Preview

Problems of Endocrinology

Advanced search

Experience with the use of domestic somatotropin Rastan for the treatment of children with Turner syndrome

https://doi.org/10.14341/probl201056311-15

Abstract

The objective of the present study was to evaluate the efficiency and safety of the treatment of girls presenting with Turner syndrome by recombinant growth hormone Rastan (Farmstandart-UfaVITA). A total 29 girls in this condition were available for observation. Their chronological age at the time of diagnosis averaged 7.9±1.2 years at the time of diagnosis and 9.8±1.6 at the onset of therapy; the bone age was 7.5±1.1 years. Duration of the treatment was 24 months. Recombinant somatotropin was used in the form of lyophilisate (1.33 mg) to prepare a solution for subcutaneous injections at a dose of 0.05 mg/kg of body weight. The solution was administered daily at evening hours. The mean growth rate of the patients with Turner syndrome was estimated at 4.2±0.6 cm/year prior to Rastan therapy, 8.7±0.6 cm/year during the first 12 months of the treatment, and 6.1±1.2 cm/yr in the second year. Overall, the height of the girls increased by 0.84 SD within the two years. No adverse effects of therapy were documented. It is concluded that the use of recombinant somatotropin Rastan is an efficacious and safe therapeutic modality for the acceleration of growth in girls with Turner syndrome.

References

1. Волеводз Н.Н. Системные и метаболические эффекты гормона роста у детей с различными вариантами низкорослости: Автореф. дис. … д-ра мед. наук. М 2005;283.

2. Дедов И.И., Волеводз Н.Н., Петеркова В.А. Синдром Шерешевского-Тернера. М 2009;56.

3. Дедов И.И., Петеркова В.А. Руководство по детской эндокринологии. М: Универсум Паблишинг 2006.

4. Lyon A.J., Preece M.A., Grant D.B. Growth curve for girls with Turner syndrome. Arch Dis Child 1985;60:10:932-935.

5. Park E., Bailey J.D., Cowell C.A. Growth and maturation of patients with Turner's syndrome. Pediat Res 1983;17:1:1-7.

6. Ranke M.B., Stubbe P., Majewski F. Spontaneous growth in Turner syndrome. Acta Paediat Scand 1988;343:Suppl:22-30.

7. Ranke M.B., Pfluger H., Rosendahl W. et al. Turner syndrome: spontaneous growth in 150 cases and review of the literature. Eur J Pediat 1983;141:2:81-88.

8. Chen H., Faigenbaum В. Psychosocial aspects of patients with the Ullrich-Turner syndrome. Am J Med Genet 1981;8:2:191- 203.

9. Tanner J.M., Whitehouse R.H. Clinical longitudinal standards for height, weight, height velocity, and the stages of puberty. Arch Dis Child 1976;51:170-179.

10. Ranke M.B. An introduction to Turners syndrome. Oxford Clinical Communications 1990.

11. Tanner J.M. Growth at Adolescence. 2nd ed. Oxford: Blackwell 1962.

12. Greulich W.W., Pule S.I. Radiographic atlas of skeletal development of the hand and wrist. 2nd ed. Stanford: University Press 1959.

13. Rao E., Weiss B., Fukami M., Rump A. Pseudoautosomal deletion encompassing a novel homeobox gene: cause of growth failure in idiopathic short stature and Turner syndrome. Nat Genet 1997;16:54-63.

14. Guyda H.J. Four Decades of Growth Hormone Therapy for Short Children: What Have We Achieved? J Clin Endocrinol Metab 1999;84:12:4307-4316.

15. Haeusler G., Schmitt K., Blumel P. et al. Growth hormone in combination with anabolic steroids in patients with Turner syndrome: effect on bone maturation and final height. Acta Paediat 1996;85:1408-1414.

16. Rosenfeld R.G., Attie K.M., Frane J. et al. Growth hormone therapy of Turner's syndrome: beneficial effect on adult height. J Pediat 1998;132:2:319-324.

17. Rosenfeld R.G. Growth hormone therapy in Turner's syndrome: an update on final height. Genentech National Cooperative Study Group. Acta Paediat 1992;Suppl:383:3-6.

18. Sas T.C., de Muinck Keizer-Schrama S.M., 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;84:12:4607-4612.

19. Baxter L., Bryant J., Cave C.B., Milne R. Recombinant growth hormone for children and adolescents with Turner syndrome. Cochrane Database Syst Rev 2007;24:1.

20. Chu C.E., Paterson W.F., Kelnar C.J. et al. Variable effect of growth hormone on growth and final adult height in Scottish patients with Turner's syndrome. Acta Paediat 1997;86:2:160-164.

21. Hsu P.Y., Tung Y.C., Tsai W.Y. et al. Effect of growth hormone therapy on adult height of children with Turner syndrome. J Formos Med Ass 2008;107:9:704-709.

22. Khadilkar V.V., Khadilkar A.V., Nandy M., Maskati G.B. Growth hormone in turner syndrome. Indian Pediat 2006;43:3:236-240.

23. Morin A., Guimarey L.M., Apezteguia M., Santucci Z.C. Adult height in Turner Syndrome girls after long-term growth hormone treatment. Medicina (B Aires) 2009;69:4:431-436.

24. 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 Inv 2005;28:4:350-356.

25. Soriano-Guillen L., Coste J., Ecosse E. et al. Adult height and pubertal growth in Turner syndrome after treatment with recombinant growth hormone. J Clin Endocrinol Metab 2005;90:9:5197-5204.

26. Stephure D.K. Canadian Growth Hormone Advisory Committee. Impact of growth hormone supplementation on adult height in turner syndrome: results of the Canadian randomized controlled trial. J Clin Endocrinol Metab 2005;90:6:3360-3366.

27. Van den Broeck J., Massa G.G., Attanasio A. et al. Final height after long-term growth hormone treatment in Turner syndrome. J Pediat 1995;127:729-735.

28. Davenport M.L., Crowe B.J., Travers S.H. et al. Growth hormone treatment of early growth failure in toddlers with Turner syndrome: a randomized, controlled, multicenter trial. J Clin Endocrinol Metab 2007;92:9:3406-3416.

29. Ranke M.B., Wilton P. Growth homone therapy in KIGS: 10 years' experience. Leipzig: Barth 1999.

30. Ranke M.B., Lindberg A., Ferrández Longás A. et al. KIGS International Board. Major determinants of height development in Turner syndrome (TS) patients treated with GH: analysis of 987 patients from KIGS. Pediat Res 2007;61:1:105-110.


Review

For citations:


Dedov I.I., Volevodz N.N., Malievskiĭ O.A., Peterkova V.A. Experience with the use of domestic somatotropin Rastan for the treatment of children with Turner syndrome. Problems of Endocrinology. 2010;56(3):11-15. https://doi.org/10.14341/probl201056311-15

Views: 555


ISSN 0375-9660 (Print)
ISSN 2308-1430 (Online)