Quantitative and ultrastructural study of Leydig cells in klinefelter's syndrome

M. Nistal; L. Santamaria; R. Paniagua

doi:10.1002/path.1711460405

HYDROXYSTEROID DEHYDROGENASES IN NORMAL AND ABNORMAL HUMAN TESTES

Journal of Endocrinology ◽

10.1677/joe.0.0350239 ◽

1966 ◽

Vol 35 (3) ◽

pp. 239-NP ◽

Cited By ~ 24

Author(s):

A. H. BAILLIE ◽

W. S. MACK

Keyword(s):

Similar Pattern ◽

Leydig Cells ◽

Seminiferous Tubules ◽

Klinefelter’S Syndrome ◽

Klinefelter's Syndrome ◽

Colour Reaction ◽

Adult Human ◽

Hydroxysteroid Dehydrogenases

SUMMARY 3α-, 3β-, 11β-, 16β-, 17β- and 20β-hydroxysteroid dehydrogenases have been localized histochemically in the Leydig cells of prepubertal and adult human testes; 3α-, 16β- and 17β-hydroxysteroid dehydrogenases were present in the seminiferous tubules also. A similar pattern was found in cryptorchid testes. In addition 3β-sulphoxy steroids, including DHA sulphate, gave a good colour reaction in human Leydig cells. Testes from oestrogen-treated subjects had no histochemically demonstrable hydroxysteroid dehydrogenases and this applied also to infarcted testes. Testes from a case of Klinefelter's syndrome were found to lack 17β- and 20β-hydroxysteroid dehydrogenases in the Leydig cells. The biochemical significance of these results is discussed.

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Immunohistochemical and quantitative study of interstitial and intratubular leydig cells in normal men, cryptorchidism, and Klinefelter's syndrome

The Journal of Pathology ◽

10.1002/path.1711640405 ◽

1991 ◽

Vol 164 (4) ◽

pp. 299-306 ◽

Cited By ~ 36

Author(s):

J. Regadera ◽

J. Codesal ◽

R. Paniagua ◽

P. Gonzalez-Peramato ◽

M. Nistal

Keyword(s):

Quantitative Study ◽

Leydig Cells ◽

Klinefelter’S Syndrome ◽

Klinefelter's Syndrome ◽

Normal Men

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47,XXY Klinefelter's Syndrome with Low FSH and LH Levels and Absence of Leydig Cells

Andrologia ◽

10.1111/j.1439-0272.1980.tb01693.x ◽

2009 ◽

Vol 12 (5) ◽

pp. 426-433 ◽

Cited By ~ 9

Author(s):

M. NISTAL ◽

R. PANIAGUA ◽

M.A. ABAURREA ◽

L.F. PALLARDO

Keyword(s):

Leydig Cells ◽

Klinefelter’S Syndrome ◽

Klinefelter's Syndrome

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Ultrastructure of Leydig cells in Klinefelter’s syndrome with 48, XXYY karyotype

Virchows Archiv B Cell Pathology ◽

10.1007/bf02889055 ◽

1978 ◽

Vol 28 (1) ◽

pp. 39-46

Author(s):

Manuel Nistal ◽

Ricardo Paniagua ◽

Isidora López-Pajares

Keyword(s):

Leydig Cells ◽

Klinefelter’S Syndrome ◽

Klinefelter's Syndrome

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

Acta Endocrinologica ◽

10.1530/acta.0.0960552 ◽

1981 ◽

Vol 96 (4) ◽

pp. 552-556 ◽

Cited By ~ 12

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.

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THE EFFECT OF GONADOTROPHIN RELEASING HORMONE ON PITUITARY-GONADAL FUNCTION IN KLINEFELTER'S SYNDROME

Acta Endocrinologica ◽

10.1530/acta.0.0830829 ◽

1976 ◽

Vol 83 (4) ◽

pp. 829-838 ◽

Cited By ~ 20

Author(s):

A. G. H. Smals ◽

P. W. C. Kloppenborg ◽

R. M. Lequin ◽

Th. J. Benraad

Keyword(s):

Leydig Cells ◽

Bolus Injection ◽

Plasma Testosterone ◽

Klinefelter’S Syndrome ◽

Klinefelter's Syndrome ◽

Releasing Hormone ◽

Basal Plasma ◽

Gonadotrophin Releasing Hormone ◽

The Mean ◽

Lh Rh

ABSTRACT The mean basal plasma LH and FSH levels in 8 patients with Klinefelter's syndrome were respectively 5 and 15-fold higher than in 8 eugonadal males, whereas plasma testosterone concentration were half the normal value After an intravenous bolus injection of gonadotrophin releasing hormone (100 μg of LH-RH) the gonadotrophin increase in the Klinefelter patients was more marked than in the control subjects, but in both groups the plasma testosterone levels remained essentially unchanged. In contrast to the bolus injection, an 8 h infusion of LH-RH after the bolus elicited a significant plasma testosterone increase in both the eugonadal males (59%) and the Klinefelter patients (51%). These findings indicate that despite an impressive endogenous hypergonadotrophism, Leydig cells in Klinefelter's syndrome can still respond to a sustained further increase of these endogenous gonadotrophins and thus still have functional reserve.

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