Molecular defects in achondroplasia and the effects of growth hormone treatment

Acta Paediatr Suppl. 1999 Feb;88(428):118-20. doi: 10.1111/j.1651-2227.1999.tb14369.x.

Abstract

Achondroplasia is a common skeletal dysplasia with severe growth retardation. Recently, mutations in the fibroblast growth factor receptor 3 (FGFR3) were identified in patients with achondroplasia. In the present study, 70 of 75 Japanese patients with achondroplasia were found to have a G1138A mutation in FGFR3, and two patients had a G1138C mutation. Growth hormone therapy was given to 145 patients with achondroplasia. Significant dose-dependent effects on skeletal growth were obtained, with no long-term adverse effects.

Publication types

  • Clinical Trial
  • Comparative Study
  • Randomized Controlled Trial

MeSH terms

  • Achondroplasia / complications
  • Achondroplasia / drug therapy*
  • Achondroplasia / genetics*
  • Body Height / drug effects*
  • Child
  • Child, Preschool
  • Female
  • Follow-Up Studies
  • Growth Disorders / drug therapy
  • Growth Disorders / etiology
  • Human Growth Hormone / administration & dosage*
  • Humans
  • Male
  • Molecular Biology
  • Mutation
  • Receptors, Fibroblast Growth Factor / genetics*
  • Treatment Outcome

Substances

  • Receptors, Fibroblast Growth Factor
  • Human Growth Hormone