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International Journal of Pediatric Endocrinology
Published in: International Journal of Pediatric Endocrinology 1/2016

Open Access 01-12-2016 | Case report

Growth and descent of the testes in infants with hypogonadotropic hypogonadism receiving subcutaneous gonadotropin infusion

Authors: Anne-Sophie Lambert, Pierre Bougneres

Published in: International Journal of Pediatric Endocrinology | Issue 1/2016

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Abstract

Background

One third of infants with congenital hypogonadotropic hypogonadism (CHH) are said to have micropenis and/or bilateral or unilateral cryptorchidism leading many of them to orchiopexy. Our previous study in two patients suggests that prolonged subcutaneous infusion of large doses of gonadotropins might normalize testicular function and growth.

Case presentation

To confirm the effects of early and prolonged subcutaneous infusion of large doses of gonadotropins on growth and descent of the testes. Eight boys with CHH, aged 0.25–11 months. Testes were non-palpable in 5 or in high scrotal position in 3. CHH was isolated in 5 infants and part of a syndrome of combined pituitary hormonal deficits in the 3 others. In response to gonadotropin infusion, mean levels of testicular hormones were normalized. Complete testis descent occurred in 6 patients. Partial descent occurred in 2. Testes re-ascended in 1 patient. Testes and penis gained normal dimensions in all cases.

Conclusion

Subcutaneous gonadotropin infusion seems able to induce testis descent in a large proportion of infants with CHH. If confirmed, this may allow patients to avoid testes surgery but studies in larger series are needed to evaluate the benefits of this treatment versus traditional orchiopexy.
Appendix
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Literature
1.
Grumbach MM. A window of opportunity: the diagnosis of gonadotropin deficiency in the male infant. J Clin Endocrinol Metab. 2005;90(5):3122–7.CrossRefPubMed
2.
Pitteloud N, Hayes FJ, Dwyer A, Boepple PA, Lee H, Crowley WF. Predictors of outcome of long-term GnRH therapy in men with idiopathic hypogonadotropic hypogonadism. J Clin Endocrinol Metab. 2002;87(9):4128–36.CrossRefPubMed
3.
Pitteloud N, Hayes FJ, Boepple PA, DeCruz S, Seminara SB, MacLaughlin DT, et al. The role of prior pubertal development, biochemical markers of testicular maturation, and genetics in elucidating the phenotypic heterogeneity of idiopathic hypogonadotropic hypogonadism. J Clin Endocrinol Metab. 2002;87(1):152–60.CrossRefPubMed
4.
Henna MR, Del Nero RGM, Sampaio CZS, Atallah AN, Schettini ST, Castro AA, et al. Hormonal cryptorchidism therapy: systematic review with metanalysis of randomized clinical trials. Pediatr Surg Int. 2004;20(5):357–9.CrossRefPubMed
5.
Penson D, Krishnaswami S, Jules A, McPheeters ML. Effectiveness of hormonal and surgical therapies for cryptorchidism: a systematic review. Pediatrics. 2013;131(6):e1897–907.CrossRefPubMedPubMedCentral
6.
Penson DF, Krishnaswami S, Jules A, Seroogy JC, McPheeters ML. Evaluation and Treatment of Cryptorchidism [Internet]. Rockville (MD): Agency for Healthcare Research and Quality (US); 2012.
7.
Main KM, Schmidt IM, Toppari J, Skakkebaek NE. Early postnatal treatment of hypogonadotropic hypogonadism with recombinant human FSH and LH. Eur J Endocrinol Eur Fed Endocr Soc. 2002;146(1):75–9.CrossRef
8.
Bougnères P, François M, Pantalone L, Rodrigue D, Bouvattier C, Demesteere E, et al. Effects of an early postnatal treatment of hypogonadotropic hypogonadism with a continuous subcutaneous infusion of recombinant follicle-stimulating hormone and luteinizing hormone. J Clin Endocrinol Metab. 2008;93(6):2202–5.CrossRefPubMed
9.
Bin-Abbas B, Conte FA, Grumbach MM, Kaplan SL. Congenital hypogonadotropic hypogonadism and micropenis: effect of testosterone treatment on adult penile size why sex reversal is not indicated. J Pediatr. 1999;134(5):579–83.CrossRefPubMed
10.
Pierik FH, Deddens JA, Burdorf A, de Muinck K-SSMPF, de Jong FH, Weber RFA. The hypothalamus-pituitary-testis axis in boys during the first six months of life: a comparison of cryptorchidism and hypospadias cases with controls. Int J Androl. 2009;32(5):453–61.CrossRefPubMed
11.
Kollin C, Granholm T, Nordenskjöld A, Ritzén EM. Growth of Spontaneously Descended and Surgically Treated Testes During Early Childhood. Pediatrics. 2013;131(4):e1174–80.CrossRefPubMed
12.
King TFJ, Hayes FJ. Long-term outcome of idiopathic hypogonadotropic hypogonadism. Curr Opin Endocrinol Diabetes Obes. 2012;19(3):204–10.CrossRefPubMed
13.
Brito VN, Berger K, Mendonca BB. Male hypogonadism: childhood diagnosis and future therapies. Pediatr Health. 2010;4(5):539–55.CrossRef
14.
Scorer CG. THE DESCENT OF THE TESTIS. Arch Dis Child déc. 1964;39:605–9.CrossRef
15.
Bay K, Main KM, Toppari J, Skakkebæk NE. Testicular descent: INSL3, testosterone, genes and the intrauterine milieu. Nat Rev Urol. 2011;8(4):187–96.CrossRefPubMed
16.
Schonfeld WA, Beebe GW. Normal growth and variation in the male genitalia from birth to maturity. J Urol. 1942;48:759–77.
17.
Lee MM, Misra M, Donahoe PK, MacLaughlin DT. MIS/AMH in the assessment of cryptorchidism and intersex conditions. Mol Cell Endocrinol. 2003;211(1–2):91–8.CrossRefPubMed
18.
Andersson AM, Toppari J, Haavisto AM, Petersen JH, Simell T, Simell O, et al. Longitudinal reproductive hormone profiles in infants: peak of inhibin B levels in infant boys exceeds levels in adult men. J Clin Endocrinol Metab. 1998;83(2):675–81.PubMed
19.
Lahlou N, Fennoy I, Carel J-C, Roger M. Inhibin B and anti-Müllerian hormone, but not testosterone levels, are normal in infants with nonmosaic Klinefelter syndrome. J Clin Endocrinol Metab. 2004;89(4):1864–8.CrossRefPubMed
20.
Trabado S, Maione L, Bry-Gauillard H, Affres H, Salenave S, Sarfati J, et al. Insulin-like peptide 3 (INSL3) in men with congenital hypogonadotropic hypogonadism/Kallmann syndrome and effects of different modalities of hormonal treatment: a single-center study of 281 patients. J Clin Endocrinol Metab. 2014;99(2):E268–75.CrossRefPubMed
21.
Liu L, Banks SM, Barnes KM, Sherins RJ. Two-year comparison of testicular responses to pulsatile gonadotropin-releasing hormone and exogenous gonadotropins from the inception of therapy in men with isolated hypogonadotropic hypogonadism. J Clin Endocrinol Metab. 1988;67(6):1140–5.CrossRefPubMed
22.
Bouloux P, Warne DW, Loumaye E, FSH Study Group in Men’s Infertility. Efficacy and safety of recombinant human follicle-stimulating hormone in men with isolated hypogonadotropic hypogonadism. Fertil Steril. 2002;77(2):270–3.CrossRefPubMed
23.
Young J, Chanson P, Salenave S, Noël M, Brailly S, O’Flaherty M, et al. Testicular anti-mullerian hormone secretion is stimulated by recombinant human FSH in patients with congenital hypogonadotropic hypogonadism. J Clin Endocrinol Metab. 2005;90(2):724–8.CrossRefPubMed
24.
Warne DW, Decosterd G, Okada H, Yano Y, Koide N, Howles CM. A combined analysis of data to identify predictive factors for spermatogenesis in men with hypogonadotropic hypogonadism treated with recombinant human follicle-stimulating hormone and human chorionic gonadotropin. Fertil Steril. 2009;92(2):594–604.CrossRefPubMed
25.
Matsumoto AM, Snyder PJ, Bhasin S, Martin K, Weber T, Winters S, et al. Stimulation of spermatogenesis with recombinant human follicle-stimulating hormone (follitropin alfa; GONAL-f): long-term treatment in azoospermic men with hypogonadotropic hypogonadism. Fertil Steril. 2009;92(3):979–90.CrossRefPubMed
26.
Bouvattier C, Tauber M, Jouret B, Chaussain JL, Rochiccioli P. Gonadotropin treatment of hypogonadotropic hypogonadal adolescents. J Pediatr Endocrinol Metab JPEM. 1999;12 Suppl 1:339–44.PubMed
27.
Raivio T, Wikström AM, Dunkel L. Treatment of gonadotropin-deficient boys with recombinant human FSH: long-term observation and outcome. Eur J Endocrinol Eur Fed Endocr Soc. 2007;156(1):105–11.CrossRef
28.
Sykiotis GP, Hoang X-H, Avbelj M, Hayes FJ, Thambundit A, Dwyer A, et al. Congenital idiopathic hypogonadotropic hypogonadism: evidence of defects in the hypothalamus, pituitary, and testes. J Clin Endocrinol Metab. 2010;95(6):3019–27.CrossRefPubMedPubMedCentral
29.
Burgués S, Calderón MD. Subcutaneous self-administration of highly purified follicle stimulating hormone and human chorionic gonadotrophin for the treatment of male hypogonadotrophic hypogonadism. Spanish Collaborative Group on Male Hypogonadotropic Hypogonadism. Hum Reprod. 1997;12(5):980–6.CrossRefPubMed
30.
Liu PY, Turner L, Rushford D, McDonald J, Baker HW, Conway AJ, et al. Efficacy and safety of recombinant human follicle stimulating hormone (Gonal-F) with urinary human chorionic gonadotrophin for induction of spermatogenesis and fertility in gonadotrophin-deficient men. Hum Reprod Oxf Engl. 1999;14(6):1540–5.CrossRef
31.
Okuyama A, Nakamura M, Namiki M, Aono T, Matsumoto K, Utsunomiya M, et al. Testicular responsiveness to long-term administration of hCG and hMG in patients with hypogonadotrophic hypogonadism. Horm Res. 1986;23(1):21–30.CrossRefPubMed
32.
Kumar PA, Pitteloud N, Andrews PAM, Dwyer A, Hayes F, Crowley WF, et al. Testis morphology in patients with idiopathic hypogonadotropic hypogonadism. Hum Reprod Oxf Engl. 2006;21(4):1033–40.CrossRef
33.
Christiansen P, Müller J, Buhl S, Hansen OR, Hobolth N, Jacobsen BB, et al. Treatment of cryptorchidism with human chorionic gonadotropin or gonadotropin releasing hormone. A double-blind controlled study of 243 boys. Horm Res. 1988;30(4–5):187–92.CrossRefPubMed
34.
Dunkel L, Taskinen S, Hovatta O, Tilly JL, Wikström S. Germ cell apoptosis after treatment of cryptorchidism with human chorionic gonadotropin is associated with impaired reproductive function in the adult. J Clin Invest. 1997;100(9):2341–6.CrossRefPubMedPubMedCentral
Metadata
Title
Growth and descent of the testes in infants with hypogonadotropic hypogonadism receiving subcutaneous gonadotropin infusion
Authors
Anne-Sophie Lambert
Pierre Bougneres
Publication date
01-12-2016
Publisher
BioMed Central
Published in
International Journal of Pediatric Endocrinology / Issue 1/2016
Electronic ISSN: 1687-9856
DOI
https://doi.org/10.1186/s13633-016-0031-9

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