Literature DB >> 28081535

Efficacy and Safety of Continuous Subcutaneous Infusion of Recombinant Human Gonadotropins for Congenital Micropenis during Early Infancy
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Athanasia Stoupa, Dinane Samara-Boustani, Isabelle Flechtner, Graziella Pinto, Isabelle Jourdon, Laura González-Briceño, Maud Bidet, Kathleen Laborde, Didier Chevenne, Anne-Elodie Millischer, Henri Lottmann, Thomas Blanc, Yves Aigrain, Michel Polak, Jacques Beltrand.   

Abstract

BACKGROUND: Early postnatal administration of gonadotropins to infants with congenital hypogonadotropic hypogonadism (CHH) can mimic minipuberty, thereby increasing penile growth. We assessed the effects of gonadotropin infusion on stretched penile length (SPL) and hormone levels in infants with congenital micropenis.
METHODS: Single-center study including 6 males with micropenis in case of isolated CHH (n = 4), panhypopituitarism (n = 1), and partial androgen insensitivity syndrome (PAIS; n = 1). Patients were evaluated at baseline, monthly and at the end of the study through a clinical examination (SPL, testicular position and size), serum hormone assays (testosterone, luteinizing hormone, follicle-stimulating hormone, inhibin B, anti-Müllerian hormone [AMH]), and ultrasound of penis/testes.
RESULTS: In CHH, significant increases occurred in serum testosterone (from undetectable level to 3.5 ± 4.06 ng/mL [12.15 ± 14.09 nmol/L]), SPL (from 13.8 ± 4.5 to 42.6 ± 5 mm; p < 0.0001), inhibin B (from 94.8 ± 74.9 to 469.4 ± 282.5 pg/mL, p = 0.04), and AMH (from 49.6 ± 30.6 to 142 ± 76.5 ng/mL, p = 0.03). Micropenis was corrected in all patients, except one. On treatment, in the patient with PAIS, SPL was increased from 13 to 38 mm.
CONCLUSIONS: Early gonadotropin infusion is a safe, well-tolerated and effective treatment. The effect in PAIS has not been reported previously. Long-term follow-up is needed to assess the impact, if any, on future fertility and reproduction.
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© 2017 S. Karger AG, Basel.

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Year:  2017        PMID: 28081535     DOI: 10.1159/000454861

Source DB:  PubMed          Journal:  Horm Res Paediatr        ISSN: 1663-2818            Impact factor:   2.852


  10 in total

Review 1.  Paediatric and adult-onset male hypogonadism.

Authors:  Andrea Salonia; Giulia Rastrelli; Geoffrey Hackett; Stephanie B Seminara; Ilpo T Huhtaniemi; Rodolfo A Rey; Wayne J G Hellstrom; Mark R Palmert; Giovanni Corona; Gert R Dohle; Mohit Khera; Yee-Ming Chan; Mario Maggi
Journal:  Nat Rev Dis Primers       Date:  2019-05-30       Impact factor: 52.329

Review 2.  Minipuberty in Klinefelter syndrome: Current status and future directions.

Authors:  Lise Aksglaede; Shanlee M Davis; Judith L Ross; Anders Juul
Journal:  Am J Med Genet C Semin Med Genet       Date:  2020-06-01       Impact factor: 3.908

3.  Treatment of congenital hypogonadotropic hypogonadism in male patients.

Authors:  Hae Sang Lee; Young Suk Shim; Jin Soon Hwang
Journal:  Ann Pediatr Endocrinol Metab       Date:  2022-09-30

4.  Minipuberty of human infancy - A window of opportunity to evaluate hypogonadism and differences of sex development?

Authors:  Christoffer Højrup Renault; Lise Aksglaede; Ditte Wøjdemann; Anna Berg Hansen; Rikke Beck Jensen; Anders Juul
Journal:  Ann Pediatr Endocrinol Metab       Date:  2020-06-30

Review 5.  Up-To-Date Review About Minipuberty and Overview on Hypothalamic-Pituitary-Gonadal Axis Activation in Fetal and Neonatal Life.

Authors:  Lucia Lanciotti; Marta Cofini; Alberto Leonardi; Laura Penta; Susanna Esposito
Journal:  Front Endocrinol (Lausanne)       Date:  2018-07-23       Impact factor: 5.555

6.  Treatment of gonadotropin deficiency during the first year of life: long-term observation and outcome in five boys.

Authors:  Ella Kohva; Hanna Huopio; Johanna Hietamäki; Matti Hero; Päivi J Miettinen; Taneli Raivio
Journal:  Hum Reprod       Date:  2019-05-01       Impact factor: 6.918

Review 7.  Congenital Hypogonadotrophic Hypogonadism: Minipuberty and the Case for Neonatal Diagnosis.

Authors:  Du Soon Swee; Richard Quinton
Journal:  Front Endocrinol (Lausanne)       Date:  2019-02-21       Impact factor: 5.555

Review 8.  Ontogeny of Hypothalamus-Pituitary Gonadal Axis and Minipuberty: An Ongoing Debate?

Authors:  Carla Bizzarri; Marco Cappa
Journal:  Front Endocrinol (Lausanne)       Date:  2020-04-07       Impact factor: 5.555

Review 9.  Congenital Micropenis: Etiology And Management.

Authors:  Marianna Rita Stancampiano; Kentaro Suzuki; Stuart O'Toole; Gianni Russo; Gen Yamada; Syed Faisal Ahmed
Journal:  J Endocr Soc       Date:  2021-11-15

Review 10.  Male Central Hypogonadism in Paediatrics - the Relevance of Follicle-stimulating Hormone and Sertoli Cell Markers.

Authors:  Romina P Grinspon; Mariela Urrutia; Rodolfo A Rey
Journal:  Eur Endocrinol       Date:  2018-09-10
  10 in total

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