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.
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.
D000293:Adolescent; D000328:Adult; D001786:Blood Glucose / Q000187:drug effects; D002352:Carrier Proteins / Q000097:blood; D002648:Child; D002675:Child, Preschool; D004392:Dwarfism / Q000097:blood / Q000188:drug therapy*; D005260:Female; D006128:Growth / Q000187:drug effects*; D013006:Growth Hormone / Q000097:blood; D006801:Humans; D018969:Insulin-Like Growth Factor Binding Proteins; D007334:Insulin-Like Growth Factor I / Q000009:adverse effects / Q000494:pharmacology / Q000627:therapeutic use*; D008297:Male; D011986:Receptors, Somatotropin; D011994:Recombinant Proteins / Q000627:therapeutic use; D013002:Somatomedins / Q000032:analysis; D013577:Syndrome
D001786:Blood Glucose; D002352:Carrier Proteins; D018969:Insulin-Like Growth Factor Binding Proteins; D011986:Receptors, Somatotropin; D011994:Recombinant Proteins; D013002:Somatomedins; D007334:Insulin-Like Growth Factor I; D013006:Growth Hormone