ADRENAL AND GONADAL CONTRIBUTION TO ANDROGEN AND OESTROGEN EXCRETION IN KLINEFELTER'S SYNDROME

1973 ◽  
Vol 72 (1) ◽  
pp. 182-190 ◽  
Author(s):  
Anders Frøland ◽  
V. Aasted Frandsen ◽  
Svend G. Johnsen
Keyword(s):  
Klinefelter’S Syndrome ◽  
Klinefelter's Syndrome ◽  
Adrenal Androgen ◽  
Comparable Group ◽  
Correlation Studies ◽  
Androgen Synthesis ◽  
The Difference ◽  
Normal Men ◽  
Steroid Excretion ◽  
Dexamethasone Suppression

ABSTRACT Dexamethasone suppression tests were carried out in a group of 42 patients with Klinefelter's syndrome having a 47,XXY karyotype. Urinary androgen excretion obtained during suppression was considered to represent the gonadal contribution. The difference between pre-suppression values and suppression values was interpreted as the adrenal contribution to sex steroid excretion. It was shown that not only was the gonadal androgen production much lower in Klinefelter's syndrome than in a comparable group of normal men, but the adrenal androgen synthesis was also significantly reduced. Adrenal dehydroepiandrosterone as against adrenal androsterone plus etiocholanolone was positively correlated in Klinefelter's syndrome in contrast to normal men. This is taken as an indication of a qualitatively altered synthesis of adrenal sex hormones in the patient group. The oestrogen excretion was not quantitatively altered in Klinefelter's syndrome when compared with normal men. Correlation studies showed that in Klinefelter's syndrome adrenal oestrogens and adrenal DHA were uncorrelated contrary to the findings in normal men. This points towards an abnormal adrenal oestrogen synthesis in Klinefelter's syndrome.

ANALYSIS OF A DEXAMETHASONE / CHORIONIC GONADOTROPHIN TEST OF ADRENO-GONADAL FUNCTION IN NORMAL MEN AS COMPARED WITH DEXAMETHASONE TEST IN NORMAL WOMEN

1971 ◽  
Vol 66 (4) ◽  
pp. 587-605 ◽  
Author(s):  
Svend G. Johnsen ◽  
Peter Christiansen ◽  
V. Aasted Frandsen ◽  
Anders Frøland ◽  
Jan Nielsen
Keyword(s):  
Chorionic Gonadotrophin ◽  
Sex Hormone ◽  
Reserve Capacity ◽  
Adrenal Androgen ◽  
Mutual Correlation ◽  
Normal Limits ◽  
The Difference ◽  
Normal Women ◽  
Normal Men ◽  
Dexamethasone Suppression

ABSTRACT 42 normal men aged 20–40 years were subjected to a functional test involving chorionic gonadotrophin (HCG) stimulation performed during dexamethasone suppression of the adrenals and with determinations of urinary androgen and oestrogen metabolites. For comparison, dexamethasone suppression was performed in 21 normal women aged 20–40 years. Steroids of adrenal origin were determined as the difference of values before and during dexamethasone suppression, and testicular reserve capacity as the difference before and after HCG. Furthermore, some ratios between steroids were calculated, and a total of 27 steroid parameters were obtained. Normal means and normal limits are given for all these parameters. All parameters were subjected to mutual correlation analyses. These showed, among several other findings, the following: Leydig cell reserve capacity for androgen falls significantly with age; adrenal and gonadal sex hormone productions are independent of each other in men but not in women; men have a significant adrenal androgen production which is not related to the adrenal production of dehydroepiandrosterone; testicular reserve capacity is not correlated with the spontaneous testicular steroid production; a high correlation exists between dehydroepiandrosterone and oestrogen excretion in men; the testicular reserve capacities for androgen and oestrogen are completely independent of each other. From these and other findings, sex hormone relationships and particularly the origin of oestrogens in men are discussed.

THE EFFECT OF OESTROGEN ADMINISTRATION ON PLASMA TESTOSTERONE, FSH AND LH LEVELS IN PATIENTS WITH KLINEFELTER'S SYNDROME AND NORMAL MEN

1974 ◽  
Vol 77 (4) ◽  
pp. 765-783 ◽  
Author(s):  
A. G. H. Smals ◽  
P. W. C. Kloppenborg ◽  
R. M. Lequin ◽  
Th. J. Benraad
Keyword(s):  
Normal Subjects ◽  
Plasma Testosterone ◽  
Klinefelter’S Syndrome ◽  
Klinefelter's Syndrome ◽  
Testosterone Levels ◽  
Control Subjects ◽  
Dose Dependent Decrease ◽  
Normal Males ◽  
Normal Men ◽  
Dose Dependent

ABSTRACT In 6 eugonadal males and 6 patients with Klinefelter's syndrome the effect of increasing amounts of ethinyloestradiol (EE) (15, 30 and 150 μg daily for 7 days) on plasma levels of LH, FSH and testosterone was studied. Control levels of LH and FSH in the Klinefelter patients were significantly higher than in the normal males, whereas plasma testosterone levels were significantly lower. In 3 of the 6 Klinefelter patients plasma gonadotrophin levels were clearly elevated despite normal plasma testosterone concentrations. After EE administration a dose-dependent decrease of plasma FSH and testosterone levels was observed in both the control subjects and the Klinefelter patients, whereas the LH decrease was dose-dependent in the Klinefelter patients, but not however, in the eugonadal males. Despite significant testosterone suppression plasma LH and FSH levels in the Klinefelter patients remained supranormal when compared with the levels of the control subjects. Amounts of EE, roughly equivalent to the physiological oestrogen production (15 μg of EE daily) in men, decreased plasma LH and testosterone levels in the normal males, not however, in the Klinefelter patients. The suppression of plasma testosterone by EE in both the normal subjects and the Klinefelter patients could readily be overcome by exogenous gonadotrophin administration, favouring the concept that the EE induced testosterone decrease is predominantly gonadotrophin mediated. It is concluded that small amounts of oestrogens play a role in the pituitary-gonadal axis in normal males. Although higher doses are needed to modulate this axis in Klinefelter's syndrome, the hypothalamic-pituitary-gonadal feedback in this disorder is still operative, though at a higher setting.

KLINEFELTER'S SYNDROME: EFFECTS OF OESTROGEN ON GROWTH HORMONE, PROLACTIN AND THYROTROPHIN RELEASE, AND ON THYROTROPHIN AND PROLACTIN RESPONSES TO THYROTROPHIN-RELEASING HORMONE

1979 ◽  
Vol 92 (2) ◽  
pp. 347-357 ◽  
Author(s):  
Antonino Barbarino ◽  
Laura De Marinis
Keyword(s):  
Growth Hormone ◽  
Normal Male ◽  
Klinefelter’S Syndrome ◽  
Klinefelter's Syndrome ◽  
Serum Growth Hormone ◽  
Oestrogen Treatment ◽  
Thyrotrophin Releasing Hormone ◽  
Trh Stimulation ◽  
Control Serum ◽  
Normal Men

ABSTRACT In 4 normal men and 8 patients with Klinefelter's syndrome the effects of oestradiol-17 β (15μg/kg daily for 8–13 days) on serum levels of pituitary hormones were investigated. Control levels of gonadotrophins in the Klinefelter patients were significantly higher than in the normal males, whereas serum testosterone (T) levels were lower. Oestradiol induced a decrease in serum gonadotrophin concentrations in both the control subjects and the Klinefelter patients, whereas a testosterone suppression was observed in the normal subjects, but not in the Klinefelter patients. Control serum growth hormone (hGH), and prolactin (hPRL) levels were of comparable magnitude in both groups and significantly increased during oestradiol administration. Serum thyrotrophin (TSH) levels were normal before and during oestrogen treatment. Basal serum oestradiol levels were within the normal male range, and were increased during treatment. Prolactin and thyrotrophin responsiveness to TRH stimulation was examined in Klinefelter patients before and during oestrogen administration. Before treatment, hPRL responses to TRH were higher than those observed in normal men. During oestrogen treatment hPRL responses to TRH were significantly increased when compared to those observed before treatment. TSH responses to TRH were normal both before and during treatment. These studies indicate that in patients with Klinefelter's syndrome pharmacological doses of oestrogen induce different effects on the hypothalamic-pituitary axis, as regards the release of gonadotrophins, prolactin, thyrotrophin, and growth hormone, similar to those observed in normal men. This fact supports the conclusion that in Klinefelter's syndrome the abnormality in the pituitary-gonadal feedback mechanism is selectively confined to the testosterone feedback control of gonadotrophin secretion. Finally, in patients with Klinefelter's syndrome, oestrogen is capable of inducing a significant increase of the hPRL response to TRH stimulation.

Indirect evidence of chronic Leydig cell desensitization in Klinefelter's syndrome

1981 ◽  
Vol 96 (4) ◽  
pp. 552-556 ◽  
Author(s):  
Anthony G. Smals ◽  
Gerlach F. Pieters ◽  
Peter W. Kloppenborg
Keyword(s):  
Leydig Cells ◽  
Response Pattern ◽  
Indirect Evidence ◽  
Chorionic Gonadotrophin ◽  
Human Chorionic Gonadotrophin ◽  
Klinefelter’S Syndrome ◽  
Klinefelter's Syndrome ◽  
Basal Plasma ◽  
Normal Men ◽  
T Values

Abstract. The basal plasma 17α-hydroxyprogesterone (17-OHP) and testosterone (T) levels were proportionally decreased in 10 hypergonadotropic patients with Klinefelter's syndrome. The ratio 17-OHP to T was however about twice as high as in 10 eugonadal male controls, suggesting the presence of a block in the conversion of 17-hydroxylated steroids to androgens in the Klinefelter patients under basal circumstances. Administration of human chorionic gonadotrophin (hCG, 1500 IU im daily for 3 days) to the Klinefelter patients disclosed a response pattern quite different from that observed in controls. In the control subjects 17-OHP and the ratio 17-OHP/T sharply rose to maximum values at 24 h after the first injection. Thereafter both progressively fell to lowest values at 72 h, when T levels reached their maximum. In the Klinefelter patients the T response to hCG administration was greatly diminished but the 17-OHP response was similar to that in the controls. Maximum 17-OHP and 17-OHP/T values however were not achieved until 72 h after the first injection when T levels also reached their maximum. Unlike in the controls in the Klinefelter patients maximum 17-OHP and T increments and the 17-OHP and T levels 48 and 72 h after the injection were positively correlated. Together the findings of a decreased T synthesis and reserve in the presence of relative 17-OHP accumulation, further increasing after acute hCG administration in a pattern quite different from that in normal men, suggest that in Klinefelter's syndrome the Leydig cells may be chronically desensitized by the persistent endogenous hypergonadotropism.

SIMPLIFIED DEXAMETHASONE SUPPRESSION TEST

1969 ◽  
Vol 61 (2) ◽  
pp. 219-231 ◽  
Author(s):  
V. H. Asfeldt
Keyword(s):  
Cushing’S Syndrome ◽  
Normal Subjects ◽  
Cushing's Syndrome ◽  
Dexamethasone Suppression Test ◽  
Clinical Value ◽  
Suppression Test ◽  
The One ◽  
Steroid Excretion ◽  
Dexamethasone Suppression

ABSTRACT This is an investigation of the practical clinical value of the one mg dexamethasone suppression test of Nugent et al. (1963). The results, evaluated from the decrease in fluorimetrically determined plasma corticosteroids in normal subjects, as well as in cases of exogenous obesity, hirsutism and in Cushing's syndrome, confirm the findings reported in previous studies. Plasma corticosteroid reduction after one mg of dexamethasone in cases of stable diabetes was not significantly different from that observed in control subjects, but in one third of the insulin-treated diabetics only a partial response was observed, indicating a slight hypercorticism in these patients. An insufficient decrease in plasma corticosteroids was observed in certain other conditions (anorexia nervosa, pituitary adenoma, patients receiving contraceptive or anticonvulsive treatment) with no hypercorticism. The physiological significance of these findings is discussed. It is concluded that the test, together with a determination of the basal urinary 17-ketogenic steroid excretion, is suitable as the first diagnostic test in patients in whom Cushing's syndrome is suspected. In cases of insufficient suppression of plasma corticosteroids, further studies, including the suppression test of Liddle (1960), must be carried out.

Klinefelter's syndrome (47,XXY) in male systemic lupus erythematosus

2019 ◽  
Author(s):  
Hela Marmouch ◽  
Haythem Jenzri ◽  
Houssem Mrabet ◽  
Hamza Fekih ◽  
Ines Khochtali
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Klinefelter’S Syndrome ◽  
Klinefelter's Syndrome ◽  
Systemic Lupus
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