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)

Authors

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

DOI: 10.1007/BF02282833

Cite this article as:
Rakover, Y., Dietsch, S., Ambler, G.R. et al. Eur J Pediatr (1996) 155: 851. doi:10.1007/BF02282833

Abstract

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 syndromeGrowth hormoneGrowthIntrauterine growth retardation

Abbreviations

BA

bone age

CA

chronological age

EMH

estimated mature height

GH

growth hormone

GV

growth velocity

IUGR

intrauterine growth retardation

MPH

mid-parental height

SDS

standard deviation score

SRS

Silver Russell syndrome

Copyright information

© Springer-Verlag 1996