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 presenting with Prader-Willi syndrome. Half of them received growth hormone (GH) therapy (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 the two groups. In group 2, the mean GH peak after two provocative tests was 3.8 +/- 2.4 microg/l, the mean SDS values for insulin-like growth factor I levels were -2.0 +/- 1.5 (range from -0.5 to -5.0). The mean duration of GH treatment 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 height (-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. -0.14 +/- 0.76; group 1 vs. group 2). This retrospective study shows that, in children with Prader-Willi syndrome and true GH deficiency, long-term GH therapy is effective in increasing growth velocity and in maintaining body mass index.
Tauber
M
M
Service d'Endocrinologie Pédiatrique, Hôpital des Enfants, Toulouse, France. tauber.mt@chu-toulouse.fr
Barbeau
C
C
Jouret
B
B
Pienkowski
C
C
Malzac
P
P
Moncla
A
A
Rochiccioli
P
P
D000293:Adolescent; D000328:Adult; D001827:Body Height; D015992:Body Mass Index; D002648:Child; D002675:Child, Preschool; D003456:Cryptorchidism / Q000150:complications / Q000175:diagnosis; D005260:Female; D019382:Human Growth Hormone / Q000097:blood / Q000627:therapeutic use*; D006801:Humans; D006946:Hyperinsulinism / Q000150:complications / Q000175:diagnosis; D007037:Hypothyroidism / Q000150:complications / Q000175:diagnosis; D007223:Infant; D007334:Insulin-Like Growth Factor I / Q000032:analysis; D008279:Magnetic Resonance Imaging; D008297:Male; D010902:Pituitary Gland / Q000473:pathology; D011218:Prader-Willi Syndrome / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology; D011627:Puberty; D012189:Retrospective Studies; D016896:Treatment Outcome
D019382:Human Growth Hormone; D007334:Insulin-Like Growth Factor I