Article (Scientific journals)
Quand proposer une gonadectomie chez les patientes présentant une insensibilité complète aux androgènes ?
Vereecken, Thibaut; Parent, Anne-Simone; Van Linthout, Christine et al.
2022In Revue Médicale de Liège, 77 (12), p. 728-732
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Keywords :
Anti-Mullerian Hormone; Humans; Male; Female; Androgen-Insensitivity Syndrome/genetics/surgery/complications; Anti-Mullerian Hormone/genetics; Neoplasms; Mutation; Androgen insensitivity syndrome; Germinal tumor; Gonadectomy; Hormone replacement therapy; Disorders of sexual development
Abstract :
[en] Complete androgen insensitivity syndrome is the most frequent cause of disorder of sexual development in 46 XY patients. It is caused by mutations of the AR gene coding for the androgen receptor. Transmission is X-linked and mutations are most of the time inherited. It leads to a complete lack of response to androgen resulting in the presence of female external genitalia in 46 XY patients, normal but undescended testes and lack of female internal genitalia due to the secretion of anti-Müllerian hormone by male gonads. Traditionally, gonadectomy was proposed before puberty to decrease the risk of gonadal malignancy. However, more recent studies underlined the benefits of postponing gonadectomy until after pubertal development. Benefits of deferred gonadectomy are spontaneous pubertal development through peripheral aromatization of testosterone into oestrogens and the chance for the patient to have an active role in the decision-making process. After gonadectomy, hormone replacement therapy is required in order to prevent complications due to hypogonadism such as osteoporosis, cardiovascular diseases and a reduction of life expectancy.
Disciplines :
Endocrinology, metabolism & nutrition
Reproductive medicine (gynecology, andrology, obstetrics)
Pediatrics
Author, co-author :
Vereecken, Thibaut ;  Centre Hospitalier Universitaire de Liège - CHU ; Assistant en Médecine générale.
Parent, Anne-Simone ;  Centre Hospitalier Universitaire de Liège - CHU > > Service de pédiatrie ; Service de Pédiatrie, CHR Citadelle, Liège, Belgique.
Van Linthout, Christine ;  Centre Hospitalier Universitaire de Liège - CHU > > Service de gynécologie-obstétrique
Laterre, Marie ;  Centre Hospitalier Universitaire de Liège - CHU > > Service de génétique
Fudvoye, Julie ;  Université de Liège - ULiège > GIGA ; Service de Pédiatrie, CHR Citadelle, Liège, Belgique.
NECHIFOR, Vlad Andrei ;  Centre Hospitalier Universitaire de Liège - CHU > > Service d'urologie
Demarche, M;  Service de Chirurgie générale, CHU Liège, Belgique.
Pintiaux, Axelle ;  Centre Hospitalier Universitaire de Liège - CHU > > Service de gynécologie-obstétrique
Language :
French
Title :
Quand proposer une gonadectomie chez les patientes présentant une insensibilité complète aux androgènes ?
Alternative titles :
[en] Timing of gonadectomy in patients with complete androgen insensitivity syndrome.
Publication date :
December 2022
Journal title :
Revue Médicale de Liège
ISSN :
0370-629X
eISSN :
2566-1566
Publisher :
Université de Liège. Revue Médicale de Liège, Liège, Be
Volume :
77
Issue :
12
Pages :
728-732
Peer reviewed :
Editorial Reviewed verified by ORBi
Available on ORBi :
since 17 February 2023

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