0O21-972X/78/4705-1144$O2.0O/0 Journal of Clinical Endocrinology and Metabolism Copyright © 1978 by The Endocrine Society
Vol. 47, No. 5 Printed in U.S.A.
Basal and Human Chorionic Gonadotropin-Stimulated 17a-Hydroxyprogesterone and Testosterone Levels in Klinefelter's Syndrome A. G. H. SMALS, P. W. C. KLOPPENBORG, G. F. F. M. PIETERS, D. C. LOSEKOOT, AND Th. J. BENRAAD Department of Medicine, Division of Endocrinology, University of Nijmegen, Nijmegen, The Netherlands ABSTRACT. In nine patients with Klinefelter's syndrome, mean basal plasma levels of testosterone (302 ± 145 ng/100 ml) and its major precursor, 17a-hydroxyprogesterone (17-OHP; 86 ± 46 ng/100 ml), were significantly lower (P 0.10).
The Endocrine Society. Downloaded from press.endocrine.org by [${individualUser.displayName}] on 13 November 2015. at 05:14 For personal use only. No other uses without permission. . All rights reserved.
COMMENTS enzymic synthesis of testosterone in Klinefelter's syndrome. Prolonged hCG stimulation might then be needed to activate the later enzymic steps in the production of testosterone. In fact, the latter hypothesis is supported by our finding of normal basal 17-OHP to testosterone ratios in all of these patients with endogenous hypergonadotropism. Increased precursor availability due to short term exogenous hCG stimulation showed that the biosynthetic machinery distal to 17-OHP may be rate limiting in the production of testosterone in Klinefelter's syndrome.
Acknowledgments We are indebted to Mrs. S. van der Werf for preparation of the manuscript and to Mr. E. Wilms and L. H. Coenen for the assays of testosterone.
1147
5. SMALS, A. G. H., P. W. C. KLOPPENBORO, AND TH. .J. BEN-
RAAD, The effect of short and long term human chorionic gonadotropin (HCG) administration on plasma testosterone levels in Klinefelter's syndrome, Ada Knriocrin.nl (Kbh) 77: 753, 1974. 6. SMALS, A. G. H., P. W. C. KLOPPENBORG, AND TH. J. BEN-
RAAD, Body proportions and androgenicity in relation to plasma testosterone levels in Klinefelter's syndrome, Ada Endocrinol (Kbh) 77: 387, 1974. 7. RUDER, H. J., D. L. LORIAUX, R. J. SHERINS, AND M. B.
LIPSETT, Leydig cell function in men with disorder of spermatogenesis, ,1 Clin Rnriocrinol Metab 38: 244, 1974. 8. SMALS, A. G. H., P. W. C. KLOPPENBORG, AND TH. .J. BEN-
RAAD, Plasma testosterone in Klinefelter's syndrome: diurnal variation and response to ACTH and dexamethasone, Ada Rnriocrinol (Kbh) 78: 604, 1975. 9. WANG, C, H. W. G. BAKER, H. G. BURGER, D. M. DE KRET-
SER, AND B. HUDSON, Hormonal studies in Klinefelter's syndrome, Clin Rnriocrinol 4: 399, 1975. 10. MAHOUDEAU, J. A., J. VALCKF,, AND H. BRICAIRE, Dissociated
response of plasma testosterone and estradiol to human chorionic gonadotropin in adult men, >1 Clin Rnriocrinol Metab 41: 13, 1975. 11. SMALS, A. G. H., P. W. C. KLOPPENBORG, R. M. LF.quiN, AND
TH. J. BENRAAD, The effect of gonadotrophin releasing hormone on pituitary-gonadal function in Klinefelter's syndrome, Ada Rnriocrinol (Kbh) 83: 829, 1976. 12. STROTT, C. A., T. YOSHIMI, AND M. B. LIPSETT, Plasma
References 1. LIPSF.TT, M. B., T. E. DAVIS, H. WILSON, AND C. J. CANFIELD,
Testosterone production in chromatine-positive Klinefelter's syndrome, J Clin Rnriocrinol Metab 25: 1027, 1965. 2. PAULSEN, C. A., D. L. GORDON, R. W. CARPENTER, H. M.
CANDY, AND W. I). DRUCKER, Klinefelter's syndrome and its
variants: a hormonal and chromosomal study, Recent Prog Hnrm Res 24: 321, 1968. 3. WKINSTKIN, R. L., S. L. KAPLAN, AND M. M. GRUMBACH,
Gonadal and pituitary insensitivity to HCG and clomiphene stimulation in Klinefelter's syndrome. Program of the 5.3rd Meeting of The Endocrine Society, Ran Francisco, 1971, p. A 102 (Abstract. 119). 4. STEWART-BENTLEY, M., AND R. HORTON, Leydig cell function in Klinefelter's syndrome, Metabolism 22: 875, 1974.
progesterone and 17-hydroxyprogesterone in normal men and children with congenital hyperplasia, J Clin Invest 48: 930, 1969. 13. VERMEULEN, A., AND L. VERDONCK, Radioimmunoassay of
17/
Vol. 47, No. 5 Printed in U.S.A.
Basal and Human Chorionic Gonadotropin-Stimulated 17a-Hydroxyprogesterone and Testosterone Levels in Klinefelter's Syndrome A. G. H. SMALS, P. W. C. KLOPPENBORG, G. F. F. M. PIETERS, D. C. LOSEKOOT, AND Th. J. BENRAAD Department of Medicine, Division of Endocrinology, University of Nijmegen, Nijmegen, The Netherlands ABSTRACT. In nine patients with Klinefelter's syndrome, mean basal plasma levels of testosterone (302 ± 145 ng/100 ml) and its major precursor, 17a-hydroxyprogesterone (17-OHP; 86 ± 46 ng/100 ml), were significantly lower (P 0.10).
The Endocrine Society. Downloaded from press.endocrine.org by [${individualUser.displayName}] on 13 November 2015. at 05:14 For personal use only. No other uses without permission. . All rights reserved.
COMMENTS enzymic synthesis of testosterone in Klinefelter's syndrome. Prolonged hCG stimulation might then be needed to activate the later enzymic steps in the production of testosterone. In fact, the latter hypothesis is supported by our finding of normal basal 17-OHP to testosterone ratios in all of these patients with endogenous hypergonadotropism. Increased precursor availability due to short term exogenous hCG stimulation showed that the biosynthetic machinery distal to 17-OHP may be rate limiting in the production of testosterone in Klinefelter's syndrome.
Acknowledgments We are indebted to Mrs. S. van der Werf for preparation of the manuscript and to Mr. E. Wilms and L. H. Coenen for the assays of testosterone.
1147
5. SMALS, A. G. H., P. W. C. KLOPPENBORO, AND TH. .J. BEN-
RAAD, The effect of short and long term human chorionic gonadotropin (HCG) administration on plasma testosterone levels in Klinefelter's syndrome, Ada Knriocrin.nl (Kbh) 77: 753, 1974. 6. SMALS, A. G. H., P. W. C. KLOPPENBORG, AND TH. J. BEN-
RAAD, Body proportions and androgenicity in relation to plasma testosterone levels in Klinefelter's syndrome, Ada Endocrinol (Kbh) 77: 387, 1974. 7. RUDER, H. J., D. L. LORIAUX, R. J. SHERINS, AND M. B.
LIPSETT, Leydig cell function in men with disorder of spermatogenesis, ,1 Clin Rnriocrinol Metab 38: 244, 1974. 8. SMALS, A. G. H., P. W. C. KLOPPENBORG, AND TH. .J. BEN-
RAAD, Plasma testosterone in Klinefelter's syndrome: diurnal variation and response to ACTH and dexamethasone, Ada Rnriocrinol (Kbh) 78: 604, 1975. 9. WANG, C, H. W. G. BAKER, H. G. BURGER, D. M. DE KRET-
SER, AND B. HUDSON, Hormonal studies in Klinefelter's syndrome, Clin Rnriocrinol 4: 399, 1975. 10. MAHOUDEAU, J. A., J. VALCKF,, AND H. BRICAIRE, Dissociated
response of plasma testosterone and estradiol to human chorionic gonadotropin in adult men, >1 Clin Rnriocrinol Metab 41: 13, 1975. 11. SMALS, A. G. H., P. W. C. KLOPPENBORG, R. M. LF.quiN, AND
TH. J. BENRAAD, The effect of gonadotrophin releasing hormone on pituitary-gonadal function in Klinefelter's syndrome, Ada Rnriocrinol (Kbh) 83: 829, 1976. 12. STROTT, C. A., T. YOSHIMI, AND M. B. LIPSETT, Plasma
References 1. LIPSF.TT, M. B., T. E. DAVIS, H. WILSON, AND C. J. CANFIELD,
Testosterone production in chromatine-positive Klinefelter's syndrome, J Clin Rnriocrinol Metab 25: 1027, 1965. 2. PAULSEN, C. A., D. L. GORDON, R. W. CARPENTER, H. M.
CANDY, AND W. I). DRUCKER, Klinefelter's syndrome and its
variants: a hormonal and chromosomal study, Recent Prog Hnrm Res 24: 321, 1968. 3. WKINSTKIN, R. L., S. L. KAPLAN, AND M. M. GRUMBACH,
Gonadal and pituitary insensitivity to HCG and clomiphene stimulation in Klinefelter's syndrome. Program of the 5.3rd Meeting of The Endocrine Society, Ran Francisco, 1971, p. A 102 (Abstract. 119). 4. STEWART-BENTLEY, M., AND R. HORTON, Leydig cell function in Klinefelter's syndrome, Metabolism 22: 875, 1974.
progesterone and 17-hydroxyprogesterone in normal men and children with congenital hyperplasia, J Clin Invest 48: 930, 1969. 13. VERMEULEN, A., AND L. VERDONCK, Radioimmunoassay of
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