Abstract
GHIS (Laron Syndrome) is characterised by normal or elevated GH secretion, impaired IGF-I production, characteristic facial phenotype and extreme growth failure. 3 patients with GHIS [basal GH(mU/L) 102, 34.5, 99.5 respectively; IGF-I (μg/L) generation test (GH 0.1U/kg/day for 4 days s.c.) 24 (before) −27 (after), 26 - 26, 24 - 20] received therapy with recombinant IGF-I (IGEF, Kabi Pharmacia) at 120 μg/kg b.d. s.c. Anthropometry gave the following results:
Craniofacial measurements during 4 months therapy (patient 3) showed the following changes (cm): head length (−4 SD)+0.9, head circumference (−3.5SDS)+0.5, skull height(−3.5SD)+0.8, facial height(−8.0SD)+0.3. Standardised photography in all 3 patients showed a change in facial phenotype.
IGF-I stimulates linear growth in all patients, the greatest changes being in the youngest. Skinfold thicknesses fell during treatment indicating a lipolytic effect of IGF-I. Anthropometric and photographic assessment suggest a change in facial morphology.
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Leonard, J., Samuels, M., Murphy, J. et al. ANTHROPOMLTRIC ASSESSMENT OF ANABOLIC AND LIPOLYTIC EFFECTS OF RECOMBINANT IGF-I THERAPY IN 3 CHILDREN WITH GROWTH HORMONE INSENSITIVITY SYNDROME(GHIS). Pediatr Res 33 (Suppl 5), S43 (1993). https://doi.org/10.1203/00006450-199305001-00242
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DOI: https://doi.org/10.1203/00006450-199305001-00242