title: Growth hormone treatment of children with Prader-Willi syndrome affects linear growth and body composition favourably.
abstract: We have compared the growth and the body composition in children with Prader-Willi syndrome (PWS) with and without growth hormone treatment (recombinant GH 0.1 IU/kg/day) after a 1-y period. Twenty-nine prepubertal children with PWS, with mean body mass index (BMI) SDS of 2.2, and 10 (control) healthy obese children with mean BMI SDS of 5.6, underwent 24-h frequent blood sampling. Both PWS and control obese children had low and similar GH levels (0.7 microg/l +/- 0.4SD). Serum IGF-I levels, however, were significantly lower in children with PWS (-1.5SDS +/- 0.8SD vs -0.2SDS +/- 0.8SD). The 29 PWS children were randomized into 2 groups of 15 and 14 subjects for GH treatment and no treatment, respectively. Height velocity increased from -1.9SDS to + 6.0SDS in the treated group (p < 0.001) and decreased from -0.1SDS to -1.4SDS in the control PWS group during the study year. BMI decreased significantly for the treated group (+3.0SDS to +2.0SDS). Relative fat mass decreased significantly, while fat-free mass increased (p < 0.001) for the treated group. No significant changes were noticed in body composition in the control PWS group. In conclusion, the low spontaneous 24-h GH secretion, regardless of body weight, and the exceptional response to growth hormone treatment together with the finding of low IGF-I levels suggest that growth hormone deficiency is a common feature of PWS, as a result of hypothalamic dysfunction. Treatment with growth hormone is beneficial for the majority of PWS children.
authors.affiliation: Department of Woman and Child Health, Karolinska Hospital, Stockholm, Sweden.
authors.forename: A C
authors.initials: AC
authors.lastname: Lindgren
authors.forename: L
authors.initials: L
authors.lastname: Hagenäs
authors.forename: J
authors.initials: J
authors.lastname: Müller
authors.forename: S
authors.initials: S
authors.lastname: Blichfeldt
authors.forename: M
authors.initials: M
authors.lastname: Rosenborg
authors.forename: T
authors.initials: T
authors.lastname: Brismar
authors.forename: E M
authors.initials: EM
authors.lastname: Ritzén
mesh_terms: D001823:Body Composition / Q000187:drug effects*; D001827:Body Height / Q000187:drug effects; D015992:Body Mass Index; D002648:Child; D002657:Child Development / Q000187:drug effects*; D002675:Child, Preschool; D002851:Chromatography, High Pressure Liquid; D005260:Female; D005500:Follow-Up Studies; D006442:Glycated Hemoglobin / Q000032:analysis; D019382:Human Growth Hormone / Q000008:administration & dosage*; D006801:Humans; D007279:Injections, Subcutaneous; D008297:Male; D009765:Obesity / Q000175:diagnosis / Q000503:physiopathology; D011218:Prader-Willi Syndrome / Q000175:diagnosis / Q000188:drug therapy* / Q000503:physiopathology; D011379:Prognosis; D012044:Regression Analysis
chemical_list: D006442:Glycated Hemoglobin; D019382:Human Growth Hormone