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Growth and Sexual Development Before and After Sex Steroid Therapy in Patients With Thalassemia Major

Growth and Sexual Development Before and After Sex Steroid Therapy in Patients With Thalassemia... Abstract • Growth, sexual development, and hypothalamic-pituitary-gonadal function were evaluated in 23 patients with thalassemia major (14 female and nine male) aged 13 to 29 years. Five women (group 1) with hemoglobin levels of less than 7 g/dL, which were maintained by transfusions during childhood, did not spontaneously enter puberty. They had evidence of severe hypothalamic-pituitary dysfunction. Maintaining hemoglobin levels of about 8 g/dL resulted in spontaneous onset of puberty in seven of nine female patients (group 2), but had no such ameliorative effect on the nine male patients. In the latter, peak luteinizing hormone (LH) responses to gonadotropin releasing hormone correlated with bone age. Treatment with testosterone produced inconsistent partial inhibition of LH and follicle-stimulating hormone (FSH) responses to stimulation. After discontinuation of testosterone treatment, a rebound of basal testosterone, LH, and FSH levels was observed, but this was not sustained. These findings are compatible either with dysfunction of hypothalamic maturation or with partial pituitary dysfunction. Four of the group 1 females and six of the males treated with appropriate sex hormones showed satisfactory pubertal progression. Acceleration in linear growth was observed in four of the male patients whose epiphyses were still open. Treatment was well tolerated in all patients. (Arch Intern Med 1984;144:2341-2346) References 1. Kletzky OA, Costin G, Marrs RP, et al: Gonadotropin insufficiency in patients with thalassemia major. J Clin Endocrinol Metabol 1979;48:901-905.Crossref 2. Costin G, Kogut MD, Hyman CB, et al: Endocrine abnormalities in thalassemia major. AJDC 1979;133:497-502. 3. Vannasaeng S, Ploybutr S, Visutkul P, et al: Endocrine function in thalassemia. Clin Endocrinol 1981;14:165-173.Crossref 4. Cividalli G, Kerem H, Rachmilewitz EA: Globin synthesis in severe and intermediate homozygous thalassemia in Israel: Fourth conference on Cooley's anemia. Ann NY Acad Sci 1980;344:132-140.Crossref 5. Frisch RE, McArthur JW: Menstrual cycles: Fatness as a determinant of minimum weight for height necessary for their maintenance or onset. Science 1974;185:949-951.Crossref 6. Tanner JM: Growth and endocrinology of the adolescents , in Gardner LI (ed): Endocrine and Genetic Diseases of Childhood . Philadelphia, WB Saunders Co, 1975, pp 14-35. 7. Prader A: Testicular size: Assessment and clinical importance. Triangle 1966;7:240-243. 8. Greulich WW, Pyle SI: Radiographic Atlas of the Skeletal Development of the Hand and Wrist , ed 2. Stanford, Calif, Stanford University Press, 1959. 9. Spitz IM, Le Roith D, Livshin Y, et al: Exaggerated prolactin response to thyrotropin-releasing hormone and metoclopromide in primary testicular failure. Fertil Steril 1980;34:573-580. 10. Weiner M, Karpatkin M, Hart D, et al: Cooley anemia: High transfusion regimen and chelation therapy, results and perspective. J Pediatr 1978;92:653-658.Crossref 11. Normand M, Fortier C: Numerical versus analytical integration or hormonal disappearance data. Can J Physiol Pharmacol 1970;48:247-281.Crossref 12. Modell B: Total management of thalassemia major. Arch Dis Child 1977;52:489-500.Crossref 13. Spitz IM, Landau H, Gross V, et al: Prolactin responsiveness to TRH and metoclopromide in thalassaemia. Clin Endocrinol 1982;16:275-282.Crossref 14. Landau H, Spitz IM, Cividalli G, et al: Gonadotropin thyrotrophin and prolactin reserve in thalassaemia. Clin Endocrinol 1978;9:163-173.Crossref 15. Kelch RP, Markovs M, Huss J: LH and FSH responsiveness to intravenous gonadotropin-releasing hormone (GnRH) in children with hypothalamic or pituitary disorder: Lack of effect of replacement therapy with human growth hormone. J Clin Endocrinol Metabol 1976;42:1104-1113.Crossref 16. Hopwood NJ, Kelch RP, Zipf WB, et al: The effect of synthetic androgens on the hypothalamic-pituitary-gonadal axis in boys with constitutionally delayed growth. J Pediatr 1979;94:657-662.Crossref 17. Caminos-Torres R, Ma L, Snyder PJ: Testosterone-induced inhibition of the LH and FSH responses to gonadotropin-releasing hormone occurs slowly. J Clin Endocrinol Metabol 1977;44:1142-1153.Crossref 18. Mauss J, Börsch G, Bormacher K, et al: Effect of long-term testosterone oenanthate administration on male reproductive function: Clinical evaluation, serum FSH, LH, testosterone and seminal fluid analyses in normal men. Acta Endocrinol 1975;78:373-384. 19. Rosenfield RL, Rich BH, Lucky AW: Adrenarche as a cause of benign pseudopuberty in boys. J Pediatr 1982;101:1005-1009.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Internal Medicine American Medical Association

Growth and Sexual Development Before and After Sex Steroid Therapy in Patients With Thalassemia Major

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Publisher
American Medical Association
Copyright
Copyright © 1984 American Medical Association. All Rights Reserved.
ISSN
0003-9926
eISSN
1538-3679
DOI
10.1001/archinte.1984.00350220057012
Publisher site
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Abstract

Abstract • Growth, sexual development, and hypothalamic-pituitary-gonadal function were evaluated in 23 patients with thalassemia major (14 female and nine male) aged 13 to 29 years. Five women (group 1) with hemoglobin levels of less than 7 g/dL, which were maintained by transfusions during childhood, did not spontaneously enter puberty. They had evidence of severe hypothalamic-pituitary dysfunction. Maintaining hemoglobin levels of about 8 g/dL resulted in spontaneous onset of puberty in seven of nine female patients (group 2), but had no such ameliorative effect on the nine male patients. In the latter, peak luteinizing hormone (LH) responses to gonadotropin releasing hormone correlated with bone age. Treatment with testosterone produced inconsistent partial inhibition of LH and follicle-stimulating hormone (FSH) responses to stimulation. After discontinuation of testosterone treatment, a rebound of basal testosterone, LH, and FSH levels was observed, but this was not sustained. These findings are compatible either with dysfunction of hypothalamic maturation or with partial pituitary dysfunction. Four of the group 1 females and six of the males treated with appropriate sex hormones showed satisfactory pubertal progression. Acceleration in linear growth was observed in four of the male patients whose epiphyses were still open. Treatment was well tolerated in all patients. (Arch Intern Med 1984;144:2341-2346) References 1. Kletzky OA, Costin G, Marrs RP, et al: Gonadotropin insufficiency in patients with thalassemia major. J Clin Endocrinol Metabol 1979;48:901-905.Crossref 2. Costin G, Kogut MD, Hyman CB, et al: Endocrine abnormalities in thalassemia major. AJDC 1979;133:497-502. 3. Vannasaeng S, Ploybutr S, Visutkul P, et al: Endocrine function in thalassemia. Clin Endocrinol 1981;14:165-173.Crossref 4. Cividalli G, Kerem H, Rachmilewitz EA: Globin synthesis in severe and intermediate homozygous thalassemia in Israel: Fourth conference on Cooley's anemia. Ann NY Acad Sci 1980;344:132-140.Crossref 5. Frisch RE, McArthur JW: Menstrual cycles: Fatness as a determinant of minimum weight for height necessary for their maintenance or onset. Science 1974;185:949-951.Crossref 6. Tanner JM: Growth and endocrinology of the adolescents , in Gardner LI (ed): Endocrine and Genetic Diseases of Childhood . Philadelphia, WB Saunders Co, 1975, pp 14-35. 7. Prader A: Testicular size: Assessment and clinical importance. Triangle 1966;7:240-243. 8. Greulich WW, Pyle SI: Radiographic Atlas of the Skeletal Development of the Hand and Wrist , ed 2. Stanford, Calif, Stanford University Press, 1959. 9. Spitz IM, Le Roith D, Livshin Y, et al: Exaggerated prolactin response to thyrotropin-releasing hormone and metoclopromide in primary testicular failure. Fertil Steril 1980;34:573-580. 10. Weiner M, Karpatkin M, Hart D, et al: Cooley anemia: High transfusion regimen and chelation therapy, results and perspective. J Pediatr 1978;92:653-658.Crossref 11. Normand M, Fortier C: Numerical versus analytical integration or hormonal disappearance data. Can J Physiol Pharmacol 1970;48:247-281.Crossref 12. Modell B: Total management of thalassemia major. Arch Dis Child 1977;52:489-500.Crossref 13. Spitz IM, Landau H, Gross V, et al: Prolactin responsiveness to TRH and metoclopromide in thalassaemia. Clin Endocrinol 1982;16:275-282.Crossref 14. Landau H, Spitz IM, Cividalli G, et al: Gonadotropin thyrotrophin and prolactin reserve in thalassaemia. Clin Endocrinol 1978;9:163-173.Crossref 15. Kelch RP, Markovs M, Huss J: LH and FSH responsiveness to intravenous gonadotropin-releasing hormone (GnRH) in children with hypothalamic or pituitary disorder: Lack of effect of replacement therapy with human growth hormone. J Clin Endocrinol Metabol 1976;42:1104-1113.Crossref 16. Hopwood NJ, Kelch RP, Zipf WB, et al: The effect of synthetic androgens on the hypothalamic-pituitary-gonadal axis in boys with constitutionally delayed growth. J Pediatr 1979;94:657-662.Crossref 17. Caminos-Torres R, Ma L, Snyder PJ: Testosterone-induced inhibition of the LH and FSH responses to gonadotropin-releasing hormone occurs slowly. J Clin Endocrinol Metabol 1977;44:1142-1153.Crossref 18. Mauss J, Börsch G, Bormacher K, et al: Effect of long-term testosterone oenanthate administration on male reproductive function: Clinical evaluation, serum FSH, LH, testosterone and seminal fluid analyses in normal men. Acta Endocrinol 1975;78:373-384. 19. Rosenfield RL, Rich BH, Lucky AW: Adrenarche as a cause of benign pseudopuberty in boys. J Pediatr 1982;101:1005-1009.Crossref

Journal

Archives of Internal Medicine – American Medical Association

Published: Dec 1, 1984

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