Efficacy and Safety of Continuous Subcutaneous Infusion of Recombinant Human Gonadotropins for Congenital Micropenis during Early Infancy

Autor: Laura González-Briceño, Graziella Pinto, Anne-Elodie Millischer, Jacques Beltrand, Isabelle Jourdon, Dinane Samara-Boustani, Yves Aigrain, Athanasia Stoupa, Maud Bidet, Michel Polak, Thomas Blanc, Isabelle Flechtner, Kathleen Laborde, Henri Lottmann, Didier Chevenne
Rok vydání: 2017
Předmět:
Anti-Mullerian Hormone
Male
0301 basic medicine
endocrine system
medicine.medical_specialty
Endocrinology
Diabetes and Metabolism
030209 endocrinology & metabolism
Infusions
Subcutaneous
law.invention
03 medical and health sciences
Testosterone blood
0302 clinical medicine
Endocrinology
law
Internal medicine
Testis
medicine
Humans
Inhibins
Testosterone
business.industry
Hypogonadism
fungi
Infant
food and beverages
Micropenis
Luteinizing Hormone
Early infancy
medicine.disease
Recombinant Proteins
Continuous subcutaneous infusion
030104 developmental biology
Pediatrics
Perinatology and Child Health
Recombinant DNA
Androgen insensitivity syndrome
Congenital Hypogonadotropic Hypogonadism
Follicle Stimulating Hormone
Genital Diseases
Male
business
Gonadotropins
hormones
hormone substitutes
and hormone antagonists
Penis
Zdroj: Hormone Research in Paediatrics. 87:103-110
ISSN: 1663-2826
1663-2818
Popis: 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.
Databáze: OpenAIRE
Externí odkaz: