Recombinant Human FSH Treatment Outcomes in Five Boys With Severe Congenital Hypogonadotropic Hypogonadism

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Kohva , E , Huopio , H , Hero , M , Miettinen , P J , Vaaralahti , K , Sidoroff , V , Toppari , J & Raivio , T 2018 , ' Recombinant Human FSH Treatment Outcomes in Five Boys With Severe Congenital Hypogonadotropic Hypogonadism ' , Journal of the Endocrine Society , vol. 2 , no. 12 , pp. 1345-1356 . https://doi.org/10.1210/js.2018-00225

Title: Recombinant Human FSH Treatment Outcomes in Five Boys With Severe Congenital Hypogonadotropic Hypogonadism
Author: Kohva, Ella; Huopio, Hanna; Hero, Matti; Miettinen, Päivi J.; Vaaralahti, Kirsi; Sidoroff, Virpi; Toppari, Jorma; Raivio, Taneli
Contributor: University of Helsinki, Raivio Group
University of Helsinki, Raivio Group
University of Helsinki, Centre of Excellence in Stem Cell Metabolism
University of Helsinki, Raivio Group
University of Helsinki, Department of Physiology
Date: 2018-10-15
Language: eng
Number of pages: 12
Belongs to series: Journal of the Endocrine Society
ISSN: 2472-1972
URI: http://hdl.handle.net/10138/292439
Abstract: Context Recombinant human FSH (r-hFSH), given to prepubertal boys with hypogonadotropic hypogonadism (HH), may induce Sertoli cell proliferation and thereby increase sperm-producing capacity later in life. Objective To evaluate the effects of r-hFSH, human chorionic gonadotropin (hCG), and testosterone (T) in such patients. Design and Setting Retrospective review in three tertiary centers in Finland between 2006 and 2016. Patients Five boys: ANOS1 mutation in two, homozygous PROKR2 mutation in one, FGFR1 mutation in one, and homozygous GNRHR mutation in one. Prepubertal testicular volume (TV) varied between 0.3 and 2.3 mL; three boys had micropenis, three had undergone orchidopexy. Interventions Two boys received r-hFSH (6 to 7 months) followed by r-hFSH plus hCG (33 to 34 months); one received T (6 months), then r-hFSH plus T (29 months) followed by hCG (25 months); two received T (3 months) followed by r-hFSH (7 months) or r-hFSH plus T (8 months). Main Outcome Measures TV, inhibin B, anti-Müllerian hormone, T, puberty, sperm count. Results r-hFSH doubled TV (from a mean ± SD of 0.9 ± 0.9 mL to 1.9 ± 1.7 mL; P < 0.05) and increased serum inhibin B (from 15 ± 5 ng/L to 85 ± 40 ng/L; P < 0.05). hCG further increased TV (from 2.1 ± 2.3 mL to 8.6 ± 1.7 mL). Two boys with initially extremely small testis size (0.3 mL) developed sperm (maximal sperm count range, 2.8 to 13.8 million/mL), which was cryopreserved. Conclusions Spermatogenesis can be induced with gonadotropins even in boys with HH who have extremely small testes, and despite low-dose T treatment given in early puberty. Induction of puberty with gonadotropins allows preservation of fertility
Subject: 3121 General medicine, internal medicine and other clinical medicine
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