Noonan's syndrome: abnormalities of the growth hormone/IGF-I axis and the response to treatment with human biosynthetic growth hormone

Acta Paediatr Scand. 1991 Apr;80(4):446-50. doi: 10.1111/j.1651-2227.1991.tb11880.x.

Abstract

Auxological and endocrine data from 6 children (3 male, 3 female) aged 8.5-12.8 years with Noonan's syndrome and the results of treatment with human biosynthetic growth hormone (hGH) are presented. All the children were short (Ht SDS -3.5 to -2.3) and height velocity SDS ranged between -1.76 and +0.03. The maximum plasma growth hormone (GH) response to standard provocation tests ranged from 17 to 52 mU/l, yet, plasma insulin-like growth factor I (IGF-I) concentrations were low or low normal. Overnight GH secretory profiles were normal in all but 2 children who had disordered pulsatility with high trough concentrations. In 5 children who have completed one year of hGH therapy mean height velocity increased from 4.8 to 7.4 cm/year and the height velocity SDS ranged from +0.2 to +3.75. This improvement was associated with an increase in plasma IGF-I in three subjects. These results suggest that a defect of the GH/IGF-I axis may be present in some children with Noonan's syndrome and hGH therapy may have a role in the management of the short stature in these children.

MeSH terms

  • Age Determination by Skeleton
  • Body Height
  • Child
  • Female
  • Growth Disorders / drug therapy
  • Growth Disorders / metabolism
  • Growth Disorders / physiopathology
  • Growth Hormone / metabolism*
  • Growth Hormone / therapeutic use
  • Humans
  • Insulin-Like Growth Factor I / analysis*
  • Male
  • Noonan Syndrome / drug therapy
  • Noonan Syndrome / metabolism*
  • Noonan Syndrome / physiopathology

Substances

  • Insulin-Like Growth Factor I
  • Growth Hormone