Skip to main content
Postgraduate Medical Journal logoLink to Postgraduate Medical Journal
. 1992 Mar;68(797):225–228. doi: 10.1136/pgmj.68.797.225

Testotoxicosis: gonadotrophin-independent male sexual precocity.

A A Aziz 1, S M Jafri 1, N U Haque 1
PMCID: PMC2399261  PMID: 1589386

Abstract

In this era of rapidly developing investigational tools and pharmacology, the pathophysiology of precocious puberty is becoming well defined. What was previously thought to be a form of gonadotrophin releasing hormone (GNRH)-dependent central precocious puberty is now classified as GNRH-independent familial testotoxicosis. We present two such cases and review the clinical features, pathophysiology and treatment of testotoxicosis.

Full text

PDF
225

Images in this article

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Aoki A., Fawcett D. W. Is there a local feedback from the seminiferous tubules affecting activity of the Leydig cells? Biol Reprod. 1978 Aug;19(1):144–158. doi: 10.1095/biolreprod19.1.144. [DOI] [PubMed] [Google Scholar]
  2. Braunstein G. D., Kamdar V., Rasor J., Swaminathan N., Wade M. E. Widespread distribution of a chorionic gonadotropin-like substance in normal human tissues. J Clin Endocrinol Metab. 1979 Dec;49(6):917–925. doi: 10.1210/jcem-49-6-917. [DOI] [PubMed] [Google Scholar]
  3. Caruso-Nicoletti M., Cassorla F., Skerda M., Ross J. L., Loriaux D. L., Cutler G. B., Jr Short term, low dose estradiol accelerates ulnar growth in boys. J Clin Endocrinol Metab. 1985 Nov;61(5):896–898. doi: 10.1210/jcem-61-5-896. [DOI] [PubMed] [Google Scholar]
  4. Cassorla F. G., Skerda M. C., Valk I. M., Hung W., Cutler G. B., Jr, Loriaux D. L. The effects of sex steroids on ulnar growth during adolescence. J Clin Endocrinol Metab. 1984 Apr;58(4):717–720. doi: 10.1210/jcem-58-4-717. [DOI] [PubMed] [Google Scholar]
  5. Corvol P., Michaud A., Menard J., Freifeld M., Mahoudeau J. Antiandrogenic effect of spirolactones: mechanism of action. Endocrinology. 1975 Jul;97(1):52–58. doi: 10.1210/endo-97-1-52. [DOI] [PubMed] [Google Scholar]
  6. Covey D. F., Hood W. F. A new hypothesis based on suicide substrate inhibitor studies for the mechanism of action of aromatase. Cancer Res. 1982 Aug;42(8 Suppl):3327s–3333s. [PubMed] [Google Scholar]
  7. Cutler G. B., Jr, Cassorla F. G., Ross J. L., Pescovitz O. H., Barnes K. M., Comite F., Feuillan P. P., Laue L., Foster C. M., Kenigsberg D. Pubertal growth: physiology and pathophysiology. Recent Prog Horm Res. 1986;42:443–470. doi: 10.1016/b978-0-12-571142-5.50014-8. [DOI] [PubMed] [Google Scholar]
  8. Dunaif A., Longcope C., Canick J., Badger T., Crowley W. F., Jr The effects of the aromatase inhibitor delta 1-testolactone on gonadotropin release and steroid metabolism in polycystic ovarian disease. J Clin Endocrinol Metab. 1985 Apr;60(4):773–780. doi: 10.1210/jcem-60-4-773. [DOI] [PubMed] [Google Scholar]
  9. Egli C. A., Rosenthal S. M., Grumbach M. M., Montalvo J. M., Gondos B. Pituitary gonadotropin-independent male-limited autosomal dominant sexual precocity in nine generations: familial testotoxicosis. J Pediatr. 1985 Jan;106(1):33–40. doi: 10.1016/s0022-3476(85)80460-1. [DOI] [PubMed] [Google Scholar]
  10. Gondos B., Egli C. A., Rosenthal S. M., Grumbach M. M. Testicular changes in gonadotropin-independent familial male sexual precocity. Familial testotoxicosis. Arch Pathol Lab Med. 1985 Nov;109(11):990–995. [PubMed] [Google Scholar]
  11. Holland F. J., Fishman L., Bailey J. D., Fazekas A. T. Ketoconazole in the management of precocious puberty not responsive to LHRH-analogue therapy. N Engl J Med. 1985 Apr 18;312(16):1023–1028. doi: 10.1056/NEJM198504183121604. [DOI] [PubMed] [Google Scholar]
  12. Kaplan S. L., Grumbach M. M. Clinical review 14: Pathophysiology and treatment of sexual precocity. J Clin Endocrinol Metab. 1990 Oct;71(4):785–789. doi: 10.1210/jcem-71-4-785. [DOI] [PubMed] [Google Scholar]
  13. MORTIMER E. A., Jr Familial constitutional precocious puberty in a boy three years of age: report of a case. Pediatrics. 1954 Feb;13(2):174–177. [PubMed] [Google Scholar]
  14. McGregor W. G., Raymoure W. J., Kuhn R. W., Jaffe R. B. Fetal tissue can synthesize a placental hormone. Evidence for chorionic gonadotropin beta-subunit synthesis by human fetal kidney. J Clin Invest. 1981 Jul;68(1):306–309. doi: 10.1172/JCI110249. [DOI] [PMC free article] [PubMed] [Google Scholar]
  15. Menard R. H., Stripp B., Gillette J. R. Spironolactone and testicular cytochrome P-450: decreased testosterone formation in several species and changes in hepatic drug metabolism. Endocrinology. 1974 Jun;94(6):1628–1636. doi: 10.1210/endo-94-6-1628. [DOI] [PubMed] [Google Scholar]
  16. Reiter E. O., Grumbach M. M. Neuroendocrine control mechanisms and the onset of puberty. Annu Rev Physiol. 1982;44:595–613. doi: 10.1146/annurev.ph.44.030182.003115. [DOI] [PubMed] [Google Scholar]
  17. Risbridger G. P., Kerr J. B., de Kretser D. M. Evaluation of Leydig cell function and gonadotropin binding in unilateral and bilateral cryptorchidism; evidence for local control of Leydig cell function by the seminiferous tubule. Biol Reprod. 1981 Apr;24(3):534–540. doi: 10.1095/biolreprod24.3.534. [DOI] [PubMed] [Google Scholar]
  18. Rosenthal S. M., Grumbach M. M., Kaplan S. L. Gonadotropin-independent familial sexual precocity with premature Leydig and germinal cell maturation (familial testotoxicosis): effects of a potent luteinizing hormone-releasing factor agonist and medroxyprogesterone acetate therapy in four cases. J Clin Endocrinol Metab. 1983 Sep;57(3):571–579. doi: 10.1210/jcem-57-3-571. [DOI] [PubMed] [Google Scholar]
  19. Santen R. J., Van den Bossche H., Symoens J., Brugmans J., DeCoster R. Site of action of low dose ketoconazole on androgen biosynthesis in men. J Clin Endocrinol Metab. 1983 Oct;57(4):732–736. doi: 10.1210/jcem-57-4-732. [DOI] [PubMed] [Google Scholar]
  20. Schedewie H. K., Reiter E. O., Beitins I. Z., Seyed S., Wooten V. D., Jimenez J. F., Aiman E. J., DeVane G. W., Redman J. F., Elders M. J. Testicular leydig cell hyperplasia as a cause of familial sexual precocity. J Clin Endocrinol Metab. 1981 Feb;52(2):271–278. doi: 10.1210/jcem-52-2-271. [DOI] [PubMed] [Google Scholar]
  21. Schürmeyer T., Nieschlag E. Effect of ketoconazole and other imidazole fungicides on testosterone biosynthesis. Acta Endocrinol (Copenh) 1984 Feb;105(2):275–280. doi: 10.1530/acta.0.1050275. [DOI] [PubMed] [Google Scholar]
  22. Sharpe R. M., Fraser H. M., Cooper I., Rommerts F. F. Sertoli-Leydig cell communication via an LHRH-like factor. Nature. 1981 Apr 30;290(5809):785–787. doi: 10.1038/290785a0. [DOI] [PubMed] [Google Scholar]
  23. Stripp B., Taylor A. A., Bartter F. C., Gillette J. R., Loriaux D. L., Easley R., Menard R. H. Effect of spironolactone on sex hormones in man. J Clin Endocrinol Metab. 1975 Oct;41(4):777–781. doi: 10.1210/jcem-41-4-777. [DOI] [PubMed] [Google Scholar]

Articles from Postgraduate Medical Journal are provided here courtesy of BMJ Publishing Group

RESOURCES