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

Abstract

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.

• Authors: M Tauber, C Barbeau, B Jouret, C Pienkowski, P Malzac, A Moncla, P Rochiccioli
• Journal: Hormone research (Horm Res) Vol. 53 Issue 6 Pg. 279-87 ( 2000) ISSN: 0301-0163 [Print] Switzerland
• PMID: 11146368 (Publication Type: Journal Article)
• Copyright: Copyright 2001 S. Karger AG, Basel

Chemical References

• Human Growth Hormone
• Insulin-Like Growth Factor I

Topics

• Adolescent
• Adult
• Body Height
• Body Mass Index
• Child
• Child, Preschool
• Cryptorchidism (complications, diagnosis)
• Female
• Human Growth Hormone (blood, therapeutic use)
• Humans
• Hyperinsulinism (complications, diagnosis)
• Hypothyroidism (complications, diagnosis)
• Infant
• Insulin-Like Growth Factor I (analysis)
• Magnetic Resonance Imaging
• Male
• Pituitary Gland (pathology)
• Prader-Willi Syndrome (complications, drug therapy, physiopathology)
• Puberty
• Retrospective Studies
• Treatment Outcome