Get 20M+ Full-Text Papers For Less Than $1.50/day. Start a 14-Day Trial for You or Your Team.

Learn More →

A Comprehensive Team Approach to the Management of Patients with Prader-Willi Syndrome

A Comprehensive Team Approach to the Management of Patients with Prader-Willi Syndrome INTRODUCTION Prader-Willi syndrome (PWS) is a genetic disorder characterized by extreme obesity accompanied by other, multisystem clinical manifestations encompassing both physical and behavioral/cognitive abnormalities. The multidimensional problems of patients with PWS cannot be treated with a single intervention and benefit from a team approach to management to optimize outcomes. Childhood stature below target height and reduced final height are some defining characteristics of PWS, and compelling evidence from growth hormone (GH) treatment trials suggests that hypothalamic GH deficiency exists. Treatment with GH has been shown to increase height velocity in children with PWS, decrease weight-for-height index values and body fat mass, and have a positive effect on lean body mass during at least the first year of therapy. In addition to medical concerns, the behavioral manifestations, including an uncorrectable deficit in appetite control, and cognitive limitations associated with PWS, require longterm multidisciplinary management. KEY WORDS growth, growth hormone, metabolic disturbance, behavioral problems, congenital disorders, obesity, Prader-Willi syndrome Reprint address: Dr. med. Urs Eiholzer Foundation Growth Puberty A d o l e s c e n c e Moehrlistrasse 6 9 C H - 8 0 0 6 Zürich, Switzerland e-mail: urs.eiholzer@childgrowth.org VOLUME 17, NO. 9, 2004 Prader-Willi syndrome http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Journal of Pediatric Endocrinology and Metabolism de Gruyter

A Comprehensive Team Approach to the Management of Patients with Prader-Willi Syndrome

Loading next page...
 
/lp/de-gruyter/a-comprehensive-team-approach-to-the-management-of-patients-with-StwA6CF0BG

References

References for this paper are not available at this time. We will be adding them shortly, thank you for your patience.

Publisher
de Gruyter
Copyright
Copyright © 2004 by the
ISSN
0334-018X
eISSN
2191-0251
DOI
10.1515/JPEM.2004.17.9.1153
Publisher site
See Article on Publisher Site

Abstract

INTRODUCTION Prader-Willi syndrome (PWS) is a genetic disorder characterized by extreme obesity accompanied by other, multisystem clinical manifestations encompassing both physical and behavioral/cognitive abnormalities. The multidimensional problems of patients with PWS cannot be treated with a single intervention and benefit from a team approach to management to optimize outcomes. Childhood stature below target height and reduced final height are some defining characteristics of PWS, and compelling evidence from growth hormone (GH) treatment trials suggests that hypothalamic GH deficiency exists. Treatment with GH has been shown to increase height velocity in children with PWS, decrease weight-for-height index values and body fat mass, and have a positive effect on lean body mass during at least the first year of therapy. In addition to medical concerns, the behavioral manifestations, including an uncorrectable deficit in appetite control, and cognitive limitations associated with PWS, require longterm multidisciplinary management. KEY WORDS growth, growth hormone, metabolic disturbance, behavioral problems, congenital disorders, obesity, Prader-Willi syndrome Reprint address: Dr. med. Urs Eiholzer Foundation Growth Puberty A d o l e s c e n c e Moehrlistrasse 6 9 C H - 8 0 0 6 Zürich, Switzerland e-mail: urs.eiholzer@childgrowth.org VOLUME 17, NO. 9, 2004 Prader-Willi syndrome

Journal

Journal of Pediatric Endocrinology and Metabolismde Gruyter

Published: Sep 1, 2004

There are no references for this article.