The Effect of Short-Term Testosterone Administration on Serum FSH, LH and Testosterone Levels: Evidence for Selective Abnormality in LH Control in Patients with Klinefelter's Syndrome

Pt, Capell; Ca, Paulsen; Derleth, Douglas P.; Skoglund, Rodney D.; Plymate, Stephen R.

doi:10.1210/jcem-37-5-752

Cited by 67 publications

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“…Towards mid-puberty, however, there was an evident arrest oftesticular growth, probably due to fibrosis and hyalinization of the seminiferous tubules [2,3,16,26] and subsequently the testes even diminished in size. This was accompanied by a decrease in or complete lack of spermatogenesis, one of the main characteristics of this syndrome [4], with all but one patient having azospermia or severe oligospermia. (10) 6 (10) 5 (10) 5 (5) 3 (5) Thus there was an evident dissociation between the normal progression of the androgen-dependent pubertal signs and testicular development.…”

Section: Discussionmentioning

confidence: 99%

Puberty in 24 patients with Klinefelter syndrome

et al. 1982

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Abstract. Twenty four boys with Klinefelter syndrome, 18 of whom were diagnosed prepubertally, were observed until adulthood. Onset of puberty, as judged from testicular enlargement and pubic hair development, occurred between 11 to 14 years in the above 18 patients. By the age of 17 pubic hair, penile length and height had reached the adult stage in all patients, but arrest of testicular growth was noted at midpuberty, 13 years, with maximal mean (+_ SD) volume attained being 3.5 4-1.5 ml. The first conscious ejaculation was reported to have occurred between 13 to 16 years in 10 patients and in the remaining 4 between 17 to 18 years of age. Sperm counts obtained after the age of 18 revealed azospermia or severe oligospermia in all patients except one, who had a sperm count of 30 • 106/ml. The hypothalamic-pituitary-gonadal axis, assessed by LH-RH and hCG stimulation tests, was found to be normal in prepuberty and during early pubertal stages. From mid-puberty the basal levels of plasma FSH and the response to LH-RIt showed a gradual increase above the normal. Towards late puberty (> 15 years) basal and peak levels of LH were above normal with a concomitant decrease in the basal level of testosterone and its response to hCG.These findings indicate that during childhood and early puberty function of the hypothalamic-pituitary-gonadal axis is normal in Klinefelter syndrome, allowing the onset of pubertal signs at the appropriate age, and that until late puberty there is a relative preservation of function in the testicular Leydig cells, permitting the normal sequential development of the androgen-dependent pubertal signs. The measurement of testicular testosterone reserve by means of hCG stimulation constitutes a useful aid in determining when and if testosterone replacement therapy should be instituted. Key words: Klinefelter syndrome -PubertyThe clinical, hormonal and histological picture of Klinefelter syndrome has been given wide attention in the literature since the original description published in 1942 [13]. Histological studies indicate that testicular damage is already present in the perinatal and prepubertal periods [15,21]. The progression of these changes during puberty has been found to lead to faulty spermatogenesis [10] as well as a decrease in testosterone production and secretion by the testes [14,31] with signs of hypovirilization in most of these patients [8].Reprint requests to: Prof. Z. Laron, Institute of Pediatric and Adolescent Endocrinology, Beilinson Medical Center, Petah Tikva 49 100, Israel Presented here are the findings made during long-term follow-up in 24 patients with Klinefelter syndrome, most of whom had been diagnosed before puberty. It was the aim of this study to investigate the sequence of the clinical and hormonal changes taking place and their inter-relationships, before and during puberty. Patients and MethodsThe patient material comprised 24 patients in all of whom the diagnosis of Klinefelter syndrome was confirmed by chromosomal studies. In 18, diagnosis was made before ...

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Section: Discussionmentioning

confidence: 99%

Puberty in 24 patients with Klinefelter syndrome

et al. 1982

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“…In this connection we think that our results do not definitely exclude the occurrence of an annual PRL or LH rhythm in KS. However, the comparison versus con trols, investigated by the same protocol, leads us to suggest the following considerations: (I) primary gonadal failure changes, but does not obliterate the circannual T and FSH rhythmicity as demonstrated by others for the circadian T rhythmicity [20] and for circadian, ultradian, and infradian gonadotropin rhythmicity [3,6,12,13,[26][27][28]; (2) the cir cannual T rhythm in KS seems unrelated to the LH or FSH rhythms because the first is not found and the second pre sents a phase difference which does not strenghten a tem poral relationship or a common synchronizing mechanism: (3) the lack of a circannual LH rhythm in KS seems to con firm a selective abnormality in the T-LH feedback mecha nism in this syndrome as already suggested by some au thors [1, 5,9], and it could be related to the desynchronizing effect of altered T metabolism and/or to a central disorder in hypothalamic-pituitary regulation involving the circan nual hormone rhythmicity. This last assumption seems also to be stressed by the disappearance of circannual TSH rhythm recently evidenced in KS [7]; (4) finally, with re spect to clinical implications, we think useful, in modulat ing the treatment of this syndrome, to aim also at restoring the circannual hormone rhythmicity.…”

Section: Discussionmentioning

confidence: 83%

“…In Klinefel ter's syndrome (KS) an altered secretion of T, LH. FSH, and PRL has been found in both basal and dynamic condi tions [1,5,9,14], whereas no consistent changes in circadian and/or ultradian periodicity are referred [19,21,28]. The aim of this study was to ascertain the circannual pattern of secretion of LH, FSH, T and PRL in adult males with pri mary testicular failure secondary to Klinefelter's syndrome and to compare this to that found in normal age-matched males.…”

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confidence: 97%

Circannual Variations of Plasma Testosterone, Luteinizing Hormone, Follicle-Stimulating Hormone and Prolactin in Klinefelter’s Syndrome

Bellastella¹,

Criscuolo²,

Sinisi³

et al. 1986

Neuroendocrinology

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To clarify the influence of primary testicular failure upon circannual hormone rhythmicity we have been studying, by cross-sectional design, 93 adult patients with Klinefelter’s syndrome (KS) and 64 adult healthy males to look for evidence of circannual rhythms in testosterone (T), LH, FSH and PRL plasma concentrations. Plasma samples were taken from 08.00 to 09.00 h and all hormones were measured by radioimmunoassay. The data were assessed by the single cosinor method in order to obtain evidence for any significant rhythm and to estimate its parameters mesor, amplitude and acrophase. In the controls, annual rhythms were validated for the secretion of T (annual crest time in September), LH (annual crest time in February) and FSH (annual crest time in January), whereas PRL did not show a significant annual rhythm. In KS, significant circannual rhythms were validated for the secretion of T (annual crest time in April) and FSH (annual crest time in September), but not for LH and PRL secretion. Our results suggest that in KS the circannual hormone rhythmicity may be influenced by seminiferous tubule dysgenesis.

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“…It has been suggested that in patients with Klinefelter’s syndrome, the markedly elevated levels of gonadotrophins in the presence of low levels of testosterone is attributed to a reduced sensitivity of the hypothalamic-pituitary axis to testosterone negative feedback [6, 7, 9]. However, whether an oestrogen positive feedback is concurrently present in Klinefelter’s syndrome remains controversial.…”

Section: Discussionmentioning

confidence: 99%

“…In most patients, levels of FSH and LH are high while testosterone levels are either below or within the lower range of normal men [1]. Studies conducted so far seem to suggest that the reason for the high FSH and LH levels is due, in part, to the reduced sensitivity of the hypothalamic-pituitary axis to the negative feedback effect of testosterone [6, 7, 8, 9]. However, it remains controversial whether a reduced sensitivity to the testosterone-negative feedback has led to the activation of the positive oestrogen feedback in Klinefelter’s syndrome.…”

Section: Introductionmentioning

confidence: 99%

Does a Positive Oestrogen Feedback on the Hypothalamic-Pituitary Axis Exist Concurrently with a Defective Testosterone Feedback in Klinefelter’s Syndrome?

Goh

Lee²

1998

Horm Res Paediatr

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The oestrogen provocation and GnRH challenge tests were carried out in 5 subjects with Klinefelter’s syndrome who had no history of previous hormonal therapy. The oestrogen provocation test consisted of an intramuscular injection of 10 mg of oestradiol valerate and blood samples were collected daily from day 0 to day 5. In 2 subjects, three GnRH challenge tests were carried out before (day 0) and on days 2 and 4 after the intramuscular injection of oestradiol valerate. During each GnRH challenge test, 7 blood samples were collected at 30-min intervals, 30 min and immediately before and for 150 min after the bolus dose of GnRH. Plasma concentrations of FSH, LH, testosterone and oestradiol were measured by established radioimmunoassays. Plasma levels of oestradiol rose significantly a day following the intramuscular injection of 10 mg of oestradiol valerate, reaching a peak on day 2 and then falling significantly to lower levels by days 4 and 5, although these levels were still significantly higher than the corresponding baseline levels. In the presence of high levels of oestradiol, the high basal levels of FSH were significantly suppressed, and remained suppressed throughout the 5 days of the study. LH, on the other hand, had a biphasic response; an initial significant suppression by day 1 persisting to day 3, but by days 4 and 5 a rebound in basal LH levels was noted. However, the levels on day 5 were not significantly higher than baseline levels. The pituitary responsiveness as far as the LH and FSH secretions were concerned reflected the baseline levels. The results of the present study refuted the claim that a positive oestrogen feedback exists in men including Klinefelter’s syndrome as a result of the removal of or reduced testosterone. In addition, the attenuated testosterone feedback in Klinefelter’s syndrome is responsible for the greatly amplified pituitary responsiveness to the trophic action of GnRH and this, in part, may be responsible for the elevated levels of FSH and LH seen in such patients.

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The Effect of Short-Term Testosterone Administration on Serum FSH, LH and Testosterone Levels: Evidence for Selective Abnormality in LH Control in Patients with Klinefelter's Syndrome

Cited by 67 publications

References 0 publications

Puberty in 24 patients with Klinefelter syndrome

Puberty in 24 patients with Klinefelter syndrome

Circannual Variations of Plasma Testosterone, Luteinizing Hormone, Follicle-Stimulating Hormone and Prolactin in Klinefelter’s Syndrome

Does a Positive Oestrogen Feedback on the Hypothalamic-Pituitary Axis Exist Concurrently with a Defective Testosterone Feedback in Klinefelter’s Syndrome?

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