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. 1974 Feb;53(2):408–415. doi: 10.1172/JCI107574

Pituitary-testicular responsiveness in male hypogonadotropic hypogonadism.

R L Weinstein 1, R E Reitz 1
PMCID: PMC301483  PMID: 11344554

Abstract

An isolated deficiency of pituitary gonadotropins was demonstrated in six 46 XY males, 22 to 36 years of age, with and without anosmia. Undetectable or low levels of serum follicle-stimulating hormone (FSH) and luteinizing hormone (LH) clearly separated hypogonadotropic from normal adult males. Chronic (8-12 wk) administration of clomiphene citrate caused no increase in serum FSH or LH in gonadotropin-deficient subjects. However, the administration of synthetic luteinizing hormone releasing factor (LRF) resulted in the appearance of serum LH and, to a lesser degree, serum FSH in three subjects tested. While levels of plasma testosterone were significantly lower in gonadotropin-deficient subjects, plasma androstenedione and dehydroepiandrosterone were in a range similar to that of age-matched normal men. Treatment with human chorionic gonadotropin (HCG) increased levels of plasma testosterone to normal adult male values in all gonadotropin-deficient subjects. Cessation of treatment with HCG resulted in the return of plasma testosterone to low, pretreatment levels. That HCG therapy with resultant normal levels of plasma testosterone may somehow stimulate endogenous gonadotropin secretion in gonadotropin-deficient subjects was not evident. The adult male levels of serum FSH and LH after LRF, and plasma testosterone after HCG, confirm pituitary and Leydig cell responsiveness in these subjects.

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Selected References

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  1. ALBERT A., UNDERDAHL L. O., GREENE L. F., LORENZ N. Male hypogonadism. II. Classification. Proc Staff Meet Mayo Clin. 1953 Oct 7;28(20):557–567. [PubMed] [Google Scholar]
  2. ALBERT A., UNDERDAHL L. O., GREENE L. F., LORENZ N. Male hypogonadism. IV. The testis in prepubertal or pubertal gonadotropic failure. Proc Staff Meet Mayo Clin. 1954 Feb 24;29(4):131–136. [PubMed] [Google Scholar]
  3. Amoss M., Burgus R., Blackwell R., Vale W., Fellows R., Guillemin R. Purification, amino acid composition and N-terminus of the hypothalamic luteinizing hormone releasing factor (LRF) of ovine origin. Biochem Biophys Res Commun. 1971 Jul 2;44(1):205–210. doi: 10.1016/s0006-291x(71)80179-1. [DOI] [PubMed] [Google Scholar]
  4. BIBEN R. L., GORDAN G. S. Familial hypogonadotropic eunuchoidism. J Clin Endocrinol Metab. 1955 Aug;15(8):931–942. doi: 10.1210/jcem-15-8-931. [DOI] [PubMed] [Google Scholar]
  5. Bardin C. W., Ross G. T., Rifkind A. B., Cargille C. M., Lipsett M. B. Studies of the pituitary-Leydig cell axis in young men with hypogonadotropic hypogonadism and hyposmia: comparison with normal men, prepuberal boys, and hypopituitary patients. J Clin Invest. 1969 Nov;48(11):2046–2056. doi: 10.1172/JCI106170. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Boyar R. M. The effect of clomiphene citrate in anosmic hypogonadotrophism. Ann Intern Med. 1969 Dec;71(6):1127–1131. doi: 10.7326/0003-4819-71-6-1127. [DOI] [PubMed] [Google Scholar]
  7. Ewer R. W. Familial monotropic pituitary gonadotropin insufficiency. J Clin Endocrinol Metab. 1968 Jun;28(6):783–788. doi: 10.1210/jcem-28-6-783. [DOI] [PubMed] [Google Scholar]
  8. Faiman C., Ryan R. J. Radioimmunoassay for human follicle stimulating hormone. J Clin Endocrinol Metab. 1967 Mar;27(3):444–447. doi: 10.1210/jcem-27-3-444. [DOI] [PubMed] [Google Scholar]
  9. Gandy H. M., Peterson R. E. Measurement of testosterone and 17-ketosteroids in plasma by the double isotope dilution derivative technique. J Clin Endocrinol Metab. 1968 Jul;28(7):949–977. doi: 10.1210/jcem-28-7-949. [DOI] [PubMed] [Google Scholar]
  10. HUNTER W. M., GREENWOOD F. C. Preparation of iodine-131 labelled human growth hormone of high specific activity. Nature. 1962 May 5;194:495–496. doi: 10.1038/194495a0. [DOI] [PubMed] [Google Scholar]
  11. Hamilton C. R., Henkin R. I., Weir G., Kliman B. Olfactory status and response to clomiphene in male gonadotrophin deficiency. Ann Intern Med. 1973 Jan;78(1):47–55. doi: 10.7326/0003-4819-78-1-47. [DOI] [PubMed] [Google Scholar]
  12. LE MARQUAND H. S. Congenital hypogonadotrophic hypogonadism in five members of a family, three brothers and two sisters. Proc R Soc Med. 1954 Jun;47(6):442–446. [PubMed] [Google Scholar]
  13. LYNCH H. T., OZER F., MCNUTT C. W., JOHNSON J. E., JAMPOLSKY N. A. Secondary male hypogonadism and congenital ichthyosis: association of two rare genetic diseases. Am J Hum Genet. 1960 Dec;12:440–447. [PMC free article] [PubMed] [Google Scholar]
  14. Lynch H. T., Kaplan A. R., Henn M. J., Krush A. J. Familial coexistence of diabetes mellitus, hyperlipemia, short stature, and hypogonadism. Am J Med Sci. 1966 Sep;252(3):323–330. doi: 10.1097/00000441-196609000-00012. [DOI] [PubMed] [Google Scholar]
  15. MATTHEWS W. B., RUNDLE A. T. FAMILIAL CEREBELLAR ATAXIA AND HYPOGONADISM. Brain. 1964 Sep;87:463–468. doi: 10.1093/brain/87.3.463. [DOI] [PubMed] [Google Scholar]
  16. Monahan M., Rivier J., Burgus R., Amoss M., Blackwell R., Vale W., Guillemin R. Synthèse totale par phase solide d'un décapeptide qui stimule la sécrétion des gonadotropines hypophysaires LH et FS. C R Acad Sci Hebd Seances Acad Sci D. 1971 Jul 26;273(4):508–510. [PubMed] [Google Scholar]
  17. NOWAKOWSKI H., LENZ W. Genetic aspects in male hypogonadism. Recent Prog Horm Res. 1961;17:53–95. [PubMed] [Google Scholar]
  18. Nankin H. R., Field J. B., Troen P. Clomiphene therapy in hypogonadotrophism with defective olfaction. Ann Intern Med. 1970 Oct;73(4):660–661. doi: 10.7326/0003-4819-73-4-660. [DOI] [PubMed] [Google Scholar]
  19. Poznanski W., Bennett J., McKendry J. B. Urinary steroid pattern in male hypogonadotrophic hypogonadism. Can Med Assoc J. 1967 Feb 4;96(5):273–277. [PMC free article] [PubMed] [Google Scholar]
  20. ROSEWATER S., GWINUP G., HAMWI G. J. FAMILIAL GYNECOMASTIA. Ann Intern Med. 1965 Sep;63:377–385. doi: 10.7326/0003-4819-63-3-377. [DOI] [PubMed] [Google Scholar]
  21. Rifkind A. B., Kulin H. E., Ross G. T. Follicle-stimulating hormone (FSH) and luteninizing hormone (LH) in the urine of prepubertal children. J Clin Invest. 1967 Dec;46(12):1925–1931. doi: 10.1172/JCI105682. [DOI] [PMC free article] [PubMed] [Google Scholar]
  22. Rivarola M. A., Bergada C., Cullen M. HCG stimulation test in prepubertal boys with cryptorchidism, in bilateral anorchia and in male pseudohermaphroditism. J Clin Endocrinol Metab. 1970 Nov;31(5):526–530. doi: 10.1210/jcem-31-5-526. [DOI] [PubMed] [Google Scholar]
  23. Rodbard D., Rayford P. L., Cooper J. A., Ross G. T. Statistical quality control of radioimmunoassays. J Clin Endocrinol Metab. 1968 Oct;28(10):1412–1418. doi: 10.1210/jcem-28-10-1412. [DOI] [PubMed] [Google Scholar]
  24. Roth J. C., Kelch R. P., Kaplan S. L., Grumbach M. M. FSH and LH response to luteinizing hormone-releasing factor in prepubertal and pubertal children, adult males and patients with hypogonadotropic and hypertropic hypogonadism. J Clin Endocrinol Metab. 1972 Dec;35(6):926–930. doi: 10.1210/jcem-35-6-926. [DOI] [PubMed] [Google Scholar]
  25. SCHALCH D. S., PARKER M. L. A SENSITIVE DOUBLE ANTIBODY IMMUNOASSAY FOR HUMAN GROWTH HORMONE IN PLASMA. Nature. 1964 Sep 12;203:1141–1142. doi: 10.1038/2031141a0. [DOI] [PubMed] [Google Scholar]
  26. Saez J. M., Bertrand J. Studies on testicular function in children: plasma concentrations of testosterone, dehydroepiandrosterone and its sulfate before and after stimulation with human chorionic gonadotrophin. (1). Steroids. 1968 Dec;12(6):749–761. doi: 10.1016/s0039-128x(68)80029-7. [DOI] [PubMed] [Google Scholar]
  27. Santen R. J., Leonard J. M., Sherins R. J., Gandy H. M., Paulsen C. A. Short- and long-term effects of clomiphene citrate on the pituitary-testicular axis. J Clin Endocrinol Metab. 1971 Dec;33(6):970–979. doi: 10.1210/jcem-33-6-970. [DOI] [PubMed] [Google Scholar]
  28. Saxena B. B., Demura H., Gandy H. M., Peterson R. E. Radioimmunoassay of human follicle stimulating and luteinizing hormones in plasma. J Clin Endocrinol Metab. 1968 Apr;28(4):519–534. doi: 10.1210/jcem-28-4-519. [DOI] [PubMed] [Google Scholar]
  29. Schalch D. S., Parlow A. F., Boon R. C., Reichlin S. Measurement of human luteinizing hormone in plasma by radioimmunoassay. J Clin Invest. 1968 Mar;47(3):665–678. doi: 10.1172/JCI105762. [DOI] [PMC free article] [PubMed] [Google Scholar]
  30. Schroffner W. G., Furth E. D. Hypogonadotropic hypogonadism with anosmia (Kallmann's syndrome) unresponsive to clomiphene citrate. J Clin Endocrinol Metab. 1970 Sep;31(3):267–270. doi: 10.1210/jcem-31-3-267. [DOI] [PubMed] [Google Scholar]
  31. Sparkes R. S., Simpson R. W., Paulsen C. A. Familial hypogonadotropic hypogonadism with anosmia. Arch Intern Med. 1968 Jun;121(6):534–538. [PubMed] [Google Scholar]
  32. VESTERGAARD P. Rapid micro-modification of the Zimmermann/Callow procedure for the determination of 17-ketosteroids in urine. Acta Endocrinol (Copenh) 1951;8(3):193–214. doi: 10.1530/acta.0.0080193. [DOI] [PubMed] [Google Scholar]
  33. VOLPE R., METZLER W. S., JOHNSTON M. W. Familial hypogonadotrophic eunuchoidism with cerebellar ataxia. J Clin Endocrinol Metab. 1963 Jan;23:107–115. doi: 10.1210/jcem-23-1-107. [DOI] [PubMed] [Google Scholar]
  34. Winter J. S., Taraska S., Faiman C. The hormonal response to HCG stimulation in male children and adolescents. J Clin Endocrinol Metab. 1972 Feb;34(2):348–353. doi: 10.1210/jcem-34-2-348. [DOI] [PubMed] [Google Scholar]
  35. Zárate A., Kastin A. J., Soria J., Canales E. S., Schally A. V. Effectf of synthetic luteinizing hormone-releasing hormone (LH-RH) in two brothers with hypogonadotropic hypogonadism and anosmia. J Clin Endocrinol Metab. 1973 Mar;36(3):612–614. doi: 10.1210/jcem-36-3-612. [DOI] [PubMed] [Google Scholar]

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