Gonadotropin secretion in prepubertal and pubertal primary hypogonadism: response to LHRH

Plasma LH and FSH were measured before and after LHRH administration in 10 patients with Turner's syndrome, in 7 with anorchia, in 2 castrates, in 18 with Klinefelter's syndrome, and in 11 prepubertal subjects with unilateral cryptochidism used as a control group. Basal LH was elevated in 4 and basal FSH in 8 of 10 patients with Turner's syndrome. Four patients with anorchia showed elevations of LH while FSH had increased in all of them. The two castrates had normal or slightly increased basal LH and definite elevations of FSH. Prepubertal subjects with Klinefelter's syndrome had normal plasma LH and FSH levels, but showed a marked elevation when they developed puberty. After LHRH administration, mean LH increased by 297% and FSH by 81% in Turner's syndrome, while in anorchia LH increased 757% and FSH 104%. After LHRH administration, patients with unilateral cryptorchidism had an LH increment of 316% and a FSH increment of 164%. Patients with prepubertal Klinefelter's syndrome showed elevations of 261% for LH and 221% for FSH after LHRH treatment. Adolescent subjects with Klinefelter's syndrome had an increment of 352% for LH and only 13% for FSH after LHRH administration. We have concluded that patients without functioning gonads fail to suppress gonadotropin secretion even before puberty while the gonads of the prepubertal Klinefelter's syndrome are able to control LH and FSH release. After puberty, in spite of the hypersection of LH and FSH observed in all subjects with agonadism there is a large pituitary reserve of the gonadotropins. We suggest that the relative inability of pubertal patients with Klinefelter's syndrome to increase FSH after LHRH treatment might be due to the presence of an abnormal compound secreted by the gonads.