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
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Publicado: MDPI AG 2021
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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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