scholarly journals MANAGEMENT OF ENDOCRINE DISEASE: Reversible hypogonadotropic hypogonadism

2016 ◽  
Vol 174 (6) ◽  
pp. R267-R274 ◽  
Author(s):  
Andrew A Dwyer ◽  
Taneli Raivio ◽  
Nelly Pitteloud
Keyword(s):  
Hypogonadotropic Hypogonadism ◽  
Steroid Treatment ◽  
Treatment Withdrawal ◽  
Close Monitoring ◽  
Epigenetic Factors ◽  
Endocrine Disease ◽  
Congenital Hypogonadotropic Hypogonadism ◽  
Reproductive Axis ◽  
Periodic Treatment

Congenital hypogonadotropic hypogonadism (CHH) is characterized by lack of puberty and infertility. Traditionally, it has been considered a life-long condition yet cases of reversibility have been described wherein patients spontaneously recover function of the reproductive axis following treatment. Reversibility occurs in both male and female CHH cases and appears to be more common (~10–15%) than previously thought. These reversal patients span a range of GnRH deficiency from mild to severe and many reversal patients harbor mutations in genes underlying CHH. However, to date there are no clear factors for predicting reversible CHH. Importantly, recovery of reproductive axis function may not be permanent. Thus, CHH is not always life-long and the incidence of reversal warrants periodic treatment withdrawal with close monitoring and follow-up. Reversible CHH highlights the importance of environmental (epigenetic) factors such as sex steroid treatment on the reproductive axis in modifying the phenotype. This review provides an overview and an update on what is known about this phenomenon.

scholarly journals Gonadotropin-Induced Spermatogenesis in CHH Patients with Cryptorchidism

2019 ◽  
Vol 2019 ◽  
pp. 1-6 ◽  
Author(s):  
Zhaoxiang Liu ◽  
Jiangfeng Mao ◽  
Hongli Xu ◽  
Xi Wang ◽  
Bingkun Huang ◽  
...  
Keyword(s):  
Confidence Interval ◽  
Median Time ◽  
Hypogonadotropic Hypogonadism ◽  
Sperm Concentration ◽  
Congenital Hypogonadotropic Hypogonadism

Congenital hypogonadotropic hypogonadism (CHH) patients with cryptorchidism history usually have poor spermatogenesis outcome, while researches focusing on this population are rare. This study retrospectively evaluated gonadotropin-induced spermatogenesis outcome in CHH patients with cryptorchidism (n = 40). One hundred and eighty-three CHH patients without cryptorchidism were served as control. All patients received combined gonadotropins therapy (HCG and HMG) and were followed up for at least 6 months. The median follow-up period was 24 (15, 33) months (totally 960 person-months). Sperm (>0/ml) initially appeared in semen at a median of estimated 24 months (95% confidence interval (CI) 17.8–30.2). Twenty (20/40, 50%) patients succeeded in producing sperms, and the average time to produce first sperm was 19 ± 8 months. Five pregnancies were achieved in 9 (5/9, 56%) couples who desired for children. Compared with CHH patients without cryptorchidism (n = 183), cryptorchid patients had longer median time for sperm appearance in semen (24 months vs. 15 months, P<0.001), lower rate of spermatogenesis (50% vs. 67%, P=0.032), and lower mean sperm concentration (1.9 (0.5, 8.6) million/ml vs. 11.1(1.0, 25.0) million/ml, P=0.006) at the last visit. In conclusion, CHH patients with cryptorchidism require a longer period for gonadotropin-induced spermatogenesis. The successful rate and sperm concentration were lower than patients without cryptorchidism.

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.

Gamma Knife surgery for low-flow cavernous sinus dural arteriovenous fistulas

2010 ◽  
Vol 113 (Special_Supplement) ◽  
pp. 21-27 ◽  
Author(s):  
Hyun Ho Jung ◽  
Jong Hee Chang ◽  
Kum Whang ◽  
Jin Soo Pyen ◽  
Jin Woo Chang ◽  
...  
Keyword(s):  
Gamma Knife ◽  
Cavernous Sinus ◽  
Transarterial Embolization ◽  
Gamma Knife Surgery ◽  
Low Flow ◽  
Close Monitoring ◽  
Arteriovenous Fistulas ◽  
Ocular Symptoms ◽  
Dural Arteriovenous Fistulas

Object The purpose of this study was to assess the efficacy of Gamma Knife surgery (GKS) for treating cavernous sinus dural arteriovenous fistulas (CSDAVFs). Methods Of the 4123 GKSs performed between May 1992 and March 2009, 890 procedures were undertaken to treat vascular lesions. In 24 cases, the vascular lesion that was treated was a dural arteriovenous fistula, and in 6 of these cases, the lesion involved the cavernous sinus. One of these 6 cases was lost to follow-up, leaving the other 5 cases (4 women and 1 man) to comprise the subjects of this study. All 5 patients had more than 1 ocular symptom, such as ptosis, chemosis, proptosis, and extraocular movement palsy. In all patients, CSDAVF was confirmed by conventional angiography. Three patients were treated by GKS alone and 2 patients were treated by GKS combined with transarterial embolization. The median follow-up period after GKS in these 5 cases was 30 months (range 9–59 months). Results All patients experienced clinical improvement, and their improvement in ocular symptoms was noticed at a mean of 17.6 weeks after GKS (range 4–24 weeks). Two patients received embolization prior to GKS but did not display improvement in ocular symptoms. An average of 20 weeks (range 12–24 weeks) was needed for complete improvement in clinical symptoms. There were no treatment-related complications during the follow-up period. Conclusions Gamma Knife surgery should be considered as a primary, combined, or additional treatment option for CSDAVF in selected cases, such as when the lesion is a low-flow shunt without cortical venous drainage. For those selected cases, GKS alone may suffice as the primary treatment method when combined with close monitoring of ocular symptoms and intraocular pressure.

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