An Update on Prader-Willi Syndrome with Diabetes Mellitus

Abstract

Prader-Willi syndrome (PWS) is a complex multisystem genetic disorder that arises from lack of expression of paternally inherited imprinted genes on chromosome 15q11-q13 1). PWS is characterized by infantile hypotonia, followed by progressive obesity and hyperphagia in childhood. They often develop type 2 diabetes mellitus (T2DM) related to severe morbid obesity. Obesity is a critical problem in PWS and the yearly mortality rate for obesity induced complication is about 7% 1). In this mini review, we discuss an update on PWS with disorder of glucose metabolism , including epidemiology and management of PWS patients with T2DM.