A comprehensive team approach to the management of patients with Prader-Willi syndrome.
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 multi-dimensional 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 long-term multidisciplinary management.
Eiholzer
Urs
U
Foundation Growth Puberty Adolescence, Zurich, Switzerland. urs.eiholzer@childgrowth.org
Whitman
Barbara Y
BY
D000293:Adolescent; D001823:Body Composition; D001827:Body Height; D015992:Body Mass Index; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D002149:Energy Intake; D015444:Exercise; D005260:Female; D013006:Growth Hormone / Q000627:therapeutic use; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010348:Patient Care Team; D011218:Prader-Willi Syndrome / Q000175:diagnosis / Q000453:epidemiology* / Q000628:therapy*; D011379:Prognosis; D018570:Risk Assessment; D012720:Severity of Illness Index; D016019:Survival Analysis; D016896:Treatment Outcome
D013006:Growth Hormone