Abstract
To investigate the therapeutic effect of pulsatile gonadotropin-releasing hormone (GnRH) on ovulation and fertility in a patient with congenital hypogonadotropic hypogonadism (CHH). We report on a 25-year-old woman diagnosed with CHH and treated with hormone replacement therapy to develop secondary sexual characteristics and maintain normal hormone levels. At the age of 35, she wished to conceive a baby for which pulsatile GnRH (15µg/90 min) was used. Following a five-month treatment period, the patient became pregnant and she delivered a 3,150-gram male child after normal labour at 37 weeks of pregnancy. Long-term management of female patients with CHH is critical. Hormone replacement therapy promotes the development of secondary sexual characteristics and maintains normal hormone levels. Ovulation induction and pulsatile GnRH therapy are required when the patient requests fertility treatment.
References
Boehm U, Bouloux PM, Dattani MT, et al. Expert consensus document: European Consensus Statement on congenital hypogonadotropic hypogonadism–pathogenesis, diagnosis and treatment. Nat Rev Endocrinol. 2015;11:547-64.
Quinton R, Duke VM, Robertson A, et al. Idiopathic gonadotrophin deficiency: genetic questions addressed through phenotypic characterization. Clin Endocrinol (Oxf). 2001;55:163-74.
Varimo T, Miettinen PJ, Känsäkoski J, Raivio T, Hero M. Congenital hypogonadotropic hypogonadism, functional hypogonadotropism or constitutional delay of growth and puberty? An analysis of a large patient series from a single tertiary center. Hum Reprod. 2017;32:147-53.
Dzemaili S, Tiemensma J, Quinton R, Pitteloud N, Morin D, Dwyer AA. Beyond hormone replacement: quality of life in women with congenital hypogonadotropic hypogonadism. Endocr Connect. 2017;6:404-12.
Emmanuel M, Bokor BR. Tanner stages. 2022 Dec 11. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan–.
Ojeda SR, Lomniczi A. Puberty in 2013: unravelling the mystery of puberty. Nat Rev Endocrinol. 2014;10:67-9.
Fan HQ, Wang YC, He W, Zhou HW, Yang T. Changes in levels of testosterone, insulin sensitivity and metabolic profiles during GnRH therapy: reciprocity between insulin sensitivity and pituitary responsiveness to GnRH in teenage and young male patients with congenital hypogonadotropic hypogonadism. Clin Endocrinol (Oxf). 2022;97:783-91.
Chaudhary S, Walia R, Bhansali A, et al. Unravelling a novel, promising and convenient tool for differential diagnosis of delayed puberty: GnRHa-stimulated inhibin B (GnRH-iB). J Endocrinol Invest. 2022;45:2265-73.
Dwyer AA, Smith N, Quinton R. Psychological aspects of congenital hypogonadotropic hypogonadism. Front Endocrinol (Lausanne). 2019;10:353.
Young J, Xu C, Papadakis GE, et al. Clinical management of congenital hypogonadotropic hypogonadism. Endocr Rev. 2019;40:669-710.
Genazzani AD, Santagni S, Chierchia E, et al. Estimation of instantaneous secretory rates and intrinsic characteristics of luteinizing hormone secretion in women with Kallmann syndrome before and after estriol administration. Reprod Biol. 2011;11:284-93.
Li S, Zhao Y, Nie M, et al. Comparison of clinical characteristics and spermatogenesis in CHH patients caused by PROKR2 and FGFR1 mutations. Reprod Sci. 2021;28:3219-27.
Macut D, Micić D, Zorić S, et al. Decrease of serum leptin levels in adult male with idiopathic hypogonadotropic hypogonadism (IHH) treated with pulsatile gonadotropin-releasing hormone (GnRH). J Endocrinol Invest. 2001;24:135-6.
Leyendecker G. GnRH ovulation induction. Fertil Steril. 1986;45:737-8.
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;102:2291-300.
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Copyright (c) 2023 Yuejiao Wang, Xiangyan Ruan, Muqing Gu, Alfred O. Mueck