HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text Full Text (PDF) All Versions of this Article: EJE-07-0709v1 158/6/803    most recent Alert me when this article is cited Alert me if a correction is posted Services Similar articles in this journal Similar articles in ISI Web of Science Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Google Scholar Articles by Aksglaede, L. Articles by Juul, A. PubMed PubMed Citation Articles by Aksglaede, L. Articles by Juul, A.

Increased basal and pulsatile secretion of FSH and LH in young men with 47,XXY or 46,XX karyotypes.

University Department of Growth and Reproduction GR, Rigshospitalet, University of Copenhagen, Section 5064, Blegdamsvej 9, DK-2100 Copenhagen Ø, Denmark¹ Department of Internal Medicine, Mayo Clinic, Rochester, 55905 Minnesota, USA and ² Department of Statistics,, University of Virginia, Charlottesville, 22908 Virginia, USA

(Correspondence should be addressed to L Aksglaede; Email: lise.aksglaede{at}rh.hosp.dk)

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 neuroendocrine integrity of the pituitary–gonadal axis in patients with primary testicular failure due to supernumerary X chromosomes.

Design: Cross-sectional study.

Methods: In this study, 7 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 healthy controls participated. Reproductive hormones, testicular size, and overnight LH and 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 h, as estimated by the Weibull analysis. There was no difference between approximate entropy ratios or Weibull analyses indicating comparable orderliness and regularity of LH and FSH secretion. Overnight urinary LH and FSH excretion was significantly elevated in patients compared with 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 h 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.