Abstract
The main clinical feature of Turner syndrome (TS) is growth failure, with a mean spontaneous adult height ranging between 136 and 147 cm, according to the specific curves of various populations. Though a classical deficiency of GH has not been generally demonstrated, GH has been administered since 1980 in trials, using replacement doses just initially, with a subsequent trend to increase it. We report the outcome of GH therapy given at the fixed dose of 0.33 mg/kg/week in 60 TS girls observed until adult height; 59 untreated TS girls, matched for auxological, karyotypical characteristics and time of observation, born within the same decade served as controls to evaluate GH efficacy. The calculation of the gain in cm over PAH was performed on specific Italian Turner curves, as well as height evaluation as SD score and growth velocity. The same calculations were made using Lyon references and Tanner standards. The mean CA at the beginning of GH treatment was 10.9±2.76 yr (range 4.5–15.9). Mean adult height of treated group was 151±6.1 cm with a gain over the PAH calculated at start of therapy (142.9±5.3 cm) of 8.2±3.9 cm. Ns change was observed between the PAH at first observation (143.6±7.0 cm) and adult height (144.3±5.6 cm) in the control group. Treatment was well tolerated, no relevant side effects were observed, glucose metabolism resulted no more affected than in untreated subjects, IGF-I levels remained within 2 SD. Our results in 60 TS girls, though the dose remained unchanged throughout the treatment, show a good response, characterized by a striking variability in each patient (mean gain in cm over PAH at adult height of 8.17±3.9, range 3–21 cm), and significant also in comparison with the control group. As the chronological age at start of therapy ranged between 4.5 to 15.9 yr, the results were further evaluated dividing the patients into two groups, according to the age, < or >11 yr. Thirty girls were <11 yr (mean 8.7±1.76 yr) and 30 were >11 yr (mean 13.2±1.4 yr). The gain in cm over the PAH in each group was, respectively, 8.1±3.4 and 8.2±4.3 cm without any significant difference between the two groups, showing no negative correlation between the CA at the beginning of GH and the response to treatment.
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Lyon AJ, Preece MA, Grant DB. Growth curve for girls with Turner syndrome. Arch Dis Child 1985, 60: 932–5.
Ranke MB, Stubbe P, Majewski F, Bierich JR. Spontaneous growth in Turner’s syndrome. Acta Paediatr Scand 1988, 343 (Suppl): 22–30.
Lippe BM, Plotnick L, Attie KM, Frane J. Growth in Turner syndrome: updating the United States experience. In: Hibi I, Takano K eds. Basic and clinical approach to Turner syndrome. Amsterdam: Elsevier. 1993, 77–82.
Bernasconi S, Larizza D, Benso L, et al. Turner’s syndrome in Italy: familial characteristics, neonatal data, standards for birth weight and for height and weight from infancy to adulthood. Acta Paediatr 1994, 83: 292–8.
Rongen-Westerlaken C, Corel L, van den Broeck J, et al. Reference values for height, height velocity and weight in Turner’s syndrome. Swedish Study Group for GH treatment. Acta Paediatr 1997, 86: 937–42.
Wit JM, Massarano AA, Kamp GA, et al. Growth hormone secretion in patients with Turner’s syndrome as determined by time series analysis. Acta Endocrinol (Copenh) 1992, 127: 7–12.
Pasquino AM, Bernandini S, Cianfarani S, et al. GH assessment and three years hGH therapy in girls with Turner syndrome. Horm Res 1992, 38: 120–4.
Rao W, Weiss B, Fukami M, et al. Pseudoautosomal deletions encompassing a novel homeobox gene cause growth failure in idiopathic short stature and Turner syndrome. Nat Genet 1997, 16: 54–63.
Ross JL, Scott C, Marttila P, et al. Phenotypes associated with SHOX deficiency. J Clin Endocrinol Metab 2001, 86: 5674–80.
Rosenfeld RG, Frane J, Attie KMA, 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.
Vanderschueren-Lodeweyckx M, Massa G, Maes M, 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.
De Schepper J, Craen M, Massa G, et al. Growth hormone therapy in Turner’s syndrome: one versus two daily injections. J Clin Endocrinol Metab 1994, 79: 489–94.
Van den Broeck J, Massa CG, Attanasio A, et al. Final height after long-term growth hormone treatment in Turner syndrome. European Study Group. J Pediatr 1995, 127: 729–35.
Massa G, Otten BJ, De Muinck Keizer-Schrama S, et al. Treatment with two growth hormone regimens in girls with Turner syndrome: final height results. Dutch Growth Hormone Working Group. Horm Res 1995, 43: 144–6.
Tillmann V, Bucknall JL, Price DA, Clayton PE. Growth to final height in Turner syndrome factors predicting the response to growth hormone therapy. Horm Res 1995, 44 (Suppl 1): 279.
Frisch H, Hauesler G, Schmitt K, Blumel P, Plochl E, Waldhor T. Conventional GH doses in combination with oxandrolone improve final height in Turner syndrome: effect of age at start of therapy on bone age development and final height. Horm Res 1995, 44 (Suppl 1): 280.
Rochiccioli P, Battin J, Bertrand AM, et al. Final height in Turner syndrome patients treated with growth hormone. Horm Res 1995, 44: 172–6.
Nilsson KO, Albertsson-Wikland K, Alm J, et al. Improved final height in girls with Turner’s syndrome treated with growth hormone and oxandrolone. J Clin Endocrinol Metab 1996, 81: 635–40.
Pasquino AM, Passeri F, Municchi G, et al. Final height in Turner syndrome patients treated with growth hormone. Horm Res 1996, 46: 269–72.
Taback SP, Collu R, Deal CL, et al. Does growth hormone supplementation affect adult height in Turner’s syndrome? Lancet 1996, 348: 25–7.
Chu CE, Peterson WF, Kelnar CJ, Smail PJ, Greene SA, Donaldson MD. Variable effect of growth hormone on growth and final adult height in Scottish patients with Turner’s syndrome. Acta Paediatr 1997, 86: 160–4.
Carel JC, Mathivon L, Gendrel C, Ducret JP, Chaussain JL. Near normalization of final height with adapted doses of growth hormone in Turner’s syndrome. J Clin Endocrinol Metab 1998, 83: 1462–466.
Schweizer R, Ranke MB, Binder G, et al. Experience with growth hormone therapy in Turner sindrome in a single centre: low total gain, no further gains after puberty onset and unchanged body proportions. Horm Res 2000, 53: 228–38.
Haeusler G. Growth hormone therapy in patients with Turner syndrome. Horm Res 1998, 49 (Suppl 2): 62–6.
Plotnick L, Attie KM, Blethen SL, Sy JP. Growth hormone treatment of girls with Turner syndrome: the national cooperative growth study experience. Pediatrics 1998, 102: 479–81.
Rosenfeld RG, Attie KM, Frane J, et al. Growth hormone therapy of Turner’s syndrome: beneficial effect on adult height. J Pediatr 1998, 132: 319–24.
Cacciari E, Mazzanti L, and the Italian Study Group for Turner syndrome. Final height of patients with Turner’s syndrome treated with growth hormone (GH): indications for GH therapy alone at high doses and late estrogen therapy. J Clin Endocrinol Metab 1999; 84: 4510–5.
Hochberg Z, Zadik Z. Final height in young women with Turner syndrome after GH therapy: an open controlled study. Eur J Endocrinol 1999, 141: 218–24.
Sas TC, de Muinck Keizer-Schrama SMPF, Stijnen T, et al. Normalization of height in girls with Turner syndrome after long term growth hormone treatment: results of a randomized dose-response trial. J Clin Endocrinol Metab 1999, 84: 4607–12.
Chernausek SD, Attie KM, Cara JF, et al. Growth hormone therapy of Turner syndrome: the impact of age of estrogen replacement on final height. J Clin Endocrinol Metab 2000, 85: 2439–45.
Reiter EO, Blethen SL, Baptista J, Price L. Early initiation of growth hormone treatment allows age-appropriate estrogen use in Turner’s syndrome. J Clin Endocrinol Metab 2001, 86: 1936–41.
Quigley CA, Crowe BJ, Anglin DG, Chipman JJ, and the US Turner syndrome study group. Growth hormone and low dose estrogen in Turner syndrome: results of a United States multi-center trial to near-final height. J Clin Endocrinol Metab 2002, 87: 2033–41.
Ranke MB, Partsch CJ, Lindberg A, et al. Adult height after GH therapy in 188 Ullrich-Turner syndrome patients: results of the German IGLU follow-up study. 2001. Eur J Endocrinol 2002, 147: 625–33.
Van Pareren YK, de Muinck Keizer-Schrama SM, Stijen T, et al. Final height in girls with Turner syndrome after long-term growth hormone treatment in three dosages and low estrogens. J Clin Endocrinol Metab 2003, 88: 1119–25.
Massa G, Heinrichs C, Verlinde S, et al. Late or delayed induced or spontaneous puberty in girls with Turner syndrome treated with growth hormone does not affect final height. J Clin Endocrinol Metab 2003, 88: 4168–74.
Tanner JM, Whitehouse RH, Takaishi M. Standards from birth to maturity for height, weight, height velocity and weight velocity: British children, 1965. Arch Dis Child 1966, 41: 454–71.
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Pasquino, A.M., Pucarelli, I., Segni, M. et al. Adult height in sixty girls with Turner syndrome treated with growth hormone matched with an untreated group. J Endocrinol Invest 28, 350–356 (2005). https://doi.org/10.1007/BF03347202
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DOI: https://doi.org/10.1007/BF03347202