Auxological and endocrine evolution of 28 children with Prader-Willi syndrome: Effect of GH therapy in 14 children

We report on the auxological and endocrine evolution of 28 patients present ing with Prader-Willi syndrome, Half of them received growth hormone (GH) t herapy (group 2). The spontaneous auxological evolution was analyzed in the two groups from 2 to 8 years; the mean SDS for height remained stable (-0. 6 +/- 0.6) in group 1 and decreased (from -2.0 +/- 0.9 to -2.7 +/- 0.6) in group 2. Magnetic resonance imaging showed marked pituitary hypoplasia in t he two groups. In group 2, the mean GH peak after two provocative tests was 3.8 +/- 2.4 mug/l, the mean SDS values for insulin-like growth factor I le vels were -2.0 +/- 1.5 (range from -0.5 to -5.0), The mean duration of GH t reatment was 3.6 +/- 2.9 (range 1-9.3) years. 14 children completed 1 year of treatment. The two groups had opposite evolutions in Delta SDS for heigh t (-0,8 +/- 0.8 vs. +1.1 +/- 0.8), for growth velocity (-1.9 +/- 2.2 vs. +2 .9 +/- 2.7), and for Z score of the body mass index (+0.37 +/- 1.3 vs. -0.1 4 +/- 0.76; group 1 vs. group 2). This retrospective study shows that, in c hildren with Prader-Willi syndrome and true GH deficiency, long-term GH the rapy is effective in increasing growth velocity and in maintaining body mas s index. Copyright (C) 2001 S. Karger AG, Basel.