Diabetes Mellitus in Prader-Willi Syndrome: Natural History during the Transition from Childhood to Adulthood in a Cohort of 39 Patients

Type 2 diabetes mellitus (T2DM) affects 20% of patients with Prader-Willi syndrome (PWS), with many cases diagnosed during the transition period. Our aim was to describe the natural history of T2DM in patients with PWS before the age of 25 years and to develop screening and preventive strategies. Th...

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Autores principales:	Alice Clerc, Muriel Coupaye, Héléna Mosbah, Graziella Pinto, Virginie Laurier, Fabien Mourre, Christine Merrien, Gwenaëlle Diene, Christine Poitou, Maithé Tauber
Formato:	article
Lenguaje:	EN
Publicado:	MDPI AG 2021
Materias:	Prader-Willi syndrome type 2 diabetes mellitus syndromic obesity Medicine R
Acceso en línea:	https://doaj.org/article/27b7cfefc28e41828d8db289215545be
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spelling	oai:doaj.org-article:27b7cfefc28e41828d8db289215545be2021-11-25T18:01:41ZDiabetes Mellitus in Prader-Willi Syndrome: Natural History during the Transition from Childhood to Adulthood in a Cohort of 39 Patients10.3390/jcm102253102077-0383https://doaj.org/article/27b7cfefc28e41828d8db289215545be2021-11-01T00:00:00Zhttps://www.mdpi.com/2077-0383/10/22/5310https://doaj.org/toc/2077-0383Type 2 diabetes mellitus (T2DM) affects 20% of patients with Prader-Willi syndrome (PWS), with many cases diagnosed during the transition period. Our aim was to describe the natural history of T2DM in patients with PWS before the age of 25 years and to develop screening and preventive strategies. Thirty-nine patients followed in the French PWS Reference Center were included (median age 25.6 years [23.7; 31.7]). Twenty-one had been treated with growth hormone (GH), fifteen had not, and three had an unknown status. The median age at T2DM diagnosis was 16.8 years (11–24) and the median BMI was 39 kg/m<sup>2</sup> [34.6; 45], with 34/35 patients living with obesity. The patients displayed frequent psychiatric (48.3% hospitalization,) and metabolic (56.4% hypertriglyceridemia,) comorbidities and a parental history of T2DM (35.7%) or overweight (53.6%) compared to the PWS general population. There was no difference in BMI and metabolic complications between the GH-treated and non-GH-treated groups at T2DM diagnosis. Patients with PWS who develop early T2DM have severe obesity, a high frequency of psychiatric and metabolic disorders, and a family history of T2DM and overweight. These results underline the need for early identification of patients at risk, prevention of obesity, and repeated blood glucose monitoring during the transition period.Alice ClercMuriel CoupayeHéléna MosbahGraziella PintoVirginie LaurierFabien MourreChristine MerrienGwenaëlle DieneChristine PoitouMaithé TauberMDPI AGarticlePrader-Willi syndrometype 2 diabetes mellitussyndromic obesityMedicineRENJournal of Clinical Medicine, Vol 10, Iss 5310, p 5310 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Prader-Willi syndrome type 2 diabetes mellitus syndromic obesity Medicine R
spellingShingle	Prader-Willi syndrome type 2 diabetes mellitus syndromic obesity Medicine R Alice Clerc Muriel Coupaye Héléna Mosbah Graziella Pinto Virginie Laurier Fabien Mourre Christine Merrien Gwenaëlle Diene Christine Poitou Maithé Tauber Diabetes Mellitus in Prader-Willi Syndrome: Natural History during the Transition from Childhood to Adulthood in a Cohort of 39 Patients
description	Type 2 diabetes mellitus (T2DM) affects 20% of patients with Prader-Willi syndrome (PWS), with many cases diagnosed during the transition period. Our aim was to describe the natural history of T2DM in patients with PWS before the age of 25 years and to develop screening and preventive strategies. Thirty-nine patients followed in the French PWS Reference Center were included (median age 25.6 years [23.7; 31.7]). Twenty-one had been treated with growth hormone (GH), fifteen had not, and three had an unknown status. The median age at T2DM diagnosis was 16.8 years (11–24) and the median BMI was 39 kg/m<sup>2</sup> [34.6; 45], with 34/35 patients living with obesity. The patients displayed frequent psychiatric (48.3% hospitalization,) and metabolic (56.4% hypertriglyceridemia,) comorbidities and a parental history of T2DM (35.7%) or overweight (53.6%) compared to the PWS general population. There was no difference in BMI and metabolic complications between the GH-treated and non-GH-treated groups at T2DM diagnosis. Patients with PWS who develop early T2DM have severe obesity, a high frequency of psychiatric and metabolic disorders, and a family history of T2DM and overweight. These results underline the need for early identification of patients at risk, prevention of obesity, and repeated blood glucose monitoring during the transition period.
format	article
author	Alice Clerc Muriel Coupaye Héléna Mosbah Graziella Pinto Virginie Laurier Fabien Mourre Christine Merrien Gwenaëlle Diene Christine Poitou Maithé Tauber
author_facet	Alice Clerc Muriel Coupaye Héléna Mosbah Graziella Pinto Virginie Laurier Fabien Mourre Christine Merrien Gwenaëlle Diene Christine Poitou Maithé Tauber
author_sort	Alice Clerc
title	Diabetes Mellitus in Prader-Willi Syndrome: Natural History during the Transition from Childhood to Adulthood in a Cohort of 39 Patients
title_short	Diabetes Mellitus in Prader-Willi Syndrome: Natural History during the Transition from Childhood to Adulthood in a Cohort of 39 Patients
title_full	Diabetes Mellitus in Prader-Willi Syndrome: Natural History during the Transition from Childhood to Adulthood in a Cohort of 39 Patients
title_fullStr	Diabetes Mellitus in Prader-Willi Syndrome: Natural History during the Transition from Childhood to Adulthood in a Cohort of 39 Patients
title_full_unstemmed	Diabetes Mellitus in Prader-Willi Syndrome: Natural History during the Transition from Childhood to Adulthood in a Cohort of 39 Patients
title_sort	diabetes mellitus in prader-willi syndrome: natural history during the transition from childhood to adulthood in a cohort of 39 patients
publisher	MDPI AG
publishDate	2021
url	https://doaj.org/article/27b7cfefc28e41828d8db289215545be
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Diabetes Mellitus in Prader-Willi Syndrome: Natural History during the Transition from Childhood to Adulthood in a Cohort of 39 Patients

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