title: Body composition abnormalities in children with Prader-Willi syndrome and long-term effects of growth hormone therapy.
abstract: Obesity and hypothalamic GH deficiency contribute in different ways to the disturbances of body composition in Prader-Willi syndrome (PWS); while both increase the fat compartment, the reduction of lean tissue mass has been attributed mainly to GH deficiency. Therefore, body composition measured by dual-energy X-ray absorptiometry was prospectively studied in 12 overweight children with PWS and weight for height (WfH) SDS >0 before and during 3.5 years of treatment with hGH (0.037 mg/kg/day) on average. In the long term, there is a net reduction of body fat from 3.1 to 1.2 SD, with a minimum at the end of the second year of treatment. WfH SDS correctly reflects body fat mass and its changes. The initial deficit of lean mass (-1.6 SD) is counteracted by GH only during the first year of therapy (increase to -1.25 SD). But in the long term, GH therapy does not further compensate for this deficit, when lean mass is corrected for its growth-related increase. In conclusion, exogenous GH changes the phenotype of children with PWS: fat mass becomes normal, but, at least in the setting studied, GH is not sufficient to normalize lean tissue mass.
authors.affiliation: Foundation Growth Puberty Adolescence, Zurich, Switzerland. ue@active.ch
authors.forename: U
authors.initials: U
authors.lastname: Eiholzer
authors.forename: D
authors.initials: D
authors.lastname: l'Allemand
authors.forename: I
authors.initials: I
authors.lastname: van der Sluis
authors.forename: H
authors.initials: H
authors.lastname: Steinert
authors.forename: T
authors.initials: T
authors.lastname: Gasser
authors.forename: K
authors.initials: K
authors.lastname: Ellis
mesh_terms: D015502:Absorptiometry, Photon; D000273:Adipose Tissue; D000293:Adolescent; D000375:Aging; D001823:Body Composition; D001827:Body Height; D002648:Child; D002675:Child, Preschool; D005260:Female; D019382:Human Growth Hormone / Q000008:administration & dosage / Q000627:therapeutic use*; D006801:Humans; D008297:Male; D011218:Prader-Willi Syndrome / Q000188:drug therapy* / Q000503:physiopathology*; D011627:Puberty; D016896:Treatment Outcome
chemical_list: D019382:Human Growth Hormone