Antonino Crinò1, Graziano Grugni2. 1. Reference Center for Prader-Willi Syndrome, Bambino Gesù Hospital, Research Institute, Via Torre di Palidoro, 00050 Palidoro, Rome, Italy. nino3381@gmail.com. 2. Division of Auxology, S. Giuseppe Hospital, Research Institute, Italian Auxological Institute, Verbania, Italy.
Abstract
PURPOSE OF REVIEW: This review summarizes our current knowledge on type 2 diabetes mellitus (T2DM) and glucose metabolism alterations in Prader-Willi syndrome (PWS), the most common syndromic cause of obesity, and serves as a guide for future research and current best practice. RECENT FINDINGS: Diabetes occurs in 10-25% of PWS patients, usually in adulthood. Severe obesity is a significant risk factor for developing of T2DM in PWS. Paradoxically, despite severe obesity, a relative hypoinsulinemia, without the expected insulin resistance, is frequently observed in PWS. The majority of PWS subjects with T2DM are asymptomatic and diabetes-related complications are infrequent. Long-term growth hormone therapy does not adversely influence glucose homeostasis in all ages, if weight gain does not occur. Early intervention to prevent obesity and the regular monitoring of glucose levels are recommended in PWS subjects. However, further studies are required to better understand the physiopathological mechanisms of T2DM in these patients.
PURPOSE OF REVIEW: This review summarizes our current knowledge on type 2 diabetes mellitus (T2DM) and glucose metabolism alterations in Prader-Willi syndrome (PWS), the most common syndromic cause of obesity, and serves as a guide for future research and current best practice. RECENT FINDINGS:Diabetes occurs in 10-25% of PWSpatients, usually in adulthood. Severe obesity is a significant risk factor for developing of T2DM in PWS. Paradoxically, despite severe obesity, a relative hypoinsulinemia, without the expected insulin resistance, is frequently observed in PWS. The majority of PWS subjects with T2DM are asymptomatic and diabetes-related complications are infrequent. Long-term growth hormone therapy does not adversely influence glucose homeostasis in all ages, if weight gain does not occur. Early intervention to prevent obesity and the regular monitoring of glucose levels are recommended in PWS subjects. However, further studies are required to better understand the physiopathological mechanisms of T2DM in these patients.
Authors: V Laurier; A Lapeyrade; P Copet; G Demeer; M Silvie; E Bieth; M Coupaye; C Poitou; F Lorenzini; F Labrousse; C Molinas; M Tauber; D Thuilleaux; J Jauregi Journal: J Intellect Disabil Res Date: 2014-06-20
Authors: G Muscogiuri; L Barrea; F Faggiano; M I Maiorino; M Parrillo; G Pugliese; R M Ruggeri; E Scarano; S Savastano; A Colao Journal: J Endocrinol Invest Date: 2021-04-23 Impact factor: 4.256