[Evaluation of gonadotropic and gonadal secretions in primary abnormalities of the gonads and male pseudohermaphrodism before and after the age of puberty].

Blood levels of gonadotropins (FSH and LH) and gonadal steroids (testosterone or estradiol) have been evaluated in 72 children and adolescents with primary gonadal defects (45 agonadal patients, 7 with asymetrical gonadal dysgenesis, 9 with Klinefelter syndrome, 4 with partial testicular dysgenesis and 7 with partial ovarian dysgenesis) and in 17 cases of male pseudohermaphrodism. A LH-RH test has been performed in most cases, and a testicular stimulation test with chorionic gonadotropin in patients with testicular tissue. Agonadal subjects had increased blood and pituitary releasable gonadotropins: very high in infants and young children, much less from 7 to 11 years, a high spurt being observed at 12 years. This diphasic pattern relates to the varying sensitivity of hypothalamic receptors and suggests that adrenal steroids may restrain gonadotropic secretion at 7-8 years. As a diagnostic tool, the increase of gonadotropins may be missing in the 7-11 years group. In the 1-12 years patients with partially defective gonads, blood and pituitary releasable gonadotropins and blood steroids are usually normal, but testosterone response to chorionic gonadotropin may be already blunted. From 13 years the gonadotropic secretion is usually increased, even when testosterone secretion and reserve are within normal range. Most male pseudohermaphrodites showed normal gonadotropic and testicular secretions. But four patients had highly increased responses to LH-RH, suggesting a defect of testicular secretion or of receptors, and demonstrating some heterogeneity in the male pseudohermaphrodism group.