P181 - Pseudo-pubertés précoces révélatrices d’un corticosurrénalome malin

Abstract

Trente huit enfants ont été traités pendant au moins 2 ans pour une puberté précoce par un analogue retard du LH-RH. Ce traitement a été bien toléré. Il entraîne une régression des caractères secondaires, une diminution de la vitesse de croissance, un ralentissement de la maturation osseuse, une inhibition de la sécrétion des gonadotrophines hypophysaires et une normalisation des stéroïdes sexuels. La normalisation du rapport âge statural/âge osseux fait espérer une amélioration de la taille définitive.Chronic administration of luteinizing hormone-releasing hormone (LH-RH) is currently considered as the most appropriate therapy of precocious puberty. A delayed release preparation of D-Trp-6-LH-RH (Décapeptyl ® in microcapsules) designed to release the agonist for 28 days after intramuscular injection of 60 μg/kg body wt was given to 12 boys and 26 girls with precocious puberty. Plasma levels of gonadotropins and sex steroids were suppressed to prepubertal levels within 3 weeks, whilst pituitary responses to LH-RH test were almost abolished within 7 weeks.A significant improvement of secondary sex characteristics, as well as gonadal size, was observed within 6 months. Growth velocity (cm/yr, mean ± SD) was reduced in boys and girls from 9.9 ± 3.4 and 9.6 ± 3.3 respectively before treatment to 5.2 ± 1.0 and 5.3 ± 1.3 respectively during the third year of treatment. The mean ratio height age/bone age was just before treatment 0.91 in boys and 0.88 in girls and increased to 1 after 3 yr of treatment. This study shows that Decapeptyl in microcapsules induced a prolonged suppression of gonadal function and a relative blockade of bone maturation. A significant improvement of adult height relative to the effect of conventional treatments should be expected.