Role of Growth Hormone Therapy in Turner Syndrome
Abstract
The classic phenotypic appearance associated with the 45,X karyotype was first described by Otto Ullrich in 1930 and by Henry Turner in 1938 (1, 2). The syndrome is appropriately called Ullrich-Turner in Germany, but simply Turner syndrome (TS) in the United States. (We shall use the latter term by convention.) In the 1940s, the infantilism described by Turner was related to rudimentary development of the ovaries; thus the term gonadal dysgenesis (3). In 1959, not long after the correct number of human chromosomes was elucidated, the syndrome was attributed to loss of an X chromosome (4). Properly, it should be stated that X chromosome monosomy results from the loss of the second sex chromosome, whether X or Y, presumably secondary to a nondisjunctional event in an initial mitotic division. Further cytogenetic studies in the 1960s determined that TS due to structural abnormality of the second X chromosome or to various mosaicisms may be clinically indistinguishable from X chromosome monosomy.
Keywords
Standard Deviation Score Turner Syndrome Final Height Adult Height Growth Hormone TherapyPreview
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References
- 1.Ullrich P. Turner’s syndrome and status Bonnevie-Ullrich. Am J Hum Genet 1949; 1: 179–202.PubMedGoogle Scholar
- 2.Turner HH. A syndrome of infantilism, congenital webbed neck, and cubitus valgus. Endocrinology 1938; 23: 566–74.CrossRefGoogle Scholar
- 3.Grumbach MM, Van Wyk JJ, Wilkins L. Chromosomal sex in gonadal dysgenesis: relationship to male pseudohermaphroditism and theories of human sex differentiation. J Clin Endocrinol Metab 1955; 15: 1161–93.PubMedCrossRefGoogle Scholar
- 4.Ford CE, Miller OJ, Polani PE, de Almeida JC, Briggs JH. A sex chromosome anomaly in a case of gonadal dysgenesis (Turner’s syndrome). Lancet 1959; 1: 711–3.PubMedCrossRefGoogle Scholar
- 5.Ranke MB, Pfluger H, Rosendahl W, et al. Turner syndrome: spontaneous growth in 150 cases and review of the literature. Eur J Pediatr 1983; 141: 81–8.PubMedCrossRefGoogle Scholar
- 6.Naeraa RW, Nielsen J. Standards for growth and final height in Turner’s syndrome. Acta Paediatr Scand 1990; 79: 182–90.PubMedCrossRefGoogle Scholar
- 7.Lyon AJ, Preece MA, Grant DB. Growth curve for girls with Turner syndrome. Arch Dis Child 1985; 60: 932–5.PubMedCrossRefGoogle Scholar
- 8.Frane JW, Sherman BM, Genentech Collaborative Group. Predicted adult height in Turner syndrome. In: Rosenfeld RG, Grumbach MM, eds. Turner syndrome. New York: Marcel Dekker, 1990: 405–19.Google Scholar
- 9.Holland J, Brnjac L, Alexander D, et al. Turner syndrome and final adult stature: a randomized controlled trial using human growth hormone and low dose ethinyl estradiol. In: Ranke MB, Rosenfeld RG, eds. Turner syndrome: growth promoting therapies. Amsterdam: Excerpta Mediea, 1991: 195–200.Google Scholar
- 10.Sybert VP. Adult height in Turner syndrome with and without androgen therapy. J Pediatr 1984; 104: 365–9.PubMedCrossRefGoogle Scholar
- 11.Rosenfeld RG, Grumbach MM, eds. Turner syndrome. New York: Marcel Dekker, 1990.Google Scholar
- 12.Ross JL, Long LM, Loriaux DL, Cutler GB Jr. Growth hormone secretory dynamics in Turner syndrome. J Pediatr 1985; 106: 202–6.PubMedCrossRefGoogle Scholar
- 13.Massarano AA, Brook CGD, Hindmarsh PC, et al. Growth hormone secretion in Turner’s syndrome and influence of oxandrolone and ethinyl estradiol. Arch Dis Child 1989; 64: 587–92.PubMedCrossRefGoogle Scholar
- 14.Cuttler L, Van Vliet G, Conte FA, Kaplan SL, Grumbach MM. Somatomedin-C levels in children and adolescents with gonadal dysgenesis: differences from age-matched normal females and effect of chronic estrogen replacement. J Clin Endocrinol Metab 1985; 60: 1087–92.PubMedCrossRefGoogle Scholar
- 15.Mauras N, Rogol AD, Veldhuis JD. Increased hGH production rate after low-dose estrogen therapy in prepubertal girls with Turner’s syndrome. Pediatr Res 1990; 28: 626–30.PubMedCrossRefGoogle Scholar
- 16.Naeraa RW, Nielsen J, Pedersen IL, Sorensen K. Effect of oxandrolone on growth and final height in Turner’s syndrome. Acta Paediatr Scand 1990; 79: 784–9.PubMedCrossRefGoogle Scholar
- 17.Crock P, Werther GA, Wettenhall HNB. Oxandrolone increases final height in Turner syndrome. J Paediatr Child Health 1990; 26: 221–4.PubMedCrossRefGoogle Scholar
- 18.Vanderschueren-Lodeweyckx, et al. Growth-promoting effect of growth hormone and low-dose ethinyl estradiol in girls with Turner’s syndrome. J Clin Endocrinol Metab 1990; 70: 122–6.PubMedCrossRefGoogle Scholar
- 19.Escamilla RF, Hutchings JJ, Deamer WC, Li CH. Clinical experiences with human growth hormone (LI) in pituitary infantilism and in gonadal dysgenesis. Acta Endocrinol Suppl (Copenh) 1960; 51: 253A.Google Scholar
- 20.Rosenfeld RG, Frane J, Attie KM, et al. Six-year results of a randomized prospective trial of human growth hormone and oxandrolone in Turner syndrome. J Pediatr 1992; 121: 49–55.PubMedCrossRefGoogle Scholar
- 21.Stahnke N, Stubbe P, Keller E. Recombinant human growth hormone and oxandrolone in treatment of short stature in girls with Turner syndrome. Horm Res 1992; 37 (suppl 2): 37–46.PubMedCrossRefGoogle Scholar
- 22.Nilsson KO, Swedish Paediatric Study Group for Growth Hormone Treatment. The Swedish somatonorm Turner trial: two-year results. Acta Paediatr Scand Suppl 1989; 356: 160A.Google Scholar
- 23.Takano K, Shizume K, Hibi I. Turner’s syndrome: treatment of 203 patients with recombinant human growth hormone for one year. Acta Endocrinol (Copenh) 1989; 120: 559–68.Google Scholar
- 24.Takano K, Shizume K, Hibi I. Treatment of 46 patients with Turner’s syndrome with recombinant human growth hormone for three years: a multicentre study. Acta Endocrinol (Copenh) 1992; 126: 296–302.Google Scholar
- 25.Rongen-Westerlaken C, van Es A, Wit J-M, et al. Growth hormone therapy in Turner’s syndrome. Am J Dis Child 1992; 146: 817–20.PubMedGoogle Scholar
- 26.Massa G, Vanderschueren-Lodeweyckx M. Growth promoting effect of growth hormone and low dose ethinyl estradiol in girls with Turner syndrome: 4-year results. In: Hibi, I, Takano, K eds. Basic and clinical approach to Turner syndrome. Elsevier Science, 1993: 327–32.Google Scholar
- 27.Smith MA, Wilson J, Price WH. Bone demineralisation in patients with Turner’s syndrome. J Med Genet 1982; 19: 100–3.PubMedCrossRefGoogle Scholar
- 28.Naeraa RW, Brixen K, Hansen RM, et al. Skeletal size and bone mineral content in Turner’s syndrome: relation to karyotype, estrogen treatment, physical fitness, and bone turnover. Calcif Tissue Int 1991; 49: 77–83.PubMedCrossRefGoogle Scholar
- 29.Kirkland RT, Lin T-H, LeBlanc AD, Kirkland JL, Evans HJ. Effects of hormonal therapy on bone mineral density in Turner syndrome. In: Rosenfeld RG, Grumbach MM, eds. Turner syndrome. New York: Marcel Dekker, 1990: 319–25.Google Scholar
- 30.Ross JL, Long LM, Feuillan P, Cassorla F, Cutler GB. Normal bone density of the wrist and spine and increased wrist fractures in girls with Turner’s syndrome. J Clin Endocrinol Metab 1991; 73: 355–9.PubMedCrossRefGoogle Scholar
- 31.Neely EK, Marcus R, Rosenfeld RG, Bachrach LK. Turner syndrome adolescents receiving growth hormone are not osteopenic. J Clin Endocrinol Metab 1993; 76: 861–6.PubMedCrossRefGoogle Scholar