Treatment with recombinant human insulin-like growth factor I of children with growth hormone receptor deficiency (Laron syndrome). Kabi Pharmacia Study Group on Insulin-like Growth Factor I Treatment in Growth Hormone Insensitivity Syndromes

Acta Paediatr Suppl. 1992 Sep:383:137-42.

Abstract

Twenty-seven patients (14 female, 13 male; 3 pubertal) with growth hormone receptor deficiency (Laron syndrome) were treated with recombinant insulin-like growth factor I (IGF-I), 40-120 micrograms/kg body weight b.d., for up to 12 months. Height SDS was between -9.1 and -3.2 at the start of treatment (age, 3.7-22.9 years). Before treatment, most patients had increased basal serum concentrations of growth hormone (2.4-208 mU/l) and low serum concentrations of IGF-I (< 20-69 micrograms/l), IGF-II (69-295 micrograms/l) and IGF binding protein-3 (0.16-1.59 mg/l). In all but the two oldest patients, the growth rate increased by more than 2 cm/year compared with that before treatment. Asymptomatic hypoglycaemia (blood glucose < 3.0 mmol/l) was recorded in ten patients in 0.7% of measurements. Four patients experienced symptomatic hypoglycaemia. A transient asymptomatic decrease in serum potassium occurred in most patients after injections.

Publication types

  • Clinical Trial

MeSH terms

  • Adolescent
  • Adult
  • Blood Glucose / drug effects
  • Carrier Proteins / blood
  • Child
  • Child, Preschool
  • Dwarfism / blood
  • Dwarfism / drug therapy*
  • Female
  • Growth / drug effects*
  • Growth Hormone / blood
  • Humans
  • Insulin-Like Growth Factor Binding Proteins
  • Insulin-Like Growth Factor I / adverse effects
  • Insulin-Like Growth Factor I / pharmacology
  • Insulin-Like Growth Factor I / therapeutic use*
  • Male
  • Receptors, Somatotropin*
  • Recombinant Proteins / therapeutic use
  • Somatomedins / analysis
  • Syndrome

Substances

  • Blood Glucose
  • Carrier Proteins
  • Insulin-Like Growth Factor Binding Proteins
  • Receptors, Somatotropin
  • Recombinant Proteins
  • Somatomedins
  • Insulin-Like Growth Factor I
  • Growth Hormone