Pituitary-Gonadal Response to Short-term Pulsatile Luteinizing Hormone-Releasing Hormone Administration in Young Thalassemic Patients

  • M. Giusti
  • S. Valenti
  • P. G. Mori
  • R. Guido
  • C. Micalizzi
  • F. Perfumo
  • G. Giordano


Delayed puberty is one of the most common endocrine problems in severe thalassemia [1]. Because of its clinical and psychological manifestations it remains a serious problem, especially for male patients. Impaired secretion of both luteinizing hormone (LH) and follicle-stimulation hormone after LH-releasing hormone (LHRH) and, to a lesser extent, a decrease in sex-steroid secretion have been ascribed to pituitary and/or testicular damage by chronic iron overload [2]. However, because pubertal changes are linked to an increase in hypothalamic LHRH activity [3], a defect in the central operator of pubertal activation in thalassemia can also be hypothesized. On the other hand, hypothalamic dysfunction has been suggested in the early stage of genetic hemochromatosis [4]. An impairment in LHRH discharge has been recently described in uremic boys, in whom delayed puberty is a common finding [5], and short-term pulsatile LHRH administration seems to restore normal pituitary-gonadal secretion in uremic boys with delayed puberty [5]. We studied the effect of short-term pulsatile LHRH administration in a group of thalassemic patients with delayed or disordered puberty in order to evaluate the degree of hypothalamic involvement.


Luteinizing Hormone Testicular Volume Luteinizing Hormone Secretion Hypothalamic Dysfunction Hypothalamic Involvement 


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Borgna-Pignatti C, De Stefano P, Zonta L, Vullo C, De Sanctis V, Melevendi C, Naselli A, Masera G, Terzoli S, Gabutti V, Piga A (1985) Growth and sexual maturation in thalassemia major. J Pediatr 106: 150–155PubMedCrossRefGoogle Scholar
  2. 2.
    Wang C, Tso SC, Todd D (1989) Hypogonadotropic hypogonadsism in severe β-thalassemia: effect of chelation and pulsatile gonadotropin-releasing hormone therapy. J Clin Endocrinol Metab 68: 511–516PubMedCrossRefGoogle Scholar
  3. 3.
    Ducharme JR (1989) Normal puberty: clinical manifestations and their endocrine control. In: Collu R, Ducharme JR, Guyda HJ (eds) Pediatric endocrinology. Raven, New York, pp 307–330Google Scholar
  4. 4.
    Piperno A, Rivolta MR, D’Alba R, Fargio S, Rovelli F, Ghezzi A, Micheli M, Fiorelli G (1992) Preclinical hypogonadism in genetic hemochromatosis in the early stage of the disease: evidence of hypothalamic dysfunction. J Endocrinol Invest 15: 423–128PubMedGoogle Scholar
  5. 5.
    Giusti M, Perfumo F, Verrina E, Cavallero D, Piaggio G, Valenti S, Gusmano R, Giordano G (1992) Delayed puberty in uremia: pituitary-gonadal function during short-term pulsatile luteinizing hormone-releasing hormone administration. J Endocrinol Invest 15: 709–717PubMedGoogle Scholar
  6. 6.
    Dufau ML, Pock R, Neubauer A, Catt KJ (1976) In vitro bioassay of LH in human serum: the interstitial cell testosterone ( RICT) assay. J Clin Endocrinol Metab 42: 958–969PubMedCrossRefGoogle Scholar
  7. 7.
    Scharer K, Schaefer F, Trott M, Kassmann K, Gilli G, Gerhard I, Klinga K, Schonberg D, Vecsei P (1989) Pubertal development in children with chronic renal failure. In: Scharer K (ed) Pediatric and adolescent endocrinology. Karger, Basel, pp 151–168Google Scholar
  8. 8.
    Masala A, Meloni T, Gallisai D, Alagna S, Rovasio PP, Rassu S, Milia AF (1984) Endocrine functioning in multitransfused prepubertal patients with homozygous β-thalassemia. J Clin Endocrinol Metab 58: 667–670PubMedCrossRefGoogle Scholar
  9. 9.
    Balducci R, Toscano V, Finocchi G, Municchi G, Mangiantini A, Boscherini B (1990) Effect of hCG or hCG + FSH treatments in young thalassemic patients with hypogonadotropic hypogonadism. J Endocrinol Invest 13: 1–7PubMedGoogle Scholar
  10. 10.
    Holdsworth S, Atkins RC, De Kretser DM (1977) The pituitary testicular axis in men with chronic renal failure. N Engl J Med 296: 1245–1249PubMedCrossRefGoogle Scholar
  11. 11.
    Tsatsoulis A, Shalet SM, Robertson WR (1991) Bioactive gonadotrophin secretion in man. Clin Endocrinol (Oxf) 35: 193–206CrossRefGoogle Scholar
  12. 12.
    Veldhuis JD, Wilkowski MJ, Zwart AD, Urban RJ, Lizarralde G, Iranmanesh A, Bolton WK (1993) Evidence for attenuation of hypothalamic-releasing hormone ( GnRH) impulse strength with preservation of GnRH pulse frequency in men with chronic renal failure. J Clin Endocrinol Metab 76: 648–654PubMedCrossRefGoogle Scholar
  13. 13.
    Chatterjee R, Katz M, Wonke B, Porter JB (1992) Long-term follow-up of hypothalamic-pituitary axis in patients with secondary amenorrhoea. International Mediterranean Conference on Endocrine Disorders in Thalassemia, May 7–9, Cosenza, p 20Google Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 1995

Authors and Affiliations

  • M. Giusti
  • S. Valenti
  • P. G. Mori
  • R. Guido
  • C. Micalizzi
  • F. Perfumo
  • G. Giordano

There are no affiliations available

Personalised recommendations