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Update on Diabetes Mellitus and Glucose Metabolism Alterations in Prader-Willi Syndrome

  • Other Forms of Diabetes and Its Complications (J J Nolan and H Thabit, Section Editors)
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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.

Summary

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.

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Authors and Affiliations

  1. Reference Center for Prader-Willi Syndrome, Bambino Gesù Hospital, Research Institute, Via Torre di Palidoro, 00050 Palidoro, Rome, Italy

    Antonino Crinò

  2. Division of Auxology, S. Giuseppe Hospital, Research Institute, Italian Auxological Institute, Verbania, Italy

    Graziano Grugni

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  1. Antonino Crinò
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  2. Graziano Grugni
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Correspondence to Antonino Crinò.

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This article is part of the Topical Collection on Other Forms of Diabetes and Its Complications

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Crinò, A., Grugni, G. Update on Diabetes Mellitus and Glucose Metabolism Alterations in Prader-Willi Syndrome. Curr Diab Rep 20, 7 (2020). https://doi.org/10.1007/s11892-020-1284-5

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