European Journal of Pediatrics

, Volume 155, Issue 10, pp 851–857

Growth hormone therapy in Silver Russell Syndrome: 5 years experience of the Australian and New Zealand Growth database (OZGROW)

  • Y. Rakover
  • S. Dietsch
  • G. R. Ambler
  • C. Chock
  • M. Thomsett
  • C. T. Cowell


Data were analysed on 33 children (22 males) with Silver Russell syndrome treated with growth hormone for periods up to 5 years. Baseline data (medians) at commencement of growth hormone (GH) therapy were age 6.7 years, bone age delay 1.7 years, height standard deviation score (SDS)-3.2, weight SDS −3.1, and growth velocity 5.7 cm/year. All were prepubertal. Median birth weight SDS for gestational age was −3.2. GH was commenced at 14 IU/m2 per week and subsequently adjusted according to response. Growth velocity and growth velocity SDS for chronological age (CA) improved over baseline and gains in height SDS for CA were 1.0, 1.5 and 1.8 SD over 3, 4 and 5 years respectively (P < 0.001). No significant increase in height SDS for bone age was observed. Increased GH doses were required after the 1st year to maintain growth rates. Mean bone age advancement was 3.1 years after 3 years of treatment, and 6.0 years after 5 years treatment. Younger age was a predictor of the growth response over the 1st year. Predictors of response after 3 years were catch-up growth, low weight SDS at birth and low height SDS for CA. Age at onset of puberty was normal, but height at onset of puberty was lower than normal means.

Key words

Silver Russell syndrome Growth hormone Growth Intrauterine growth retardation 



bone age


chronological age


estimated mature height


growth hormone


growth velocity


intrauterine growth retardation


mid-parental height


standard deviation score


Silver Russell syndrome


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  1. 1.
    Albanese A, Stanhope R (1993) Growth and metabolic data following treatment of children with intrauterine growth retardation. Horm Res 39:8–12Google Scholar
  2. 2.
    Albertsson-Wikland K (1989) Growth hormone secretion and growth hormone treatment in children with intrauterine growth retardation. Acta Paediatric Scand [Suppl] 349:35–41Google Scholar
  3. 3.
    Albertsson-Wikland K, Kallberg J (1994) Natural growth in children born small for gestational age with and without catch-up growth. Acta Paediatr Scand [Suppl] 343:23–30Google Scholar
  4. 4.
    Angehrn V, Zachmann M, Prader A (1979) Silver-Russell syndrome, observation in 20 patients. Helv Paediatr Acta 34:397–308Google Scholar
  5. 5.
    Balsamo A, Tassoni P, Cassio A, Colli C, Tassinari D, Cicognani A, Cacciari E (1995) Response to growth hormone therapy in patients with growth hormone deficiency who at birth were small or appropriate in size for gestational age. J Pediatr 126:474–477Google Scholar
  6. 6.
    Buckler J (1990) A longitudinal study of adolescent growth. Springer, Berlin Heidelberg New York LondonGoogle Scholar
  7. 7.
    Bundiak R, Hindmarsh PC, Smith PJ, Brook CG (1988) Long term auxologic effects of human growth hormone. J Paediatr 112:875–879Google Scholar
  8. 8.
    Chatelain PG (1993) Auxology and response to growth hormone treatment of patients with intrauterine growth retardation or Silver Russell syndrome: analysis of data from the Kabi pharmacia International Growth Study. Acta Paediatric Scand [Suppl] 391:79–81Google Scholar
  9. 9.
    Chatelain P, Nicolino M (1994) Intrauterine growth retardation and Silver Russell Syndrome:demography, auxulogy and response to growth hormone treatment in the Kabi International Growth Studies. In:Ranke MB, Gunnarsson R (eds) Progress in Growth hormone therapy - 5 years of KIGS. J and J Verlag, Hannheim, pp 230–239Google Scholar
  10. 10.
    Cowell CT, Wilcox L, Moore B, Likitmaskul S (1993) The effects of growth hormone beyond the first year in non growth hormone deficient children. In: Cowell CT, Ho KKY, Werther GA (eds) Growth and sexual development. Harwood Academic, Switzerland, pp 91–102Google Scholar
  11. 11.
    Cowell CT, Dietsch S, Greenacre P. Growth hormone deficient and non growth hormone deficient children: The OZGROW experience. (in press)Google Scholar
  12. 12.
    Davies PSW, Valley R, Preece MA (1988) Adolescent growth and pubertal progression in the Silver-russell syndrome. Arch Dis Child 63:130–135Google Scholar
  13. 13.
    Duncan PA, Hall JG, Shapiro LR, Vibert BK (1990) Three-generation dominant transmission of the Silver-Russell syndrome. Am J Med Genet 35:245–250Google Scholar
  14. 14.
    Escobar V, Gleiser S, Weaver DD (1978) Phenotypical and genetic analysis of the Silver-Russell Syndrome. Clin Genet 13:278–288Google Scholar
  15. 15.
    Greulich WW, Pyle SI (1959) Radiographic atlas of skeletal development of the hands and wrist 2nd ed. Stanford University Press, Palo Alto, CaliforniaGoogle Scholar
  16. 16.
    Hamill PRV, Drizd TA, Johnson CL, Reed PR, Roche AF (1977) NCHS growth curves for children from birth to 18 years. DHEW publication no. (PHS) 78-1650, United States, 165:1–74Google Scholar
  17. 17.
    Niklasson A, Ericson A, Fryer JG, Karlberg J, Lawrence C, Karlberg P (1991) An update of the Swedish reference standards for weight, length and head circumference at birth for given gestational age (1977–1981). Acta Paediatr Scand 80:756–762Google Scholar
  18. 18.
    Patton MA (1988) Russell-Silver Syndrome. J Med Genet 25:557–560Google Scholar
  19. 19.
    Ranke MB, Stubbe P, Majewski F, Bierich JR (1988) Spontaneous growth in Turner's syndrome. Acta Paediatr Scand [Suppl] 343:22–30Google Scholar
  20. 20.
    Ranke MB, Guilbaud O, Lindberg A, Cole T (1993) Prediction of the growth response with various growth disorders treated with growth hormone: analyses of data from the Kabi Pharmacia International Growth Study. Acta Paediatr Scand [Suppl] 391:82–88Google Scholar
  21. 21.
    Russell A (1954) A syndrome of intrauterine dwarfism, recognisable at birth with craniofacial dysostosis, disproportionately short arms and other anomalies (5 examples). Proc Roy Soc Med 117:1040–1044Google Scholar
  22. 22.
    Saal HM, Pagon RA, Pepin MG (1985) Reevaluation of Russell-Silver Syndrome. J Paediatr 107:533–737Google Scholar
  23. 23.
    Silver HK (1964) Asymmetry, short stature and variations in sexual development. A syndrome of congenital malformations. Am J Dis Child 107:495–513Google Scholar
  24. 24.
    Silver HK, Kiyasu W, George J, Deamer WC (1953) Syndrome of congenital hemihypertrophy, shortness of stature and elevated urinary gonadotropins. Pediatrics 12:368–375Google Scholar
  25. 25.
    SPIDA version [computer program] (1993) Sydney: Statistical Computing Laboratory, Macquarie UniversityGoogle Scholar
  26. 26.
    Stanhope R, Hamill AG, Clayton J, Jones J, Preece MA (1989) Physiological growth hormone secretion and response to growth hormone treatment in children with short stature and intrauterine growth retardation. Acta Paediatr Scand [Suppl] 349:47–52Google Scholar
  27. 27.
    Stanhope R, Preece MA, Hamill G (1991) Does growth hormone treatment improve final height attainment of children with intrauterine growth retardation? Arch Dis Child 66:1180–1183Google Scholar
  28. 28.
    Tanaka T (1994) Pubertal aspects of idiopathic growth hormone deficiency. In:Ranke MB, Gunnarsson R (eds) Progress in growth hormone therapy - 5 years of KIGS. J and J Verlag, Mannheim, pp 112–128Google Scholar
  29. 29.
    Tanner JM, Davis PSW (1985) Clinical longitudinal standards for height and height velocity for North American children. J Paediatr 107:317–329Google Scholar
  30. 30.
    Tanner JM, Lejarraga H, Cameron N (1975) The natural history of the Silver-Russell syndrome: a longitudinal study of thirty-nine cases. Paediatr Res 9:611–623Google Scholar
  31. 31.
    Wallis S, Harvey DR (1980) Small-for dates babies, their problems and their future in:Wharton BA (ed) Topics in perinatal medicine. Pitman Medical, London, pp 50–63Google Scholar

Copyright information

© Springer-Verlag 1996

Authors and Affiliations

  • Y. Rakover
    • 1
  • S. Dietsch
    • 1
  • G. R. Ambler
    • 1
  • C. Chock
    • 1
  • M. Thomsett
    • 2
  • C. T. Cowell
    • 1
  1. 1.The Robert Vines Growth Research CentreRoyal Alexandra Hospital for ChildrenWestmeadAustralia
  2. 2.Mater Children's and the Royal Children's HospitalsBrisbaneAustralia

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