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. 2008 Jun;158(6):803-10.
doi: 10.1530/EJE-07-0709. Epub 2008 Mar 5.

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

Lise Aksglaede  1 , Rikke Beck Jensen, Elisabeth Carlsen, Petra Kok, Daniel M Keenan, Johannes Veldhuis, Niels E Skakkebaek, Anders Juul
Affiliations

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

Lise Aksglaede et al. Eur J Endocrinol. 2008 Jun.

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 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 lambda analysis. 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 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.

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Figures

Figure 1
Figure 1
Overnight serum concentration profiles of LH and FSH in (A and C) patients with Klinefelter or SRY-positive XX male syndrome and (B and D) healthy controls showing the actual fit when fast half-life is fixed for LH and fast and slow half-lives for FSH are fixed. AIC denotes Akaike’s information criterion as the statistically objective determinant of pulse number. Asterisks on X-axis defining pulse onsets (nadir preceding the following peak).
Figure 2
Figure 2
Twelve-hour urinary FSH (A) and LH (B) excretion in relation to total LH and FSH secretion by deconvolution in patients with Klinefelter or SRY-positive 46,XX male syndrome (×) and healthy controls (◇).
See this image and copyright information in PMC

References

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