Is there growth hormone deficiency in Prader-Willi syndrome? - Six arguments to support the presence of hypothalamic growth hormone deficiency in Prader-Willi syndrome

Citation
U. Eiholzer et al., Is there growth hormone deficiency in Prader-Willi syndrome? - Six arguments to support the presence of hypothalamic growth hormone deficiency in Prader-Willi syndrome, HORMONE RES, 53, 2000, pp. 44-52
Citations number
88
Language
INGLESE
art.tipo
Article
Categorie Soggetti
Endocrinology, Nutrition & Metabolism
Journal title
HORMONE RESEARCH
ISSN journal
0301-0163 → ACNP
Volume
53
Year of publication
2000
Supplement
3
Pages
44 - 52
Database
ISI
SICI code
0301-0163(2000)53:<44:ITGHDI>2.0.ZU;2-D
Abstract
Prader-Labhart-Willi syndrome (PWS) is the most frequent form of syndromal obesity. its main features are associated with hypothalamic dysfunction, wh ich has not yet been comprehensively described. The aim of th is review is to present arguments to define the presence of genuine growth hormone (GH) deficiency (GHD) in these patients. Decreasing growth velocity despite the onset of obesity, reduced lean body mass in the presence of adiposity, smal l hands and feet, relatively low insulin-like growth factor-I and low insul in levels, as well as the dramatic effect of GH treatment on growth, suppor t the presence of hypothalamic GHD in PWS. Even though it might be difficul t to ultimately prove GHD in PWS because of the obesity-induced counterregu lation, the hormonal situation differs from that in simple obesity. The eff ects of long-term therapies with GH on body composition in these patients a re summarized. GH therapy dramatically changes the phenotype of PWS in chil dhood: height and weight become normal and there is a sustained impact on t he net loss of body fat. We conclude that GHD may account for several featu res of PWS, Copyright (C) 2000 S. Karger AG, Basel.