LOW INSULIN, IGF-I AND IGFBP-3 LEVELS IN CHILDREN WITH PRADER-LABHART-WILLI-SYNDROME

It: is well established that insulin-like growth factor I (ICF-I), ins ulin-like growth factor binding protein-3 (IGFBP-3) and insulin are lo w in growth hormone deficiency, but due to their dependence on nutriti on, they are elevated in healthy obese children. As the presence of gr owth hormone deficiency in Prader-Labhart-Willi syndrome (PWS) is stil l controversial, we studied insulin, ICF-I and IGFBP-3 levels in 19 ch ildren with PWS (age range 0.5-14.6 years). Serum concentrations of in sulin (SDS: -0.7 +/- 0.9, P = 0.01) and IGF-I (SDS: -0.7 +/- 0.8,P = 0 .002) were low, but IGFBP-3 (SDS: -0.3 +/- 1.2, P = 0.2) was normal co mpared to normal weight age-matched children. Since children with PWS are typically obese, insulin, IGF-I and IGFBP-3 levels should be compa red to normal obese children who present increased levels of these hor mones. In comparison to data of healthy obese children reported in the literature, not only IGF-I, but also IGFBP-3 levels are low and fasti ng insulin levels even very low, suggesting a growth hormone deficienc y.