Prader-Willi syndrome in adults

With earlier diagnosis and institution of preventive interventions persons with Prader-Willi syndrome (PWS) can expect a life expectancy that replicates that of comparable, nonaffected young Americans. The availability of new treatment modalities for the prevention and treatment of osteoporosis, such as the bisphosphates; early initiation of gonadal hormone replacement; aggressive management of established risk factors for cardiovascular disease; and the administration of human recombinant growth hormone to ameliorate body composition and lipid abnormalities should decrease the risk of type 2 diabetes and cardiovascular disease and improve the healthspan of PWS adults. Such therapies should diminish the adverse health consequences characteristic of adult PWS and enhance quality of life for older individuals with the disorder. Accordingly, adult practitioners will need to consider diagnostic and treatment options for adults with PWS that are commonly employed in the management of older Americans. This review will summarize clinical guidelines for the diagnosis and management of prevalent conditions characteristic of adult PWS.