2013, Number 2
<< Back Next >>
Rev Cuba Endoc 2013; 24 (2)
Preliminary study on the recombinant human growth hormone treatment of Turner's syndrome
Espinosa RTM, Pérez SLA, Martínez MMÁ, Carvajal MCF
Language: Spanish
References: 44
Page: 161-175
PDF size: 92.62 Kb.
ABSTRACT
Introduction: Turner's syndrome patients present with total or partial monosomy
of X gonosome, general disgenesia, several typical physical traits, short height and
sexual infantilism.
Objective: to evaluate the effect of recombinant human growth hormone-based
treatment on the height of patients clinically and chromosomally diagnosed as
Turner's syndrome subjects.
Methods: a retrospective and descriptive study was conducted in Turner's syndrome
patients, who had been seen from May 2003 to May 2004 at the pediatric endocrinology
department of the National Institute of Endocrinology. The sample was made up of
19 girls divided into 2 groups. Group A comprised patients who were treated with
recombinant human growth hormone (n= 9) at a dose of 0.14 IU/kg/day
subcutaneously administered from 8:30 to 9:30 pm. Group B included the patients
who did not receive this treatment (n= 10). The required data for the research
stemmed from the medical history check-ups.
Results: increase of the Group A patients' height, whose mean basal height at the
beginning of the study was just 131.7 ± 7.5 cm and after one year of treatment,
they reached 137.9 ± 7.1 cm, at a rate of average growth of 6.2 ± 2.3 cm/year.
The comparison of both groups after one year showed significant differences in
mean height (p= 0.0071) and mean growth a year (p= 0.0032).
Conclusions: the treatment of these patients with the recombinant growth
hormone during the first year was effective, since it markedly accelerated the rate
of growth in girls with Turner's syndrome. The body weight gain proved to be
adequate in the study period, because it managed to keep steady nutritional
assessment without changes in the percentile canal. Inducement to puberty did not
alter the final height prognosis at the end of the study.
REFERENCES
Turner HH. A syndrome of infantilism, congenital webbed neck, and cubits valgus. Endocrinology. 1938;23:566-74.
Lyon AJ, Preece Ma, Grant DB. Growth curve for girls with Turner syndrome. Arch Dis Child. 1985;60:932-5
Padrón RS, Barón JA, Arce B. Correlación fenotipo-cariotipo en el síndrome de Turner. Rev Cub Med. 1981;20:62-7.
Cavallo L, Gurrado R. Endogenous growth hormone secretion does not correlate with growth in patients with Turner's syndrome. J Pediatr Endocrinol Metab. 1999;12:623-7.
Massa G, Vanderchueren-Lodewyckx M, Malvaux P. Linear growth in patients with Turner' syndrome: influence of spontaneous puberty and parental height. Eur J Pediar. 1990;149:246-50.
Page LA. Final heights in 45 X Turner syndrome with spontaneous sexual development. Review of European and Americans reports. J Pediatr Endocrinol. 1993;6:153-8.
Ogata T, Muroya K, Matsuo N. Structure function relation of the X chromosome in Turner Syndrome. En: Saenger P, Pasquino AM, eds. Optimising health care for Turner patients in the 21st century. Amsterdam: Elsevier; 2000. p. 9-18.
Ellison WJ, Wardak Z, Yonung MF, Gehron-Rebey P, Laig-Webster M, Chiong W. PHOG, a candidate gene for involvement in the short stature of Turner syndrome. Hum Mol Genet. 1997;6:1341-7.
Rao E, Weiss B, Fukami M, Rump A, Niesler B, Mertz A, 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.
Kosho T, Muroya K, Nagai T, Fujimoto M, Yokoya S, Sakamoto H, et al. Skeletal features and growth patterns in 14 patients with haplo insufficiency of SHOX: implications for the development of Turner syndrome. J Clin Endocrinol Metab. 1999;84:4613-21.
Takano K, Ogawa M, Tanaka T, Tachibana K, Fujira K, Hisuka N. Clinical trials of GH treatment in patients with Turner's syndrome in Japan-a consideration of final height. J Endocrinol. 1997;137:138-45.
Nelly EK, Rosenfeld RG. Turner Syndrome. En: Lifshitz F. Pediatric Endocrinology. 3a ed. New York: Marcel Dekker; 1996. p. 267-80.
Ross JL, Long LM, Loriaux DL, Cutler GB, Growth hormone secretory dynamics in Turner syndrome. J Pediatr. 1985;106:202-6.
Rongen-Westerlanken C, van Es A, Wit J-M. Growth hormone therapy in Turner's syndrome. J Des Child. 1992;146:817-20.
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.
Rosenfield RG, Frane J, Attie KM. Six year results of a randomized prospective trial of human growth hormone and oxandrolone in Turner Syndrome. J Pedriatr. 1992;121:49-55.
Garcia RC, Martinez A, Helnrich JJ, Kelcenman A, Lejaraga N, Laspiur M. Growth in Argentinean girls with Turner syndrome. Ann Hum Biol. 1995;22:533-44.
Rochiccioh P, Battin J, Bertrand AM, Bost M, Cabrol S. Final height in Turner syndrome patients treated with growth hormone. Horm Res. 1995;44:172-6.
Ferrández A, Labarta JI, Calvo M, Mayayo E, Puga B, Cáncer E, et al. Síndrome de Turner. En: Pombo M. Tratado de Endocrinología Pediátrica. 3a ed, Madrid: McGraw-Hill Interamericana; 2002. p. 780-803.
Luzuriaga C. Tratamiento con hormona de crecimiento y aspectos básicos de su administración. En: Luzuriaga C. Crecimiento y hormona de crecimiento. Salamanca: Técnica de congresos TESITEX; 1994. p. 167-91.
Argente OJ, Carrascosa LA, Gracia BR. Pruebas Funcionales en Endocrinología Pediátrica y de la Adolescencia. En: Argente OJ, Carrascosa LA, Gracia BR, Rodríguez HF. Tratado de Endocrinología Pediátrica y de la Adolescencia. Madrid: EDITORES MÉDICOS, S.A; 1995. p. 1075-136.
Vicen CE. Método de exploración de la secreción hormonal adeno-hipofisaria. En: Pombo M. Tratado de Endocrinología Pediátrica. 3a ed, Madrid: McGraw-Hill Interamericana; 2002. p. 403-9.
Luzuriaga C. Valoración de la secreción de Hormona de Crecimiento y Somatomedina. Prueba analítica. En: Luzuriaga C. Crecimiento y Hormona de crecimiento, Salamanca: Técnica de congresos TESITEX. 1994. p. 115-40.
Bona X, Luzuriaga C. Materiales y métodos para la medida y valoración del crecimiento. En: Luzuriaga C. Crecimiento y Hormona de crecimiento. Salamanca: Técnica de congresos TESITEX; 1994. p. 219-36.
Fernández RM. Patrón de crecimiento humano y su evaluación. En: Pombo M. Tratado de Endocrinología Pediátrica. 3a. ed. Madrid: McGraw-Hill Interamericana; 2002. p. 244-74.
Garagori JM, Moreno LA. Talla baja familiar y retraso constitucional del crecimiento y el desarrollo. En: Argente OJ, Carrascosa LA, Gracia BR, Rodríguez HF. Tratado de Endocrinología Pediátrica y de la Adolescencia. Madrid: EDITORES MÉDICOS, S.A; 1995. p. 141-53.
Pozo J, Argente J. Crecimiento: Valoración auxológica. En: Argente J, Carrascosa A, Gracia R, Rodríguez F. Tratado de Endocrinología Pediátrica y de la Adolescencia. 2a ed. Barcelona: Doyma; 2000. p. 177-200.
Rodríguez HF, Ballester F. Disgenesias Gonadales. En: Argente OJ, Carrascosa LA, Gracia BR, Rodríguez HF. Tratado de Endocrinología Pediátrica y de la Adolescencia. Madrid: EDITORES MÉDICOS, S.A; 1995. p. 141-53.
García NL, Luis DA, Lus SP. Uso de la hormona de crecimiento biosintética en el síndrome de Turner. En: Raúl CL, Luis DA. Fisiopatología de la pubertad y el embarazo/ /anticoncepción en la adolescencia. México: Dorantes Alvarez; 1997. p. 371-85.
Elsheikh M, Dunger DB, Conway GS, Wass JA. Turner syndrome in adulthood. Endocrinol rev. 2002;23:120-40.
Sybert VP. The adult patient with Turner syndrome. En: Albertsson WK, Ranke MB. Turner syndrome in a life span perspective: research and clinical aspects. Amsterdam: Elsevier; 1995. p. 205-18.
Weiss L. Additional evidence of gradual loss of germ cells in the pathogenesis of streak ovaries in Turner's syndrome. J Med Genet. 1971;8:540-4.
Elsheikh M, Wass JAH, Conway GS. Autoimmune thyroid disease in women with Turner's syndrome-the association with karyotype. Clin Endocrinol. 2001;55:223-6.
Stephure KD. Turner Syndrome. International Symposium on A Current of Pediatric Endocrinology. 1997;105-10.
Radetti G, Mazzanti L, Paganini C, Bernasconi S, Russo G, Rigon F, et al. Frequency, clinical and laboratory features of thyroiditis in girls with Turner's syndrome. The Italian Study Group for Turner 's syndrome. Acta Paediatr. 1995;84:909-12.
Rosenfeld RG and the Genentech National Cooperative Study Group. Growth hormone therapy in Turner's syndrome: an update on final height. Acta Paediatr Suppl. 1992;383:2-3.
Van den Broeck J, Massa GG, Attanasio A, Matranga A, Chaussain JL, Price DA, et al. Final height after long-term growth hormone treatment in Turner syndrome. J Pediat. 1995;127:729-35.
Haeusler G, Schmitt K, Blümel P, Plöchl E, Waldhör TH, Frisch H. Growth hormone in combination with anabolic steroids in patients with Turner syndrome: effect on bone maturation and final height. Acta Peadiatr. 1996;85:1408-14.
Massa G, Heinrichs C, Verlinde S, Thomas M, Bourguignon JP, Croen M, et al. Late or delayed induced or spontaneous puberty in girls with Turner syndrome treated with growth hormone does not affect final height. International Growth Monitor Rev. 2004;14:15-7.
Chernausek SD, Attie KM, Cara JF, Rosenfeld RG, Frane J. Growth hormone therapy of Turner syndrome: the impact of age of estrogen replacement on height. International Growth Rev Monitor. 2002;11:20-2.
Hochberg Z, Zadik Z. Final height in young women with Turner syndrome after GH therapy: an open controlled study. Eur Endocrinol. 1999;141:218-24.
Atti KM, Chernausek S, Frane J. Growth hormone use in Turner syndrome: a preliminary report on the effect of early versus delayed estrogen. En: Albertsson WK, Ranke MB. Turner syndrome in a life-span perspective. Research and clinical aspects. Amsterdam: Elsevier Science; 1995. p. 175-81.
Werther G, Diestsch S. Multicentre trial of synthetic growth hormone and low dose estrogen in Turner syndrome: analysis of final height. En: Albertsson WK, Ranke MB. Turner syndrome in a life-span perspective. Research and clinical aspects. Amsterdam: Elsevier Science; 1995. p. 105-12.
Nilsson OK, Albersson WK, Alm J. Improved final height in girls with Turner's syndrome treated with growth hormone and oxandrolone. J Clin Endocrinol Metab. 1996;81:635-40.