Abstract
Thirty-five patients with thalassemia major, aged 7 to 21 years, were studied to define the relationship between the pubertal development and the growth-hormone (GH) secretion during sleep, after administration of GH-releasing factor (hpGRF 1-44), and betaxolol-glucagon or arginin- insulin. Pubertal development was classified as being appropriate or delayed for chronological age. GH response to pharmacological stimuli and during sleep was not linked to the pubertal development according to the chronological age. The peak of GH secretion after GHRH injection was significantly delayed in thalassemic patients with retarded puberty. The integrated secretion of GH during the 120-min test was slightly but not significantly reduced in these patients. The prepubertal pattern of GHRH response was restored in the patients receiving substitutive therapy by HCG or testosterone. The alteration of GH response to GHRH in thalassemic patients is likely to be only due to delayed puberty and decreased endogeneous GHRH secretion since it is corrected by androgen or gonadotropin replacement.
Similar content being viewed by others
References
Borgna-Pignatti C., Stefano P., Zonta L., Vullo C., De Sanctis V., Melevendi C., Naselli A., Masera G., Terzoli S., Gabutti V., Piga A. Growth and sexual maturation in thalassemia major. J.Pediatr. 106: 150, 1985.
Copeland K.C., Paunier L., Sizonenko P.C. The secretion of adrenal androgens and growth patterns of patients with hypogonadotropic hypogonadism and idiopathic delayed puberty. J. Pediatr. 91: 985, 1977.
Vannasaeng S., Ploybur S., Visuktul P., Tandhanand S., Suwanik R., Wasi P. Endocrine function in thalassemia Clin. Endocrinol. (Oxf.) 14: 165, 1981.
Canale V.C., Steinherz P., New M.I., Erlandson M. Endocrine function in thalassemia major. Ann. N.Y. Acad. Sci. USA 232: 233, 1974.
Masala A., Meloni T., Gallisai D., Alagna S., Rovasio P.P., Rassu S., Milla A.F. Endocrine functioning in multitransfused prepubertal patients with homozygous B-thalassemia. J. Clin. Endocrinol. Metab. 58: 667, 1984.
Saenger P., Schwartz E., Markenson A.L., Graziano J.H., Levine L.S., New M.I., Hilgartner M.W. Depressed serum somatomedin activity in B-thalassemia. J. Pediatr. 96: 214, 1980.
Werther G.A., Matthews R.N., Burger H.G., Herington A.C. Lack of response of non suppressible insulin-like activity to short term administration of human growth in thalassemia major. J. Clin. Endocrinol. Metab. 53: 806, 1981.
Pintor C., Cella S.G., Manso P., Corda R., Dessi C., Locatelli V., Muller E.E. Impaired growth hormone (GH) response to GH-releasing hormone in thalassemia major. J. Clin. Endocrinol. Metab. 62: 263, 1986.
Loche S. Growth and sexual maturation in thalassemia. J. Pediatr. 107: 642, 1985.
Leger J., Girot R., Crosnier H., Postel-Vinay M.C., Rappaport R. Normal growth hormone response to GH-Releasing Hormone in children with thalassemia major before puberty: A possible age-related effect. J. Clin. Endocrinol. Metab. 69: 453, 1989.
Sempe M., Pedron G., Roy-Pernot M.P. Auxologie. Méthodes et Séquences. Theraplix, Paris, 1979.
Argente J., Evain Brion D., Donnadieu M., Garnier P., Vaudry H., Job J.C. Impaired response of growth hormone-releasing hormone (GHRH) measured in plasma after L-Dopa stimulation in patients with idiopathic delayed puberty. Acta Pediatr. Scand. 76: 266, 1987.
Bozzola M., Argente J., Cisternino M., Moretta A., Valtorta A., Biscaldi I., Donnadieu M., Evain-Brion D., Severi F. Effect of human chorionic gonadotrophin on growth velocity and biological growth parameters in adolescent with thalassemia major. Eur. J. Pediatr. 148: 300, 1989.
Liu L., Merriam G.R., Sherins R.J. Chronic sexual steroid exposure increases mean plasma growth hormone concentration and pulse amplitude in men with isolated hypogonadotropic hypogonadism. J. Clin. Endocrinol. Metab. 64: 651, 1987.
Bierich J.R. Growth hormone therapy in short children without classical growth hormone deficiency. J. Endocrinol. Invest. 12: 25, 1989.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Leheup, B.P., Cisternino, M., Bozzola, M. et al. Growth hormone response following growth hormone releasing hormone injection in thalassemia major: influence of pubertal development. J Endocrinol Invest 14, 37–40 (1991). https://doi.org/10.1007/BF03350257
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/BF03350257