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 organization: Raivio Group
Clinicum
University of Helsinki
Children's Hospital
Centre of Excellence in Stem Cell Metabolism
Timo Pyry Juhani Otonkoski / Principal Investigator
Department of Physiology
HUS Children and Adolescents
Date: 2018-10-15
Language: eng
Number of pages: 12
Belongs to series: Journal of the Endocrine Society
ISSN: 2472-1972
DOI: https://doi.org/10.1210/js.2018-00225
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
Peer reviewed: Yes
Rights: cc_by_nc_nd
Usage restriction: openAccess
Self-archived version: publishedVersion


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