semen Analysis ◽  
Hypogonadotropic Hypogonadism ◽  
Sperm Concentration ◽  
Inhibin B ◽  
Testicular Descent ◽  
Total Testosterone ◽  
Human Menopausal Gonadotropin ◽  
Beneficial Effects ◽  
Congenital Hypogonadotropic Hypogonadism

Abstract Objectives To study the effect of combined gonadotropin therapy (CGT) on testicular descent ± spermatogenesis in congenital hypogonadotropic hypogonadism (CHH) patients with cryptorchidism beyond infancy. Methods This retrospective cohort study included CHH patients with cryptorchidism [bilateral (n=5) or unilateral (n=1)] treated with CGT for testicular descent ± pubertal induction. All participants were treated with CGT [human menopausal gonadotropin (hMG) and human chorionic gonadotropin (hCG)] with hMG pretreatment in three and monitored for changes in testicular volume (TV), serum total testosterone (T), serum inhibin-B, and sperm concentration. Results Complete testicular descent to the scrotal position was achieved in 5/6 patients (10/11 testes) after 4.7 ± 1.6 months of treatment. There was 44 ± 18%, 97.5% (IQR: 44–195), 10-fold (IQR: 3–19.6), and two-fold (IQR: 1.7–9.3) increase in stretched penile length, ultrasound measured TV, T level, and serum inhibin-B from baseline, respectively. In two pediatric cases, testicular descent occurred with isolated hMG therapy. At the last follow up (median: 23.5, IQR: 10.5–38.7 months), all the descended testes remained in scrotal position. In four pubertal/postpubertal age patients, continuous CGT (18–60 months) yielded T and inhibin-B levels of 16.64 ± 1.46 nmol/l and 106 ± 32.6 pg/mL, respectively. All the three patients with available semen analysis had sperm concentration of ≥5 million/mL and one of them achieved paternity. Conclusions A trial of CGT before orchiopexy may be considered in CHH males with cryptorchidism even beyond the narrow age-window of infancy. CGT may also have beneficial effects on future spermatogenesis and fertility outcomes in these patients.

Health-related quality of life in male patients with congenital hypogonadotropic hypogonadism

2015 ◽  
Vol 83 (1) ◽  
pp. 141-143 ◽  
Author(s):  
Tero Varimo ◽  
Matti Hero ◽  
Eeva-Maria Laitinen ◽  
Harri Sintonen ◽  
Taneli Raivio
Keyword(s):  
Quality Of Life ◽  
Hypogonadotropic Hypogonadism ◽  
Health Related Quality ◽  
Congenital Hypogonadotropic Hypogonadism ◽  
Related Quality ◽  
Health Related ◽  
Male Patients
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