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Increased basal and pulsatile secretion of follicle stimulating hormone and luteinizing hormone in young men with 47,XXY or 46,XX karyotypes.

L Aksglaede, Growth and Reproduction, GR-5064, Rigshopsitalet, Copenahagen, 2100, Denmark
R Jensen, Growth and Reproduction, GR-5064, Rigshospitalet, Copenhagen, Denmark
E Carlsen, Growth and Reproduction, GR-5064, Rigshospitalet, Copenhagen, Denmark
P Kok, Internal Medicine, Mayo Clinic, Rochester, United States
D Keenan, Department of Statistics, University of Virginia, Charlottesville, United States
J Veldhuis, Endocrine Research Unit, Mayo Clinic, Rochester, United States
N Skakkebaek, Growth and Reproduction, GR-5064, Rigshospitalet, Copenhagen, Denmark
A Juul, Growth and Reproduction, GR-5064, Rigshospitalet, Copenhagen, Denmark

Correspondence: Lise Aksglaede, Email: lise.aksglaede{at}rh.hosp.dk

Abstract

Objective: The regulation of normal sexual maturation and reproductive function is dependent on a precise hormonal regulation at hypothalamic, pituitary, and gonadal levels. The aim of this study was to investigate the the pituitary-gonadal axis in patients with primary testicular failure due to supernumerary X chromosomes.

Design: Cross-sectional.

Methods: Seven untreated patients with primary gonadal insufficiency due to SRY-positive 46,XX (n=4) and 46,XXY karyotypes (n=3) aged 18.8 years and 25 age-matched controls. Reproductive hormones, testicular size, and overnight luteinizing hormone (LH) and follicle stimulating hormone (FSH) serum profiles and overnight urine LH and FSH excretion were determined.

Results: Basal LH and FSH secretion was elevated 6.3- and 25.4-fold, respectively, in the patients and the amount of LH and FSH secreted per burst were 2.0- and 6.6-fold elevated. We found significantly more LH but not FSH peaks per 24 hours. There was no difference between approximate entropy ratios or Weibull-gamma analyses indicating comparable orderliness and regularity of LH and FSH secretion. Overnight urinary LH and FSH excretion was significantly elevated in patients compared to controls and correlated significantly with calculated total overnight LH and FSH secretion, respectively, thus validating deconvolution.

Conclusion: In this group of patients with severe hypergonadotropic hypogonadism due to a supernumerary X chromosome higher basal, pulsatile and total LH and FSH secretion were associated with significantly more LH peaks per 24 hours in comparison with healthy controls. Thus, our data indicate that in patients with Klinefelter syndrome and XX-male karyotypes the entire hypothalamic-pituitary-gonadal axis has undergone functional changes.